Electrodiagnostic Medicine: A Practical Approach Nestor Galvez-Jimenez Alexandra Soriano John A. Morren

Electrodiagnostic
Medicine
A Practical Approach
Nestor Galvez-Jimenez
Alexandra Soriano
John A. Morren
Editors
123
Electrodiagnostic Medicine
Nestor Galvez-Jimenez
Alexandra Soriano • John A. Morren
Editors
Electrodiagnostic
Medicine
A Practical Approach
Editors
Nestor Galvez-Jimenez Alexandra Soriano
Braathen Neurological Center Braathen Neurological Center
Cleveland Clinic Florida Cleveland Clinic Florida
Weston, FL Weston, FL
USA USA
Cleveland Clinic Lerner College of Cleveland Clinic Lerner College of
Medicine of Case Western Reserve Medicine of Case Western Reserve
University University
Cleveland, OH Cleveland, OH
USA USA
John A. Morren
Neuromuscular Center
Neurological Institute
Cleveland Clinic, and Cleveland Clinic
Lerner College of Medicine of Case
Western Reserve University
Cleveland, OH
USA
ISBN 978-3-030-74996-5 ISBN 978-3-030-74997-2 (eBook)

https://doi.org/10.1007/978-3-030-74997-2
© Springer Nature Switzerland AG 2021, corrected publication 2022

This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or
part of the material is concerned, specifically the rights of translation, reprinting, reuse of
illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way,
and transmission or information storage and retrieval, electronic adaptation, computer software,
or by similar or dissimilar methodology now known or hereafter developed.
The use of general descriptive names, registered names, trademarks, service marks, etc. in this
publication does not imply, even in the absence of a specific statement, that such names are
exempt from the relevant protective laws and regulations and therefore free for general use.
The publisher, the authors and the editors are safe to assume that the advice and information in
this book are believed to be true and accurate at the date of publication. Neither the publisher nor
the authors or the editors give a warranty, expressed or implied, with respect to the material
contained herein or for any errors or omissions that may have been made. The publisher remains
neutral with regard to jurisdictional claims in published maps and institutional affiliations.
This Springer imprint is published by the registered company Springer Nature Switzerland AG
The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland
Dedication to Dr. Virgilio Salanga
We have been privileged to have worked
with some of the best
electrodiagnosticians, neurologists,
residents, and fellows at Cleveland Clinic.
Asa Wilbourn, Maurice Hanson, Kerry
Levin, Robert Shields, Richard Lederman,
Hiroshi Mitsumoto, and many others
come to mind immediately. However, for
most of us Dr. Virgilio Salanga, to whom
this work is dedicated, had a major
influence, and as a consequence made a lasting impact on our
interest and understanding in electrodiagnostic medicine.
Actually, Virgil taught generations of residents and fellows, both
in Ohio and in Florida, many of whom went on to prestigious
academic and practice positions in the USA and internationally.
Upon his retirement he was the holder of the John and Margaret
Krupa distinguished Chair in Neurology at Cleveland Clinic
Florida (CCFla) and Chairman of Neurology at CCFla.
Virgil is a Magna Cum Laude graduate with a Doctor of
Medicine and Surgery degree from the University of Santo
Thomas School of Medicine, in Manila, Philippines. After mixed
surgery and internal medicine internships at Michael Reese
Hospital and Medical Center in Chicago, Virgil went on to do his
residency and neurophysiology training at the Mayo Clinic in
Rochester, Minnesota, where he was a student of Edward
H. Lambert and Jasper Daube. In Rochester, he confided to us on
more than one occasion; he had some of his best formative
years, having worked under Arthur Waltz and interacted with
Thoralf Sundt both from Mayo, when working on his Master of
Science thesis on Regional Cerebral Blood Flow During
Stimulation of the Seventh Cranial Nerve. The resultant degree
was granted by the University of Minnesota. He had the privilege
to study and collaborate with many others who became well-
known electrodiagnosticians in their own right, such as Asa
Wilbourn, H. Royden Jones Jr., Ludwig Gutmann, Ram Ayyar,
and many others. Virgil rose through the ranks at Cleveland
Clinic in Cleveland to Vice-chair of Neurology and subsequently
moved to Florida in 1988 (with Maurice Hanson and
technologist Mary Ronnenberg) to establish the Department of
Neurology and the Neurophysiology Laboratory at the Cleveland
Clinic in Florida. Upon his retirement, the department had
grown to a very busy and academic unit with neurology residents
and fellows, in addition to many rotating medical students. Many
of us who had the privilege to study and work under him at
various times in our academic and professional career,
particularly in electrodiagnostic medicine and neurology, were
the recipients of an unabated commitment to teaching and
excellence. We could not wait to the time of day to be in his office
for review of the procedures performed during the day, especially
those complex studies in which we wanted to hear his
explanation and critical thinking. He was very generous with his
time despite the daily clinical responsibility. He was firm, but
non-judgmental, and he always expected the best from all of us,
never accepting less. In addition, his high-yield lectures were all
well attended by residents, fellows, staff, and technologists.
In spirit, this book is the product of the sum of those interactions
with our mentors in Cleveland, Ohio, but particularly with
Virgilio Salanga to whom this book is dedicated, and to CCFla
in which most of the authors and editors have made their
academic practice and/or formative years.
Foreword
It is with immense pleasure that I write the foreword to this book that is col-
laboratively authored by a former colleague, trainees, and laboratory tech-
nologists at the Cleveland Clinic—Florida, along with other alumni of the
Cleveland Clinic—Ohio. I greatly admire with pride and satisfaction follow-
ing the careers of my former colleague, Dr. Nestor Galvez-Jimenez, and my
former residents and fellows, Drs. Michelle Dompenciel, Raghav
Govindarajan, Ramon Lugo, John A. Morren, and Alexandra Soriano. They
are all astute clinicians and accomplished electrodiagnosticians.
This book is well written and organized, replete with visually enhancing
anatomical and technical illustrations, starting with the underlying principles
and practices, optimal techniques of nerve conduction studies, and electro-
myography. Then specific disorders of the peripheral nervous system are dis-
cussed clinically and how properly performed electrodiagnostic studies will
complement the clinical neurological assessment. This book will be a valu-
able resource in the field of electrodiagnostic medicine that trainees and
existing practitioners will find very helpful. Beginners and experts and men-
tors and mentees will find this book a user-friendly guide in correctly per-
forming and reporting clinically meaningful nerve conduction studies and
electromyography.
Finally, I am deeply humbled and appreciative that this book is dedicated
to me by the editors. It is often said: Teaching is an art that takes time and
patience. And that is certainly true of teaching electrodiagnostic studies. I
remember how I myself was taught this discipline by my mentors, the late Dr.
Jasper Daube and Dr. Edward Lambert, at the Mayo Clinic-Rochester. I am
grateful and pleased to have contributed to the training and mentoring of
several of the coauthors of this book. I wish them even more success in the
future.
Virgilio D. Salanga
Department of Neurology
Cleveland Clinic – Florida
Weston, FL, USA
vii
Preface
Few Antecedent Words of Gratitude from NGJ
When one of us (NGJ) was approached by Springer to work on a book proj-

ect, the decision to work on a book on electrodiagnostic medicine was easy.
The field of electrodiagnostic medicine has grown exponentially with a solid
body of evidence based on careful critical thinking, research, mathematical
thought, and engineering with the appropriate interfacing of neurophysiologi-
cal and neuroanatomical concepts. Hence, electrodiagnostic medicine is a
field with an interdisciplinary nature to the science, study performance, and
approach to patients. Therefore, producing a book of this nature (as has been
wisely said many times) is not done in isolation but is the product of a “vil-
lage.” Having asked Drs. John A. Morren and Alexandra Soriano to join me
on this project was one of the wisest decisions I made when organizing this
project. This is now our work as a team effort. They added editorial expertise,
breath of knowledge, a fresh look, and attention to detail so important in
projects of this type, particularly as it pertains to electrodiagnostic medicine.
My heartfelt thanks go particularly to John A. Morren who added clarity of
thought and made what appears complex to explain easy for the reader to
understand. This work is as much his work as everyone else’s. He read and
editorialized each chapter and attended to my many phone calls no matter
how basic some of those were to make sure we all understood what was con-
veyed in each chapter. It was the best decision to have John provide his insight
and knowledge and Alex adding her expertise in the performance and under-
standing on single fiber EMG, an area in which she excels like no one I know,
and her ability to write electrodiagnostic reports, which is not as easy as many
would make you believe. In addition, I have to give thanks to our technolo-
gists from Cleveland Clinic in Ohio and Florida who gave their time and
expertise and in many instances were willing to be models for the atlas. My
thanks also go to Jessica Galvez, BSc, MEd, PsyD candidate, who, in spite of
a busy doctoral study schedule, managed to participate as model for some of
the laboratory pictures that needed to be replaced on short notice, saving the
day for us. Finally, I will be remiss if I do not acknowledge all the invited
authors, all authorities, and accomplished electrodiagnosticians who took
time from their very busy schedules to participate in this project.
ix
x Preface
Using the Book
One of the critical challenges facing trainees and experienced electrodiagnos-

ticians alike is the attention to detail when performing nerve conduction stud-
ies (NCS) and needle electrode examination (NEE). This is needed for the
coherent interpretation of findings, including when to discard that relatively
unimportant finding, recognize an artifact, and ultimately know how to put all
the pieces together for a cogent EDX diagnosis. Words matter, and sentences
matter in a way that can hardly be more epitomized than with the explana-
tions and conclusions in EDX medicine. Some may argue that an imprecise
word or a superfluous comment may lead to confusion and unnecessary
assessments by the referring physician, or in the worst-case scenario, even an
unnecessary surgical procedure.
The book begins with Chap. 1, which is intended to provide the novice and
expert alike with the neurophysiology and neuroanatomical fundamentals on
the theory and principles for the practice and understanding of EDX studies.
In addition, it provides general concepts on the assessment of common neuro-
muscular complaints and how to use the EDX techniques to approach and
study these patients. More often than not, patients come with symptoms or
complaints, and less so with established neuromuscular diagnoses. Hence, the
hope is to provide enough knowledge to get a handle on EDX thought process
for an appropriate work-up. Chapter 2 describes the most common NCS tech-
niques in great detail using actual individuals in pictures demonstrating the
performance of the studies, detailing the pertinent anatomical localization
within the segment being evaluated, in addition to helpful tips and common
pitfalls. Chapter 3 provides a pictorial survey on the anatomical localization,
and appropriate placement of the needle electrode, with similarly helpful tips
and common pitfalls for each muscle that may be examined. Further, in Chap.
4, a deeper understanding of the electronics and neurophysiology relating to
NEE is facilitated, with explanation of normal and abnormal findings on
NEE. This was provided masterfully by Bryan Tsao, MD, who is a leading
expert in this area, known for his excellent teaching of the topic, particularly
at sessions of the American Association of Neuromuscular and Electrodiagnostic
Medicine (AANEM). This is followed by other excellent chapters written by
experts in their respective fields and/or academic practices with the hope of
providing added insight on the fundamentals of electrodiagnostically assess-
ing patients with neuromuscular conditions. Therefore, these chapters delve
into the major conditions often encountered in a busy neuromuscular medicine
practice. Chapter 5 expands on the mononeuropathies of the upper and lower
extremities with their respective EDX assessments written by Ramon Lugo,
MD, and Alexandra Soriano, MD. Chapter 6 written by Megha Dhamne, MD,
and John A. Morren, MD, deals with the EDX assessment and nuances of
polyneuropathies in a very scholarly fashion. For Chap. 7 on brachial plexopa-
thies, we were extremely lucky to have Mark Ferrante, MD, who is a renowned
national and international expert on the topic. He gives his insight and tremen-
dous knowledge on the EDX assessment of patients with brachial plexus
lesions. One of us (NGJ) had the privilege to be at the EMG laboratory during
residency training when Mark was a fellow at our institution working with Asa
Preface xi
Wilbourn and witnessed how between them, “the book” on the EDX assess-
ment of the brachial plexus and plexopathies was essentially rewritten the way
we know it today. Chapter 8 provides another scholarly discussion by Karen
Karwa, MD, and John A. Morren, MD, on radiculopathies, the work-up for
which is commonly requested in EDX medicine, yet continues to be a source
of spurious interpretations and repeat studies, particularly when coming from
less experienced laboratories. A major source for neuromuscular consultations
and complex EDX assessment is that for patients with disorders of the motor
neuron and mimicker conditions. Michelle Dompenciel, MD, provides a dis-
cussion of the topic in Chap. 9, and more importantly provides the EDX
approach to this complex group of patients. Michelle is an accomplished elec-
trodiagnostician, and we were excited in having her contribute this important
chapter.
Chapter 10 by Elanagan Nagarajan, MD, and Raghav Govindarajan, MD,
well known in the area of neuromuscular medicine, provides an overview of
the pathophysiology and clinical and EDX assessment of patients with neu-
romuscular junction transmission disorders. Dr. Govindarajan is quite active
in the AANEM and one of his main area of interest is that of neuromuscular
junction disorders. Their insight and approach to the work-up of these
patients, with an emphasis on technical proficiency and interpretation accu-
racy of this somewhat complex aspect of electrodiagnostic medicine, is of
great benefit to the reader. Chapter 11 is contributed by Alexandra Soriano,
MD. Alex is an accomplished electrodiagnostician particularly in the assess-
ment, performance, and interpretation of single fiber EMG. She gets referrals
from all over the region and country and has been a source of inspiration and
along with John A. Morren has provided her expertise and meticulous atten-
tion to detail on all matters of EDX medicine. Chapter 12, written by Payam
Soltanzadeh, MD, provides an excellent overview, with discourse on core
concepts and fundamentals pertaining to the assessment and interpretation of
EDX studies in patients with myopathies. The book concludes with Chap. 13
spearheaded by Alex Soriano, MD, and further refined by John A. Morren,
MD, providing a succinct overview on how to write an EDX report, particu-
larly in keeping with the recommendations set forth by the AANEM. Just as
the practice of EDX medicine is both an art and science, so is the ability to
write an EDX report, as this chapter elaborates.
It is the sincere hope of the editors of this work that we have provided a
fresh overview on the subject of electrodiagnostic medicine and that this
book will serve as a primer to many laboratories, as it will be in ours. We
anticipate that it will be very helpful as reference companion, especially to
instructors and lifelong learners in the field (not excluding former and future
residents and fellows). We also hope that our readers have ever-increasing
gratification in the practice of high-quality electrodiagnostic medicine that is
not only a meaningful tribute to their mentors in the field but a great service
to patients who trust us to do the best for them.
Weston, FL Nestor Galvez-Jimenez

Weston, FL Alexandra Soriano
Cleveland, OH John A. Morren
Contents
1 Principles of Electrodiagnosis: Introduction�� 1

Nestor Galvez-Jimenez, John A. Morren, and Alexandra Soriano
2 Atlas of Nerve Conduction Studies (NCS) �� 25
Nestor Galvez-Jimenez, John A. Morren, Alexandra Soriano,
Karin Armstrong, Melissa Goldberg, Lourdes Gonzalez,
and Dana Higginbotham
3 Atlas of Needle Electrode Examination (NEE)�� 43
Nestor Galvez-Jimenez, John A. Morren, and Alexandra Soriano
4 Introduction to Needle Electromyography�� 65
Bryan Tsao
5 Mononeuropathies �� 83
Ramon Lugo and Alexandra Soriano
6 Polyneuropathies�� 121
Megha Chetan Dhamne and John A. Morren
7 Brachial Plexopathies�� 157
Mark A. Ferrante
8 Radiculopathies�� 195
Karen A. Karwa and John A. Morren
9 Motor Neuron Diseases�� 213
Michelle M. Dompenciel
10 Disorders of the Neuromuscular Junction�� 227
Raghav Govindarajan and Elanagan Nagarajan
11 Single Fiber EMG�� 249
Alexandra Soriano
12 Myopathies�� 255
Payam Soltanzadeh
13 Recommendations for Writing an Electrodiagnostic
Study Report�� 265
Alexandra Soriano and John A. Morren
Correction to: Radiculopathies. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . C1
Index�� 271
xiii
Contributors
Karin Armstrong, C.N.C.T. Neurophysiology Lab, Cleveland Clinic

Florida, Weston, FL, USA
Megha Chetan Dhamne, MBBS, MD Department of Neurology, Dr. L H
Hiranandani Hospital, Mumbai, Maharashtra, India
Michelle M. Dompenciel, MD Department of Neurology, Cleveland Clinic,
Weston, FL, USA
Mark A. Ferrante, MD University of Tennessee Health Science Center,
Memphis, TN, USA
Nestor Galvez-Jimenez, MD, MSc, MHA, FACP Braathen Neurological
Center, Cleveland Clinic Florida, Weston, FL, USA
Cleveland Clinic Lerner College of Medicine of Case Western Reserve
University, Cleveland, OH, USA
Melissa Goldberg, BS, C.N.C.T. Neurophysiology Lab, Cleveland Clinic
Lourdes Gonzalez, C.N.C.T. Neurophysiology Lab, Cleveland Clinic
Raghav Govindarajan, MD, FAAN, FANA, FACP Department of
Neurology, Neurology Clinics, ALS and MDA Clinic, EMG/Neurophysiology
Lab, Clinical Outcomes for Department of Neurology, University of Missouri,
Columbia, MO, USA
Department of Neurology, University of Missouri, Columbia, MO, USA
Dana Higginbotham, R.NCS.T. Neurophysiology Lab, Neuromuscular
Center, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA
Karen A. Karwa, MD Neurology of the Rockies, Englewood, Colorado,
USA
Ramon Lugo, MD Cleveland Clinic Florida, Weston, FL, USA
John A. Morren, MD/MBBS (Hons), FAAN, FAANEM Neuromuscular
Center, Neurological Institute, Cleveland Clinic, and Cleveland Clinic Lerner
College of Medicine of Case Western Reserve University, Cleveland, OH, USA
xv
xvi Contributors
Elanagan Nagarajan, MD Department of Neurology, Neurology Clinics,

ALS and MDA Clinic, EMG/Neurophysiology Lab, Clinical Outcomes for
Payam Soltanzadeh, MD Neuromuscular Program, Department of
Neurology, UCLA, Los Angeles, CA, USA
Alexandra Soriano, MD Braathen Neurological Center, Cleveland Clinic
Cleveland Clinic Lerner College of Medicine of Case Western Reserve
Bryan Tsao, MD, MBA, FAAN Department of Neurology, Loma Linda
University School of Medicine, Loma Linda, CA, USA
Principles of Electrodiagnosis:
Introduction 1
Nestor Galvez-Jimenez, John A. Morren,
and Alexandra Soriano
Introduction myopathy, neuropathy or radiculopathy all in one

request or reason for study do not provide enough
Michael Aminoff [1] very eloquently defined the pertinent information and the electrodiagnosti-
common “misusage” of the term electromyogra- cian must perform an independent history and
phy (EMG) … “electromyography refers strictly examination to better grasp the clinical context in
to methods used to record the electrical activity which the study is being utilized. This is consid-
of muscle, … However … it has come to have a ered essential despite ever-escalating pressures a
wider meaning which encompasses also the elec- physician has for an increasing number of stud-
trodiagnostic techniques used to study the func- ies, and other operational and/or insurance and
tional integrity of the peripheral nerves and the reimbursements pressures to do more with less.
neuromuscular junction.” Therefore, for many Therefore, the “EMG” is best performed when
physicians and laypersons alike, the EMG is at the referring or treating physician has already in
times shrouded in mystery, often resulting in mis- mind some tentative conclusions about the
guided fears and confusion. Consequently, a fre- patient’s symptoms and request the study after
quent and highly frustrating scenario is that of a having done a careful neurological evaluation. A
request for a “screening” study as if the EMG can basic tenet in electrodiagnostic medicine is that
be equated to an electrocardiogram which may the “EMG is an extension of the neurological
be performed to “see what is found”. General examination”, not a replacement for it.
statements such as limb pain, or the “rule out” Unfortunately, the latter portion of this is often
overlooked by referring providers. As Asa
Wilbourn admonished us “…the procedure is ill
N. Galvez-Jimenez (*) ∙ A. Soriano suited to routine screening for neurologic dis-
Braathen Neurological Center, Cleveland Clinic ease. A total body EMG would require hours to
Florida, Weston, FL, USA performed, be prohibitively expensive, and cause
Cleveland Clinic Lerner College of Medicine of Case intolerable patient discomfort” [2].
Western Reserve University, Cleveland, OH, USA
e-mail: galvezn@ccf.org; soriana@ccf.org
J. A. Morren
Neuromuscular Center, Neurological Institute,
Cleveland Clinic, and Cleveland Clinic Lerner
College of Medicine of Case Western Reserve
e-mail: morrenj@ccf.org
© Springer Nature Switzerland AG 2021 1

N. Galvez-Jimenez et al. (eds.), Electrodiagnostic Medicine,
https://doi.org/10.1007/978-3-030-74997-2_1
2 N. Galvez-Jimenez et al.
Currently the preferred and accepted term is to exclude a widespread process such as motor
Electrodiagnostic (EDX) studies, which encom- neuron disease (MND). The initial reason for the
passes two main groups of techniques, namely study was foot drop however the initial findings
(a) nerve conduction studies (NCS) which may suggested a more widespread and severe disorder
include F-wave and H-reflex responses, and other and the time spent and studies done may need to
special studies such as repetitive nerve stimula- be adjusted accordingly. Therefore, it can be
tion (RNS), and (b) needle electromyography inferred from the above that the purpose of the
(EMG) recording directly from muscle [alterna- EDX study is not only to confirm the presence of
tively referred to as needle electrode examination a peripheral disorder but more importantly to
(NEE)]. A highly specialized separate technique localize where the lesion is (e.g. anterior horn
called single fiber EMG (SFEMG) falls into the cell, root, plexus, peripheral nerve/nerves, neuro-
latter category. The various components of elec- muscular junction, or muscle) and the underlying
trodiagnostic studies may provide information pathophysiological process, (e.g. demyelinating
about the peripheral sensory and/or motor nerve and/or axonal neuropathy, neuromuscular junc-
fibers (including anterior horn cells), as well as tion transmission defect, myopathy), which in
the neuromuscular junction and muscle (see turn will lead to a differential diagnosis that will
Table 1.1). help cone down the diagnostic possibilities to a
EDX studies provide an objective evaluation few targeted ones, if not one in particular. If the
of a limited portion of the peripheral nervous treating physician is uncertain about the diagno-
system, specifically the peripheral portion of sis, the EDX study may help clarify the nature of
some sensory fibers and the motor unit the patient’s symptoms, although when ordered
(Fig. 1.1), which includes the anterior horn cell/ without proper indication—may lead to more
motor neuron, the neuromuscular junction and confusion and unnecessary further studies (par-
all the muscle fibers innervated by the same ticularly if the EDX studies are not done prop-
motor neuron [2]. erly). Therefore, as Campbell [3] emphasized
The study must be tailored to the patient “… There can be no serious argument that elec-
symptoms and neurological findings and be tromyography, in all the ramifications of that
adjusted as the procedure progresses and new term, is the practice of medicine … Extensive
findings begin to accrue. For example, a patient background knowledge is required to perform
who is sent for foot drop may be found to have and accurately interpret electrodiagnostic stud-
evidence of abundant active/ongoing axonal loss ies. The necessary preparation spans electronics,
in many myotomes, and this may require extend- biomedical engineering, basic neurophysiology,
ing the NCS and the EMG to other limbs, para- anatomy, neuromuscular pathology, clinical and
spinal muscles and/or cranial innervated muscles musculoskeletal disease, the technical aspects of
performing nerve conduction studies and needle
electromyography, and, most importantly, the
Table 1.1 Types of basic electrodiagnostic examinations correlations between electrodiagnostic findings
and peripheral nerve fibers assessed and clinical disease.”
Motor Motor NCS, needle electrode
examination, F-wave responses,
RNS (NMJ study), SFEMG
(NMJ study) Biases, Advantages
Sensory Sensory NCS and Disadvantages/Limitations (see
Mixed (Motor and Mixed nerve NCS (e.g. palmar Tables 1.2, 1.3 and 1.4)
Sensory) and plantar mixed nerve
responses), H-reflexes
To clearly understand and interpret NCS, we
NCS nerve conduction studies, RNS repetitive nerve stim-
believe understanding the biases, advantages and
ulation, SFEMG single-fiber electromyography, NMJ
neuromuscular junction limitations are important before we delve into the
1 Principles of Electrodiagnosis: Introduction 3
Fig. 1.1 Illustration of

the anatomical
components of a motor
unit: an anterior horn
cell/motor neuron and
all of the muscle fibers
(muscle cells) it
innervates
Motor neuron
(spinal nerve)
Spinal cord
Axon
Muscle fibers
actual NCS. The pathophysiology and physiolog- S1 when recording sural responses (and/or L5
ical basis for clinical EDX practice should be when recording the superficial peroneal (fibu-
understood. The diagnostic value of EDX studies lar) sensory response) in the lower extremi-
may be limited, and at times severely so in some ties. These are mixed nerves (except the sural
patients, particularly those with many co- nerve) and the sensory and motor abnormali-
morbidities such as diabetes mellitus, chronic ties may reflect axonal, demyelinating, neuro-
renal and/or liver failure, prior multiple lumbosa- muscular and myopathic involvement alone or
cral surgeries, limb edema and many others alone in combination, therefore the physician must
or in combination. have special knowledge of peripheral neuro-
muscular anatomy, understanding and recog-
1. When performing and analyzing NCS, the nizing disorders that affect the nerves, muscles
physician must keep in mind that upper and and neuromuscular junction, and the observ-
lower extremity NCS are biased towards able changes in both the normal and diseased
assessing the distal limb nerves and muscles states. Consequently, abnormalities observed
and also towards the C8/T1 and L5/S1 distri- in such studies may be reflecting local muscle,
butions. This is also manifested by the median NMJ, or distal neuropathic process but also
motor and ulnar motor responses for the rou- may be reflecting more proximal alterations
tine motor NCS of the upper extremity, and affecting the root and anterior horn segment.
the tibial motor and peroneal (fibular) motor The sensory NCS are extremely useful
responses for the routine motor NCS of the in localizing the lesion either distal to or prox-
lower extremities. From a dermatome per- imal to the dorsal root ganglia (DRG)-
spective, often covered is C6 and C8 when practically defining the intraspinal localization
routinely performing upper limb sensory of the process when the sensory responses are
NCS: median sensory recording index finger present. Such findings are pivotal when plan-
and ulnar sensory recording fifth finger, and ning the NEE. Abnormalities of sensory
Table 1.2 Biases, advantages and disadvantages of EDX Table 1.3 Limitations of nerve conduction studies due to
studies underlying patient conditions (modified from [4])
Biases Limitations Results
Routine NCS studies of the upper and lower Unsedated child Poor testing; limited
extremities are biased towards assessing the distal Confused/Uncooperative study
limbs via nerves subserved by the C8/T1 and L5/S1 patient (e.g.: dementia,
anterior horn/root segments, and the C6 and C8 and S1 encephalopathy)
(+/−L5) sensory responses respectively Edema (e.g.: lymphedema Low amplitude sensory
Advantages due to axillary node and motor responses or,
Nerve conduction studies dissection in the context of if severe, unelicitable
Require only “passive” patient cooperation breast cancer, Congestive responses
Usually, particularly when performing routine studies, Heart Failure, others)
produces relatively little or minor patient discomfort Cool/cold extremities Prolonged distal
Permit the evaluation of some sensory fibers latencies, slow
Provides information regarding the state of conduction velocities
myelination of motor and sensory fibers and high amplitudes
Very useful in detecting demyelinating block/ Excessive sweating Artifact and/or
segmental demyelination and its localization. inadequate responses.
Needle electrode examination Poor electrodes
Allows for a flexible and widespread motor adherence to skin with
assessment of the peripheral nervous system, and consequent poor contact
muscle disease Skin lesions (e.g.: rashes, Inability to test the limb,
Sensitive for detecting motor axonal loss infections, hematomas, or to reliably stimulate/
Sensitive for localizing a lesion producing motor skin breakdown) record from interest site
axonal loss Central line, implanted Cannot stimulate near
Sensitive for detecting primary muscle disease (when pacemakers or the site, near wires or
appropriately performed). defibrillators, Deep Brain pacer due to consequent
Disadvantages Stimulator (DBS) chest transmission of
Nerve conduction studies generator stimulation to heart (or
Evaluates only a limited portion of the peripheral brain, in case of DBS)
nervous system Anterior neck swelling or Cannot achieve
Does not assess small sensory fibers (those involved in thickness supra-maximal
small fiber neuropathy) stimulation (e.g.: in
Are relatively insensitive to axon loss, particularly that brachial plexus, phrenic
affecting motor fibers nerve studies)
Relatively insensitive for detecting primary muscle
disorders
Concentric/monopolar needle electrode examination
Requires active patient cooperation latency, amplitude or absence of sensory
Patients may find it difficult to tolerate and a few may responses localizes the lesion to a process dis-
not complete the study tal to the DRG.
Does not evaluate sensory fibers therefore it 2. Age affects the NCS and EMG. For exam-
demonstrates motor axonal loss only
May be confusing/difficult to interpret when ple: it has been said, however somewhat
concomitant myopathy exists challenged by some recent data and our own
Does not sensitively evaluate demyelinating segmental experience, that after age 60 years, sensory
loss along motor fibers. responses of the lower extremities (sural and
May be affected by temperature changes as well: e.g.
features of active/ongoing axonal loss such as superficial peroneal (fibular)) may be absent
fibrillation potentials may disappear if limbs are cold. as a normal physiological finding, making
In monopolar needle studies, the current criteria for the assessment for a disorder distal to the
MUP analysis are different and must be kept in mind dorsal root ganglia such as a neuropathy
when using such an electrode; must have a ground and
reference electrodes connected directly to the patient. challenging. Therefore, particularly with
lower extremity disease in those above
Table 1.4 Limitations of needle electromyography due distance from the below-elbow to wrist site
to underlying patient conditions
may result in spuriously fast motor conduc-
Limitations Results tion velocities. Similar errors may be incurred
Bleeding disorders, Concerns about deep-seated in ulnar studies when performed with the arm
Use of anticoagulants, bleeding particularly
including Novel Oral intramuscularly in large extended at the elbow rather than with elbow
AntiCoagulants muscles, and in deep muscles flexion at 90 degrees, particularly when
(NOACS) (e.g.: iliacus, quadriceps), assessing velocity slowing or conduction
skin and subcutaneous block between the above-elbow and below-
hematomas
elbow sites (elbow segment).
Coma or drug induced Patient unable to participate/
sedation follow commands, with 4. Other factors that affect the reliability of the
subsequent inability to data obtained and must be keep in mind when
properly analyze recruitment performing NCS, include submaximal stim-
or MUP morphology
ulation or excessive stimulation with con-
Agitation or inability Unable to reliably assess
to relax on command insertional and/or sequent volume conduction, spread to
spontaneous activity. contiguous or nearby nerves, and electrical
Increased risk of an interference (which we experience more
accidental needle stick to the commonly in the intensive care unit, or near
examiner
radiological suites when the electrical cables/
ICU conditions such Uncooperative, or unable to
as intubated or position patient for, or wall outlets have not been properly isolated/
restrained patients participate in the study. conditioned). This “electrical noise” is quite
Unable to easily assess a problem in many areas particularly in the
paraspinal and nearby
inpatient hospital setting, making the record-
muscles
ing of responses quite challenging at times.
This is often most problematic when per-
forming sensory nerve conduction studies
60 years, what may be normal or abnormal and concentric needle electromyography in
may merge, making the EDX findings less the ICU. Other important factors include the
specific [2, 5], and this must be acknowl- correct placement of the stimulator, as
edged by the interpreting physician. Of note, well as the recording and reference elec-
tibial H-reflexes may also be absent as a nor- trodes. This may result in abnormal mor-
mal age process after 60 years. However, it phology of responses obtained, and/or
may also be the only manifestation of an S1 abnormal latencies and/or conduction
radiculopathy or a proximal demyelinating velocities.
block [3, 6, 7].
3. It is of utmost importance to pay attention to
detail when performing NCS as any deviation
of the norm or external and internal (patient) Physiopathological Basis
factors may affect the results. Temperature, for the Interpretation of NCS
distance measurements, and presence of
limb edema may all affect the results. Cold Details will be provided in the respective chap-
limbs notoriously lead to widespread distal ters (vide infra), however, some introductory
latency prolongation, conduction velocity remarks are important to have a basic foundation
slowing and abnormally high amplitudes. when interpreting these studies.
Pedal edema may result in abnormally low or Firstly, the peripheral nerves are composed of
absent sensory responses. Flawed measure- many individual nerve fibers or axons varying in
ments, such as an erroneously short measured size from 0.5 to 22 microns, surrounded by con-
nective tissue, which is a major component of the emia, and exposure to toxins including medica-
peripherals nerves, providing the support or tions (e.g. colchicine), particularly
“skeleton” for the axons and Schwann cells. chemotherapeutic agents and antibiotics (e.g.:
Another important function of the connective tis- metronidazole). Despite these varied causes the
sue is to provide protection and nutrition for the major responses to injury are axonal loss,
enclosed nerves. It is important to recognize that, demyelination, or a combination of both [5, 7].
comparatively speaking, the area occupied by Axon loss may result in conduction failure dis-
fascicles, epineurium and perineurium varies tally from the site of the injury, whereas demy-
from nerve to nerve. This is important as some elination—usually a segment or a region of
nerves or fascicles are prone to damage selec- focal demyelination, may result in conduction
tively depending on the location of the lesion (as slowing or (when severe enough) conduction
may be seen with pressure-susceptible superficial block, as seen with neurapraxia. An example
areas). For example, in some cases of ulnar neu- of this will be carpal tunnel syndrome, with the
ropathy at the elbow, the fibers to the abductor main underlying pathophysiological process
digiti minimi may be spared while those for the being focal demyelination resulting in pro-
first dorsal interossei may be preferentially longed distal latencies (sensory followed by
affected. Kline [8] has reported what was sup- alteration in the motor latencies); similar also
ported by earlier data from Sydney Sunderland seen in other compressive neuropathies such as
[9] that 85% of the cross sectional area of the sci- ulnar neuropathy at the elbow and/or wrist, also
atic nerve at the level of the hip is connective tis- acute or chronic demyelinating polyneuropa-
sue, for example. thies. In other conditions, particularly chronic
The epineurium is the outer layer covering exposure to toxic/metabolic processes such as
the nerve composed of collagen and elastic fibers diabetes mellitus, there may be both axonal and
with abundant epineural vessels longitudinally segmental demyelination. In axonotmesis,
oriented with small penetrators forming a mesh there is loss of the continuity of the axon with
or network communicating with the perineurium preservation of the connective tissue including
and endoneurium. The perineurium is that con- endoneurium surrounding the axons. With axo-
nective tissue encircling the nerve fascicles. The nal loss, there is a “dying back” or Wallerian
perineurium is the peripheral nerve equivalent degeneration, which, on average takes about
of the blood-brain barrier (blood-nerve barrier) 3 weeks to manifest typical changes during the
due to the presence of tight junctions with same needle electrode examination, consisting of
function as those noted in the central nervous fibrillation and positive sharp wave potentials.
system. Damage to the perineurium may result in These may be preceded by few days with an
axonal loss and demyelination within the fasci- increase in insertional activity—however, this
cle. Finally the endoneurium encircles each is a nonspecific finding. In the chronic phase of
myelinated axon and groups of unmyelinated and significant axon loss nerve injury, there will be
thinly/poorly myelinated axons. The endoneu- muscle atrophy, in addition to muscle weak-
rium serves as the last blood-nerve barrier, is ness. In these cases, there is typically loss of
composed of micro vessels, tight junctions and both motor and sensory function, unless the
collagen fibers. nerve is a pure sensory one (e.g. lateral femoral
Schwann cells, similar to the oligodendro- cutaneous nerve). It is important to recognize
cytes in the CNS, forms myelin coverings around that axonotmesis prognosis is distance—depen-
axons with the plasma membrane of each dent as there may be good recovery from axo-
Schwann cell wrapping around and fusing with nal re-growth if the target muscle distance to
itself forming a thick myelin sheath [7–10]. the lesion is less than approximately 20 inches
The peripheral nervous system has a limited [7, 11]. Neurotmesis is the most severe form of
repertoire to injury [7, 10]. Common forms of injury when the whole structure/continuity of
injury include compression, stretching, isch- the nerve is lost, either because of severe nerve
transection, or crushing injuries. In many When performing motor NCS, one must keep in
instances, there may be proximal axonal growth mind that the responses reflect the time it takes for
but once advancing nerve fibers reach the the stimulus to travel to the recording electrode,
stump, they fail to find their way along the orig- which is placed on the belly of the muscle where the
inal nerve course, with no recovery of function. motor point is located. Hence, motor latency: the
Consequently, there will be permanent muscle time it takes for the stimulation to travel from the
weakness and atrophy, unless there is success- stimulus artifact to the onset of the motor response
ful surgical re-anastomosis [2, 5, 9, 12]. (CMAP), is measured in milliseconds. This param-
eter reflects the time taken to travel along the fast-
conducting large myelinated fibers (abundant
Nerve Conduction Studies myelin sheath and axons), the neuromuscular junc-
tion and that needed for the activation of the muscle
During the performance of nerve conduction fibers and resultant muscle contraction. These must
studies we are measuring electrical responses be kept in mind when interpreting the motor NCS
elicited by stimulating a peripheral nerve, record- responses elicited within the specific clinical con-
ing from surface electrodes, or rarely needle elec- text of each case. Without a good neuromuscular
trodes recording from a muscle or sensory fibers. history and examination, the data may be more dif-
Those recorded from the muscle constitute motor ficult to interpret. The sensory NCS latency reflects
nerve conduction studies, and those from the sen- the time it takes the stimulus to travel via fast-
sory fibers are the sensory nerve conduction stud- conducting sensory fibers to the sensory response
ies. The motor responses are the summation of recording point. The sensory latency is measured
the electrical potentials generated by a large from the onset of the stimulus artifact to the nega-
number of muscle fibers resulting in the com- tive peak and is reported in milliseconds as well.
pound muscle action potential (CMAP) and are Specific parameters constituting a typical motor and
measure in millivolts, whereas sensory fibers sensory nerve conduction response are shown in
responses represent the summation of the direct Figs. 1.2 and 1.3 respectively.
response from the sensory fibers, which are quite Amplitude and duration of the compound
small and are measured in microvolts, and are muscle action potential (CMAP) represent the
called sensory nerve action potentials (SNAPs). number of axons and connected muscle fibers in
Because of its size, the sensory fiber responses the motor response, along with the degree of syn-
are smaller and therefore, are more difficult to chronization/desynchronization of the different
obtain, are usually affected first in a generalized fibers conduction times. This latter point is better
peripheral neuropathic process, are prone for arti- assessed by the area under the curve (AUC), and
factual effects and technical difficulties related to looking at the configuration of the CMAP, par-
patient characteristics such as limb edema. In this ticularly when there is low amplitude and disper-
latter scenario for example, the distance from sion of the motor response (discordant increase
surface electrode to nerve is increased by the in CMAP duration). The AUC is routinely calcu-
interstitial fluid, with increased electrical resis- lated by the computerized software in most NCS/
tance and high frequency filtering effects due to EMG equipment. Amplitude of the CMAP is
accentuation of connective tissue making the additionally affected by disorders of the neuro-
recording or eliciting of a reliable sensory muscular junction as exemplified by the low
response quite challenging. These challenges motor responses observed in patients with
often converge in the ICU patient who may have Lambert-Eaton myasthenic syndrome.
limb edema and/or trophic skin changes for a In our EMG laboratory, we routinely test
variety of reasons, with the sensory (more than median motor nerve responses recording from
motor) nerve conduction responses further the abductor pollicis brevis (APB) muscle, ulnar
affected by electrical “noise” in the critical care motor nerve responses recording from the abduc-
environment. tor digiti minimi (ADM) muscle, tibial nerve
Fig. 1.2 Outline of a

typical motor nerve
conduction response
showing component
parameters
Amplitude
Area
Latency Duration
responses include median sensory recording

index finger, ulnar sensory recording fifth finger,
radial sensory recording from the base of the
thumb, sural responses recording lateral leg/lat-
eral malleolus and superficial peroneal (fibular)
recording lateral leg/dorsal aspect of the ankle.
Duration (negative phase)
Depending on the clinical situation, other less
commonly performed studies are added as needed
including medial and lateral antebrachial cutane-
P
ous, median sensory recording thumb and middle
finger, dorsal ulnar recording dorsum of hand),
saphenous recording lower inner leg, axillary
Baseline to peak amplitude
nerve recording the deltoid muscle, musculocuta-
neous nerve recording from the biceps brachii
Peak to peak amplitude muscle, radial motor nerve recording from the
extensor digitorum communis (EDC) muscle,
and in the lower extremities femoral nerve
Onset latency recording from the rectus femoris (or vastus late-
ralis) muscle, peroneal (fibular) motor nerve
recording from the tibialis anterior (TA) muscle
Peak latency
(this is particularly important in patients with
foot drop), ulnar motor nerve recording from the
Fig. 1.3 Outline of a typical sensory nerve conduction first dorsal interosseous (FDI) muscle (particu-
response showing component parameters larly important in patients suspected of an ulnar
neuropathy at the elbow due to preferential fas-
motor responses recording from the abductor hal- cicular involvement in some cases sparing the
lucis (AH) muscle, and peroneal (fibular) motor ADM, however involving the FDI fibers).
nerve response recording from the extensor digi- Other studies include palmar and plantar
torum brevis (EDB) muscle. Routine sensory mixed nerve responses. The former are per-
Table 1.5 Nerve conduction studies Table 1.5 (continued)

Motor Nerve Conduction studies Sensory Nerve Conduction studies
Routine or standard motor NCS in our EMG Routine or standard sensory NCS in our EMG
laboratory laboratory
1. Median motor: recording abductor pollicis brevis 1. Median: recording index finger
(APB) 2. Ulnar: recording fifth finger
2. Ulnar motor: recording abductor digiti minimi 3. Radial: recording thumb base
(ADM) 4. Sural: recording lateral ankle
3. Tibial motor: recording abductor hallucis (AH) Other sensory NCS frequently performed
4. Peroneal (fibular) motor: recording extensor 1. Median/ulnar palmar mixed nerve responses
digitorum brevis (EDB) (performed almost exclusively for the diagnosis of
Frequently performed motor NCS in our EMG carpal tunnel syndrome or ulnar neuropathy at the
laboratory wrist/Guyon’s canal).
1. Ulnar motor: recording first dorsal interosseous 2. Lateral and Medial antebrachial cutaneous
(usually performed when an ulnar neuropathy at the responses (usually performed when a brachial plexus
elbow is suspected, in addition to the ulnar motor lesions is suspected).
response recording ADM). 3. Superficial peroneal (fibular) sensory responses
2. Radial motor: recording extensor indicis or extensor (usually performed in cases of suspected common
digitorum (usually performed when a radial peroneal (fibular) neuropathy, as may occur from
neuropathy at the spiral groove is suspected). compression at the fibular head)
3. Musculocutaneous motor: recording biceps brachii 4. Medial plantar mixed nerve responses (usually
(done in cases involving proximal upper extremity performed when distal/early generalized large fiber
weakness/brachial plexus lesion). sensorimotor neuropathy or tarsal tunnel syndrome is
4. Axillary motor: recording deltoid: (done in cases suspected).
involving proximal upper extremity weakness/brachial Sensory NCS difficult to elicit and less commonly
Plexus lesion). performed in our laboratory
5. Peroneal (fibular) motor: recording tibialis anterior 1. Saphenous responses (may consider in lumbar
(TA) (usually performed when a peroneal (fibular) plexopathy work-ups)
neuropathy at the fibular head is suspected, for 2. Lateral femoral cutaneous (when meralgia
example in patients with foot drop). paresthetica or lateral femoral cutaneous neuropathy is
6. Femoral motor: recording rectus femoris or vastus suspected. However, this response is very difficult to
lateralis (done in cases involving quadriceps weakness obtain, particularly in patients with increased
when a femoral neuropathy or lumbar plexus lesion is abdominal girth).
suspected). 3. Posterior cutaneous nerve of the thigh.
tear drop appearance [13, 14]. When the electri-

formed almost exclusively when looking for car- cal potential is generated distally to the location
pal tunnel syndrome, and the latter—particularly of the needle electrode, the potential may have a
the medial plantar response is done when looking triphasic configuration. If the origin of the elec-
for a very early generalized large fiber sensorim- trical potentials is right at the needle placement
otor neuropathy or tarsal tunnel syndrome. These area, the configuration may be that of a positive
mixed responses evoke both motor and sensory wave or biphasic wave as the needle electrode
fibers resulting in larger amplitude responses “do not see” the incoming potential [13].
when compared to the pure sensory nerve However, it must be kept in mind that according
responses (Table 1.5). to Kimura [15], the spatial relationship between
the recording needle electrode and individual
muscle fibers play the most important role in
Needle Electrode Examination determining the waveform configuration. Other
factors affecting the Motor Unit Action Potential
When performing needle electromyography the (MUAP) configuration during EMG are tempera-
area “visualized” by the electrode is of about ture (cool limbs increases the duration of the
120–300 microns, usually assessing 5–15 muscle MUP with decreased in amplitude). Motor unit
fibers at a time. In the case of the concentric nee- potentials may become polyphasic in cooler
dle electromyography, the area examined has a limbs. According to Kimura [15] and supported
by Dumitru [13], the changes in such MUAP fea- ing pattern. Usually the MAUP has a triphasic
tures are due to the effect of temperature result- configuration mimicking that observed in the
ing in accentuated differential slowing and EKG QRS complex, having an amplitude between
desynchronization of muscle fiber activation. 120 microvolts to 2 to 3 millivolts and a maximal
When performing the NEE, the examiner first duration of approximately 15 milliseconds in
notices the insertional activity and the resistance to duration with a rise time, (measure from the first
needle insertion. In cases of fibrotic muscles, such positive peak to the subsequent negative peak), of
as some longstanding, there may be a gritty or less than 100 to 200 microseconds. This results in
sand-like sensation when inserting the electrode. a crisp, sharp sound providing the best indicator
In cases of steroid myopathies the insertion may to the proximity of the recording needle tip to the
feel as if inserting the needle in butter with mini- muscle fiber. Of course, the duration and ampli-
mal resistance. Once the needle is inserted there tude depends on the muscle studied. Larger mus-
will be a very short insertional activity that may be cles tend to have larger MUAP, and small muscles
increased in cases of muscle membrane instability/ such as the orbicularis oculi tend to have a small
irritability such as active/ongoing motor axon loss MUAP. A motor unit is considered polyphasic
or myopathic states. After insertional activity, with once more than three crossings over the baseline
the needle remaining motionless in the relaxed are present, a phase being the portion of the wave-
normal muscle, there should only be electrical form between the departure from and return to the
silence constituting the normal baseline muscle baseline. Polyphasic MUAP are markers of chro-
response. With denervation and/or muscle fiber nicity and usually represent the desynchroniza-
destruction, fibrillation potentials with the config- tion of muscle fiber activation due to sprouting
uration of positive sharp waves or biphasic spikes when a muscle has been denervated or there is
responses may be observed on average about 3 destruction/drop-off of muscle fibers. Such poly-
weeks after the initial insult/injury, with these phasic MUAP are observed in about 10–15% of
denervation potentials lasting typically up to MUAP in many muscles, particularly proximal
8–10 months (or may persist considerably longer). larger muscles such as the iliacus, gluteus muscle
The presence of positive sharp wave or fibrillation groups, brachioradialis, supraspinatus, and del-
potentials defines the subacute stage of axonal toid. If the turns do not cross the baseline they are
loss. Fibrillation potentials have an almost con- serrated MUAP with the same EDX significance.
stant discharge frequency, simulating the tick-tock That is, axonal sprouting with reinnervation con-
of a clock (sometimes described as “metronomic”). sistent with chronic changes. Acute/subacute and
The amplitude of such responses may be between chronic electromyographic changes commonly
100 and 400 microvolts but may also be somewhat co-exist in the same muscle when studied.
larger or smaller. The fibrillation potentials can be Recruitment of motor units is another important
conceptualized as representing the pacemaker aspect during the performance of the NEE. Once a
activity intrinsic to each muscle fiber, which, when MUAP has been identified at the beginning of acti-
devoid of the inhibitory tonic influence of the vation, the firing frequency is noted, together with
motor axons, begin to fire independently. Hence, the recruitment ratio which is usually observed
each fiber begin to fire on its own, resulting in the once the MUAP achieves a 10–15 Hz and a second
busy screen that may be observed in such situa- MUAP is recruited and begin to fire (recruitment
tions. Other types of abnormal insertional activity ratio = firing frequency of the fastest firing MUAP/
include myotonic potentials, complex repetitive the number of different MUAPs on the screen).
discharges (CRDs), and myokymia (among other This is considered normal recruitment. If the mus-
grouped repetitive discharges), which will be dis- cle increases its force of contraction, a third MUAP
cussed in subsequent chapters. is recruited when the first MUAP achieves ~15 Hz
Once analysis of the insertional activity has and the second MUP achieves ~10 Hz, and so forth.
been performed, the next step is to evaluate the This is known as the 5:1 recruitment ratio or the
motor unit action potential configuration and fir- “rule of 5’s” [6, 13].
Late Responses These are the F-wave, and H-reflex, and the
A-wave. The M response in the context of these
Routine NCS are biased towards the assessment late responses is essentially the routinely recorded
of the most distal portions of the peripheral compound muscle action potential during NCS.
nerves. In the upper extremities, when recording
from the hand and distal forearm, the sensory and
motor responses obtained are those from the dis- F-Wave (Fig. 1.4)
tal portions of the median, ulnar and radial nerve
territories. In the lower extremities, the NCS The F wave is a pure motor response. To generate
often assess the responses from the distal compo- an F-wave response, a supramaximal stimulation
nents of the sciatic nerve below the knee namely is delivered to the nerve, and such a response will
the peroneal (fibular) and tibial nerves. They do be observed after the direct M response/recorded
not assess the more proximal segments of these CMAP during NCS. These responses are useful
nerve with the stimulation and recording sites as a measure of the proximal conduction time and
employed. motor axon responses, which are elicited anti-
However, there are other responses which may dromically, while recording orthodromically
be obtained to assess the more proximal segment most often from the median, ulnar and tibial
of peripheral nerves. With the appropriate tech- innervated muscles. In other words, the afferent/
nique, three different types of responses may be efferent pathway is within the motor fibers of the
observed following the CMAP or M responses anterior horn cell. The motor impulse travels up
recorded from the muscle during NCS. Hence, centripetally (antidromically) along the motor
they are collectively known as “late” responses. neurons, reaching the cell bodies and backfiring

the anatomical
components and
electrical pathway of the
tibial F-wave response
via the orthodromic efferent motor axon path- in which an acute inflammatory demyelinating
way. There is no synapse involved, but this trans- polyradiculoneuropathy (AIDP, or Guillain-
mission incurs an estimated minimal lag time of Barre syndrome) is suspected. This may be the
1.0 ms at the spinal cord motor neuron pool. case in a patient presenting with weakness and
Therefore, the F-wave provides an assessment of areflexia, and yet normal motor and sensory rou-
the most proximal portion of the motor axons tine NCS (noting as well that often a “sural-
comprising the peripheral nerve under study. The sparing” pattern is seen).
fibers assessed are the same fast conducting fibers It is useful to calculate the F-wave estimate,
stimulated during routine NCS. According to taking into account the patient’s height, which
Kimura, [15] this provides the rationale for the will influence the measured value of the F-wave
minimal F-wave latency serving as a measure of response (particularly, latency). Because the
the fastest conducting motor nerve fibers proxi- F-wave assesses the whole length of the nerve, it
mally. There is no sensory component therefore, is generally a very good measure of nerve pathol-
the F-wave response is not affected by the find- ogy. If the F-wave latencies are normal and
ings of the sensory NCS. When first described, within the range of the F-wave estimate, it is a
they were recorded from foot muscles, hence the good indication that the nerve fibers are conduct-
name “F”-waves. ing at the expected velocity, and this excludes a
The F-wave is generated by the random anti- significant pathological (especially demyelinat-
dromic activation of approximately 1% to 5% of ing) process within the length of the nerve.
the anterior horn cells (motor neuron) pool. However, if the measured minimal F-wave
Because of this, there is a need to obtain several response is prolonged when compared to the
responses. In our EDX laboratory we obtain a F-wave estimate, this implies a conduction delay
train of 10 responses, using the one which has the in the more proximal nerve segment, likely out of
shortest latency as the best response (i.e. “mini- proportion to what would be expected based on
mal” F-wave latency). There should be a greater the distal motor latency and other findings on
than 50% F-wave occurrence rate to be consid- routine motor nerve conduction studies. We esti-
ered normal and, on average, 70% to 80% F-wave mate the F-wave latency by measuring the length
occurrence rate is what we usually observe in our of the arm from the stimulation site to the sterno-
laboratory among normal subjects. Because the clavicular joint for the upper extremity, and from
amplitude of the F-wave is typically 1% to 5% the site of stimulation to the xiphoid process for
that of the CMAP, if the CMAP is reduced, the the lower extremity. The F-wave latency estimate
F-wave will not be observed. Amplitude, latency can be calculated as follows:
and dispersion are variables that may provide
useful information when assessed in the appro- F [ est ] = ( 2 × distance ) / CV  + DL.
priate clinical context. Apart from its low ampli-
tude, a key feature that differentiates the F-wave In this formula, the distance is the measured
from the M response is its variable inter-response distance from the cathode to the sternoclavicular
latency. Characteristically, the F-wave latency joint and xyphoid process for the upper and lower
varies from stimulation to stimulation. extremity, respectively. CV is the calculated con-
Chronodispersion (the difference between the duction velocity, and DL is the motor distal
shortest and longest F-wave latencies) is on aver- latency for the particular nerve under study. Most
age, about 4 ms in the upper extremities, and is current NCS/EMG equipment will calculate this
about 6 ms in the lower extremities. This param- value automatically if the measured distance is
eter provides an indication of proximal nerve entered. Furthermore, most current equipment
temporal dispersion. Prolongation of the F-wave will provide the FWCV. FWCV is the CV to and
latencies indicates proximal nerve segment con- from the spinal cord. The FWCV is another mea-
duction delay and it may be the only abnormal sure of the proximal segment conductivity, and
finding in an otherwise normal NCS in a patient accordingly, it is usually affected in disorders in
which there is proximal conduction slowing. Of gastrocnemius-soleus muscle complex. The ben-
note, there is a variable degree of agreement efits from such a study are the evaluation of the
among many EDX laboratories as to the ultimate proximal segment of the tibial/S1 nerve/nerve
usefulness of the F-waves. Utility of F-wave root. This response may be normally absent after
responses is less controversial in certain cases of age 60 years, and pathologically reduced or
nerve injury with early or predominant proximal absent with proximal processes such as demye-
nerve trunk and/or root-level pathology, as in linating polyradiculoneuropathies (e.g. AIDP),
early AIDP. In these cases, the F-wave may be and prior laminectomies involving the low lum-
significantly abnormal as an isolated but sensitive bar/lumbosacral/S1 region.
finding.
The A-wave or Axon Reflex

H-reflex (Fig. 1.5)
On occasion, an axon reflex or A-wave is recorded
The tibial H-reflex assesses the proximal portion when performing an F wave study. The A-wave is
of tibial nerve by orthodromically activating the a muscle response just like the CMAP but of
afferent Ia sensory fibers (bipolar cell—distal and lower amplitude and stable configuration and
proximal to the dorsal root ganglion) at the popli- latency as opposed to the F wave which is quite
teal fossa, stimulating the motor neurons in the variable in latency and configuration. The
anterior horn cells, with the efferent impulse trav- A-wave, when present, is always abnormal and is
eling orthodromically via the motor fibers to the usually observed in patients with radiculopathies
muscle, thereby eliciting a CMAP response at the or other proximal nerve/nerve root disorders

the anatomical
components and
electrical pathway of the
tibial H-reflex response
la afferent
Stimulator
Spinal cord
Alpha
motor neuron
Gastrocnemius
H-reflex
resulting in chronic axon loss. The origins of the nerve VII) efferents ultimately supplying the orbi-
A-wave are not certain. However, they may rep- cularis oculi to produce blinking (Fig. 1.6).
resent either “cross–talk” or ephaptic communi- The normal response results in two clearly
cation between injured axons proximally, or be defined components, ipsilateral orbicularis ocu-
the result of collateralization during axon lus M response labeled R1 and R2 (mediated pri-
sprouting. The A-wave is usually recorded in
marily by CrN V main sensory nucleus and spinal
between the M response and the F-wave. tract nucleus, respectively), and a contralateral
However, in some cases it may be recorded after R2 (R2C) response, mediated primarily by the
the F-wave. This later response has been attrib- CrN V spinal tract nucleus (Fig. 1.7).
uted to the ephaptic transmission mentioned These responses can be measured electro-
above due to demyelination-related conduction physiologically by stimulating the supraorbital
slowing through the collaterals. nerve [typical stimulus intensity of 8.0 mA, stim-
ulus duration 0.2 ms] resulting in an early R1
ipsilateral response with an average latency of
Blink Reflexes 10 ms [maximum 13 ms (8–13 ms)] and subse-
quent bilateral R2 responses of approximately
The neurophysiological correlate of the corneal 30 ms [maximum ipsilateral 33 ms (29–41 ms),
reflex is the blink reflex. The underlying polysyn- and maximum contralateral 34 ms (29–44 ms)].
aptic reflex arc is the basis of the blink reflex that The most reliable response is R1, which is a pon-
is measured in the EDX laboratory. Afferents tine reflex mediated by a disynaptic reflex arc
travel via the first (ophthalmic) division of the tri- formed by the V1 trigeminal afferent to the main
geminal nerve (V1) to the main sensory nucleus of sensory nucleus of CrN V, and the pontine
the trigeminal nerve in the mid-pons, as well as nucleus of CrN VII and corresponding facial
the spinal nucleus of this nerve (i.e. cranial nerve efferents. R2 responses, which are polysynaptic
V) in the medulla, connecting via interneurons to reflexes mediated via the reflex arc formed by the
the pontine facial nuclei, with facial nerve (cranial afferents coming from CrN V, bypassing the main
sensory nucleus and synapsing directly with the
spinal nucleus of CN V, then to bilateral pontine
V1 facial nuclei with efferents to the orbicularis
oculi. Therefore, these latter polysynaptic con-
nections make for a longer response onset (i.e.
longer latency), considering that R2 is a complex
pontine and lateral medullary reflex.
A side-to-side R1 latency difference exceeding
VM
1.2 ms is usually considered abnormal. The side-
VII VII to-side difference between the ipsilateral R2 laten-
cies and the contralateral R2 latencies should be
VII VII
less than 5 ms and 7 ms, respectively. The R2
responses may vary according to the state of
hyper-excitability of the inter-neurons, and can
VS
habituate upon repetitive stimulation. However,
R2 responses are very important for localization.
R1 abnormalities may be produced by disruption
in any of the components of the pontine arc:
namely the trigeminal afferents, facial efferents or
the corresponding pontine nuclei. Whereas with an
Fig. 1.6 Illustration of the anatomical components and abnormal or delayed R2, further localization may
electrical pathway of the blink reflex response be made, as an abnormality will help determine
Lt. Rt.
Amplifier
1 V Constant Stimulus
current isolation Stimulator
VII
3 2 unit unit
VII
Printer /
Oscilloscope /
Amplifier Electronic
Computer
storage
1. R1 2. Ipsilateral R2
Amplitude Average
Rt. Amplitude
Latency Duration
Latency
Average
Lt.
Amplitude
Stimulus 3. Contralateral R2
artifact
Fig. 1.7 Blink reflex-normal responses and measurements
whether an abnormal R1 is due to an afferent CrN five distinct branches including the temporal, zygo-
V or efferent CrN VII process. An ipsilateral and matic, buccal, marginal mandibular, and cervical
contralateral R2 delay, when the affected side is branches. Stimulating the facial nerve anterior to
stimulated, indicates a trigeminal nerve lesion. the mastoid process, slightly above the angle of the
This pattern is known as the “afferent delay”. With jaw over the stylomastoid foramina, recording
a lesion of the facial nerve, R2 is delayed on the from the nasalis muscle provides an objective
affected side regardless of the stimulation side/lat- recording of the state of the distal motor fibers.
erality. This is known as “efferent delay” pattern. Comparisons are made primarily in amplitude,
There are other potentially abnormal patterns, as with a greater than 50% difference in amplitude of
shown in the figure below (Fig. 1.8). However, the CMAP between sides being abnormal. Just as
using the principles outlined above, a discrete in routine NCS of the limbs, amplitude provides a
lesion localization can usually be made from the measure of the number of functional/intact motor
analysis of the R1, R2 and R2C responses. axons, and the motor latency provides a measure of
the fastest conducting fibers. In clinical practice,
we rely heavily on the side-to-side comparison for
Facial Nerve Motor Studies these responses. The latencies may be quite vari-
able between individuals and subject to false nega-
While the blink reflex assesses mostly brainstem tives even in cases of severe facial neuropathies,
components of CrN V afferents and CrN VII effer- particularly Bell’s palsy. Nonetheless, the reference
ents, the more distal fibers of CrN VII (that is, distal range for the facial nerve motor latency is generally
to the stylomastoid foramen), can be assessed by considered to be between 2.6 and 4.2 ms. Facial
stimulating the facial nerve as it exits the cranium nerve studies provide useful information about
to innervate facial muscles. The facial nerve has prognosis, particularly if repeated along the clinical
Table 1.6 Suggested standard assessment of the upper

Normal and lower extremities
Upper extremity
Nerve conduction studies
1 Vth nerve 1. Median motor recording Abductor pollicis brevis
(APB)
2. Ulnar motor recording Abductor digiti minimi
2 Vllth nerve (ADM)
3. Median sensory recording index finger
4. Ulnar sensory recording fifth finger
3 Main sensory 5. Radial sensory recording forearm
nucleus
6. Median and ulnar nerve F-wave responses
Needle electrode examination (NEE)
4 Spinal 1. Abductor pollicis brevis (APB) [C8/T1 roots,
nucleus median nerve, lower trunk/medial cord of brachial
plexus]
5 Uncrossed 2. First dorsal interosseous (FDI) [C8/T1 roots, ulnar
lnterneurons nerve, lower trunk/medial cord of brachial plexus]
3. Extensor indicis (EI) [C7, C8 roots, radial nerve,
5 Crossed middle and lower trunks/posterior cord of brachial
lnterneurons plexus]
4. Pronator teres (PT) [C6/C7 roots, median nerve,
upper and middle trunk/lateral cord of brachial plexus]
0.5 mV
Supraorbital nerve 5. Biceps brachii [C5/C6 roots, musculocutaneous
5 ms
nerve, upper trunk/lateral cord of brachial plexus]
Fig. 1.8 Most common abnormal response patterns 6. Triceps [C6, C7, C8 roots, radial nerve, upper,
observed during analysis of the blink reflex responses middle and lower trunks/posterior cord of brachial
plexus]
7. Deltoid [C5, C6 roots, axillary nerve, upper trunk/
course. A normal facial nerve CMAP response posterior cord of brachial plexus]
within the first week of injury may indicate a good 8. Cervical paraspinal muscles (if NEE of the upper
prognosis for recovery, whereas an abnormal extremity is normal otherwise, may not perform NEE
of the cervical paraspinal muscles at this stage)
CMAP response lasting beyond 3 weeks, particu- Lower extremity
larly if accompanied by signs of denervation on Nerve conduction studies
needle electromyography of facial muscles, por- 1. Tibial motor recording Abductor hallucis (AH)
tends a worse prognosis. A CMAP difference of 2. Peroneal (fibular) motor recording Extensor
90% between affected and unaffected sides sug- digitorum brevis (EDB)
gests a poorer prognosis. Additionally, a more 3. Sural responses [peroneal (fibular), tibial, sciatic,
lumbosacral plexus, and S1 root work-ups]
favorable prognosis may be predicted if the CMAP
4. Tibial H-reflex response
of the affected side is greater than 25% compared
5. Tibial F-wave response
to the unaffected side. Surgical decisions may be Concentric needle electromyography
based on the results of the facial EDX studies, in 1. Extensor digitorum brevis (EDB) [L5/S1 roots,
the context of the timeline of injury. deep peroneal (fibular) nerve, common peroneal
(fibular) nerve, sciatic nerve, lumbosacral plexus]
2. Abductor hallucis (AH) [S1 root, tibial nerve,
sciatic nerve, lumbosacral plexus]
Standard Assessments of the Upper 3. Tibialis anterior (TA) [L4/L5 roots, deep peroneal
and Lower Extremities (fibular) nerve, common peroneal (fibular) nerve,
sciatic nerve, lumbosacral plexus]
Although it should be emphasized that each EDX 4. Gastrocnemius medial head (S1/S2 roots, tibial
study should be tailored to the specific case, nerve, sciatic nerve, lumbosacral plexus)
informed by the history and exam findings and 5. Tibialis posterior OR flexor digitorum longus (either
one is acceptable) [L5 root, tibial nerve, sciatic nerve,
the resulting differential diagnosis, Table 1.6 lumbosacral plexus]
Table 1.6 (continued) disturbance may be distributed variably in the

6. Vastus lateralis [L(2)3/4 roots, femoral nerve, thumb, index of middle finger. In some, the whole
lumbar plexus] hand may be involved, and some patients report
7. Gluteus medius OR tensor fascia lata (either one is numbness/sensory disturbance or pain that
acceptable) [L5/S1 roots, superior gluteal nerve, sacral
plexus) spreads up to the forearm, sometimes even more
8. Gluteus maximus [S1(S2) > L5 roots, inferior proximally. On exam, the sensory perception at
gluteal nerve, sacral plexus] the thenar eminence must be spared as the sen-
9. Lumbar/sacral paraspinal muscles (if NEE of the sory innervation comes from the median palmar
lower extremity is normal otherwise, may not perform sensory branch which originates before (proxi-
NEE of the lumbar/sacral paraspinal muscles at this
stage). mal to) the carpal tunnel and it does not travel
within this structure.
Our suggested assessment of patients with
d iscloses the specific NCS and NEE studies that hand numbness/sensory disturbance/pain
are typically included in “standard” assessments includes the standard studies of median motor
of the upper and lower extremities. recording APB, ulnar motor recording abductor
digiti minimi, median sensory recording index
finger, ulnar sensory recording fifth finger. If
ome Common Scenarios in Which
S these studies are unremarkable, performing pal-
an Electrodiagnostic Study Is mar mixed nerve median and ulnar orthodromic
Requested studies may help increase the sensitivity as it per-
tains to the detection of a median mononeuropa-
Hand Pain/Numbness/Sensory thy at or distal to the wrist. In some laboratories,
Disturbance comparison studies using ulnar sensory responses
recording from the fourth finger, stimulating the
A common reason for an EDX evaluation in our ulnar nerve at the wrist may add some increased
laboratory is that of hand pain and/or numbness/ sensitivity when carpal tunnel (or ulnar neuropa-
sensory disturbance with or without associated thy at Guyon’s canal) is suspected. One may add
radicular symptoms. Carpal tunnel syndrome, a radial sensory study recording at the forearm as
ulnar neuropathy at the elbow or wrist, or radicu- it helps to exclude the presence of a polyneuropa-
lar symptoms with involvement of the C6 seg- thy presenting as numbness/sensory disturbance
ment (thumb), C7 (middle finger) and C8 (little in the hands particularly when the ulnar and
finger) dermatomes may all present with numb- median nerves studies demonstrate abnormali-
ness/sensory disturbance and/or pain in the hands ties. When a brachial plexus lesion is suspected
with or without referred pain to the forearm. we add sensory median studies recording thumb
Brachial plexopathies may also present with hand and middle finger in addition to the index finger,
numbness/sensory disturbance/pain and weak- and add medial and lateral antebrachial cutane-
ness (further discussed in the chapter on Brachial ous sensory studies, as well as axillary-deltoid
Plexopathies). For an in-depth discussion of and musculocutaneous-biceps responses when
mononeuropathies and radiculopathies, we also indicated. In CTS, the sensory latencies are first
refer the reader to the respective chapters later in affected due to involvement of the fast-conducting
this book. sensory fibers. Depending on the degree of sever-
In carpal tunnel syndrome (CTS), patients ity, prolongation of the sensory latency may be
often complain of hand numbness with or with- the only abnormality, followed by reduction in
out weakness affecting the thenar muscles (which the SNAP amplitude and prolongation of the
may undergo atrophy in severe cases). Sensory median motor distal latency recording
APB. When severe, there may be marked FDI may be preferentially affected at the elbow,
decrease or even absence of the median-APB sparing those destined to the abductor digiti min-
response. When only CTS in present, the ulnar imi. In patients with an ulnar neuropathy at the
nerve studies are normal. On needle electromy- elbow, the dorsal ulnar sensory response is usu-
ography, we routinely study the APB (median- ally affected along with the ulnar motor responses
T1 > C8-lower trunk/medial cord, first dorsal recording ADM and/or FDI. On needle electro-
interossei (FDI) [ulnar-C8/T1-lower t runk/ myography, we add to the standard EMG assess-
medial cord], extensor indices (EI) [radial- ment of the upper extremity, the flexor carpi
C8 > 7-lower and middle trunk/posterior cord], ulnaris, FDP-4th and 5th, in addition to the ADM
pronator teres [median-C6–C7-upper and middle and FDI. Of course, in the absence of an intraspi-
trunk/lateral cord], biceps brachii (musculocuta- nal canal process, the corresponding cervical
neous- C5–C6-upper trunk/lateral cord), triceps paraspinal muscles are normal in median and/or
(radial-C7(>C6,C8)-middle trunk/posterior ulnar mononeuropathies.
cord), deltoid (axillary-C5–C6-upper trunk/pos-
terior cord), and corresponding cervical paraspi-
nal muscles (posterior primary rami). Foot Pain/Numbness/Sensory
An ulnar neuropathy must be part of the dif- Disturbance
ferential diagnosis of patients with hand numb-
ness/sensory disturbance/pain and/or weakness. Another frequent diagnostic challenge is that of
The symptoms are often limited to the fourth and patients complaining of foot pain/sensory distur-
fifth fingers with possible radiation of symptoms bance/numbness. The pain character may be that
to the forearm. When assessing an ulnar neuropa- of burning, sharp stabbing, or electrical shocks
thy as the cause of such hand symptoms, an ulnar when walking, particularly when applying pres-
neuropathy at the elbow must be a foremost con- sure when walking to the ball of the foot. In most
sideration. With this localization, sensory instances, the patient clinical history supports that
responses at the hypothenar eminence and dorsal of a distal neuropathy particularly in the context
hand are typically reduced (dorsal sensory branch of diabetes mellitus, glucose intolerance, expo-
originates in the distal 1/3 of the forearm before sure to chemotherapeutic agents, or other medica-
entering Guyon’s canal). There may be atrophy tions (among other possible causes). If burning
of the hypothenar muscles and the hand interos- pain is present a small fiber neuropathy must be
sei resulting in a claw-appearing hand in many considered and in pure small fiber cases the EDX
cases, with marked bony prominence of the studies are usually normal. However, when a large
metacarpal bones and phalanges. Motor axon fiber neuropathy is present, other sensory modali-
loss changes in the abductor digiti minimi, first ties such as joint position sense and vibratory per-
dorsal interosseous, flexor digitorum profundus ception may be diminished with reduced/absent
(to digits 4&5), as well as flexor carpi ulnaris ankle reflexes (and possibly others, depending on
may be evident, depending on severity and/or the severity). Atrophy of the EDB and other distal
presence of fascicular sparing. foot muscles may be present. In these patients, the
As previously mentioned, radiculopathies of plantar mixed nerve responses may be absent as
the C7 and/or C8 involve the middle and/or the the first/earliest manifestation of a distal polyneu-
4th/5th fingers and may present with numbness/ ropathy. Caution should be applied when inter-
sensory disturbance/pain in the respective preting these studies as the sensory responses are
territories. of low amplitude and may be affected by many
In patients with suspected ulnar neuropathy at technical factors and patients characteristics such
the elbow, in addition to standard electrodiagnos- as age, thickness of the skin of the sole of the feet/
tic studies of the upper extremities, we add calluses, and leg/foot edema.
recording from the FDI stimulating ulnar, and Pain/sensory disturbance/numbness in the
also the dorsal ulnar sensory response. In many foot may be a result of any lesion along the L5/S1
cases of ulnar neuropathy, fibers destined to the root or segment, lumbosacral plexus, or sciatic,
peroneal (fibular) or tibial nerve pathway. be absent if an S1 root involvement is present).

Therefore, it is important to remember that all As Wilbourn stated, the NCS have the appear-
muscles below the knee are innervated via this ance of a severe axon loss lesion involving the
pathway, with the sciatic nerve dividing at the S1/S2 root segments [16]. However, if the tibial
upper angle of the popliteal fossa in its two main lesion is proximal to the contribution to the sural
components: the common peroneal (fibular) nerve the sural nerve response may be normal (or
nerve, and the tibial nerve. The sural nerve, a just slightly low in amplitude) due to the possibil-
pure sensory nerve, is composed of fibers coming ity of a relatively larger peroneal (fibular) contri-
from the common peroneal (fibular) and tibial bution. On NEE, all muscles supplied by the
nerves via the lateral sural cutaneous nerve tibial nerve are affected including the tibialis pos-
(LSCN) and the medial sural cutaneous nerve terior, flexor digitorum longus, AH and
(MSCN), respectively. ADQP. The more proximal muscles innervated
The two main PNS condition causing foot by the tibial nerve are spared including the gas-
pain/sensory disturbance/numbness in addition trocnemius, biceps femoris-long head, and other
to the polyneuropathies are the tarsal tunnel syn- S1-innervated muscles are also spared, namely
drome, and a main trunk tibial neuropathy. It gluteus maximus and biceps femoris-short head
must be kept in mind however, that more com- (peroneal (fibular)/S1 above the knee).
mon causes of foot pain are musculoskeletal
including tendinitis, plantar fasciitis, calcaneus
bone degenerative disease (also digital nerve Radiculopathies
neuromas), and the different causes of foot pain
are usually predictable based on the particular A common reason for electrodiagnostic consulta-
area affected, including the anterior, middle and tion in our laboratory is neck or low back pain
posterior foot regions. with radicular symptoms (defined as pain follow-
In tarsal tunnel syndrome, the EDX findings ing its dermatomal distribution). However, many
are those of abnormal medial/lateral plantar patients have pseudo-radicular symptoms due to
mixed nerve responses (particularly prolongation degenerative disease of the facet joints, discs and
of distal latency), with low CMAP in the distal other changes seen in spinal osteoarthritis, as well
tibial-supplied muscles of the lower extremities as narrowing of the lateral recesses and foramina,
including the abductor hallucis (AH) and abduc- which can present a challenge to the treating phy-
tor digiti quinti pedis (ADQP). NEE usually sician. Some patients may develop a radiculitis as
demonstrates findings of axon loss in the AH and a reaction to an infectious process such as herpes
ADQP. One has to be careful with the electrodi- zoster, borreliosis, sarcoidosis or Behcet’s dis-
agnosis of this condition given common con- ease. In those cases, an electrodiagnostic study is
founding factors: many patients will have either pivotal in the assessment of patients with sus-
reduced or absent medial/lateral plantar mixed pected radiculopathies. It may not only confirm
nerve responses due to local technical or patient- the presence of a radiculopathy, but the site or
related factors as aforementioned, and often there level(s) of the lesion/process, the extent/degree of
are “wear and tear” effects in the foot resulting in nerve damage, and the nature/pathophysiology of
(mostly chronic) motor axon loss changes in the the lesion, i.e.: axon loss vs demyelinating, or a
intrinsic foot muscles. Therefore, in our experi- combination of both. Furthermore, it may help
ence, despite many cases seen in our laboratory exclude a radiculopathy as the cause of the
for assessment of such a diagnosis, most com- patient’s symptoms and may help direct the treat-
monly, a distal polyneuropathy or an S1 radicu- ing physician to other possibilities or causes.
lopathy is found when there is abnormality. In It is important to recognize a basic tenet of
these cases, the EDX findings include normal lesion location when assessing radiculopathies
sural sensory responses (given the lesion is distal with nerve conduction studies. The sensory
to the tibial contribution to the sural nerve) and nerve conduction responses are typically
usually normal tibial-H reflexes (the H-reflex will intact in radiculopathies. The lesion or process
is intraspinal or at the intervertebral foramen, Again, it is important to keep in mind that

therefore the lesion is proximal to the dorsal root intrinsic foot muscles, particularly in those older
ganglia where the bipolar sensory neuron body than age 50 years, may disclose normal “wear
resides, resulting in normal sensory nerve con- and tear” and traumatic “physiologic injuries”.
duction responses. However, the patient may Accordingly, it is not infrequent to find a low/
complain of numbness, paresthesia or radicular absent response recording the AH or EDB when
pain, since the afferent pathway is still disrupted. stimulating the tibial or peroneal (fibular) nerves
A process distal to the dorsal root ganglia will respectively, particularly in the elderly. Therefore,
result in axon loss sequela with reduced or absent recording from the tibialis anterior muscle is rec-
sensory responses. ommended. It must be mentioned as well, record-
The motor nerve conduction studies may be ing from the tibialis anterior muscle while
normal in radiculopathies. However in advanced stimulating the peroneal (fibular) nerve is a must
disease the motor NCS may demonstrate in patient presenting with foot drop (vide infra).
decreased amplitude of the compound muscle Similarly, abnormalities during the NEE of such
action potential recording muscles within the muscles, namely EDB and AH must be inter-
affected myotome. preted with caution. In fact, in some laboratories
The motor conduction velocities may be nor- the AH and EDB are no longer examined in
mal or demonstrate mild slowing in keeping with patients older than 65 years of age.
axonal loss. NCS (sensory and motor) are further Although NEE abnormalities of the paraspinal
useful to exclude mimickers of radiculopathies muscles suggest a radiculopathy, it is a rather
such as peripheral polyneuropathies and non-specific finding in certain circumstances.
mononeuropathies. Paraspinal muscle NEE abnormalities may also
The spinal roots divide into the anterior and be seen compressive myelopathies, anterior horn
posterior primary rami once leaving the interver- cell disease, syringomyelia, polio/post-polio,
tebral foramen. The posterior primary rami inner- acute flaccid myelitis/myelopathy, spinal muscu-
vate the paraspinal muscles and other midline/ lar atrophy, arachnoiditis and other spinal sub-
truncal muscles and skin areas, and are very help- arachnoid infiltrative disorders such as meningeal
ful in demonstrating the presence of an intraspi- carcinomatosis, diabetes mellitus, porphyria,
nal lesion/process (e.g., when paraspinal muscles adult onset acid maltase deficiency, inflammatory
disclose active/ongoing axon loss changes). The myopathies, and as a sequela to laminectomy.
anterior primary rami are the contributors to the Specific NEE findings may include fibrillation
plexi, and peripheral nerves of the extremities. potentials, positive sharp waves, myotonic dis-
The needle electromyography is the single charges, complex repetitive discharges and fas-
most important technique to demonstrate, when ciculation potentials. On the other hand, the lack
recording directly from muscle, the involvement of abnormal NEE findings in the paraspinal mus-
of the motor fiber coming from the anterior horn cles does not exclude the presence of a motor
cell. However, it is necessary when performing the radiculopathy. Absent or abnormal sensory nerve
NEE to assess multiple muscles belonging to the conduction studies are expected when investigat-
same myotome (common nerve root innervation), ing plexus lesions (should disclose normal NEE
but of different peripheral nerve territories. findings of the paraspinal muscles). In many
Therefore, at a minimum and in its most simplistic patients other comorbidities may be present mak-
form, a radiculopathy may be considered when ing the coexistence of root involvement and pro-
abnormalities are found in two and preferably cesses distal to the dorsal root ganglia not an
more muscles innervated by the same nerve root infrequent scenario.
but different peripheral nerves, and normal find- It can be concluded that NEE is pivotal in con-
ings are obtained in muscles supplied by normal firming the presence of a (motor) radiculopathy,
adjacent roots. Commonly, an abnormality limited but goes further to disclose the underlying patho-
to one muscle is insufficient for diagnosis. physiologic process (such axon loss) and its
severity, may help date the process as acute/sub- the semitendinous, semimembranous, biceps
acute [especially presence of abundant fibrillation femoris long and short heads, and all muscles
and/or positive sharp wave potentials (including below the knee.
in proximal muscles), commencing about 3 week When there is anterior thigh weakness or
after injury and remaining until ~8 months to a proximal lower limb weakness, it is difficult on
year after injury (with successful re-innervation); clinical grounds alone to determine if the weak-
vs more chronic as demonstrated by the presence ness is due to a plexus lesion, L2–L4 radiculopa-
of long duration, high amplitude (+/−polyphasic) thy, or a femoral neuropathy. Furthermore, a
motor unit potentials—indicating collateral myopathic process often cannot be completely
sprouting and re-innervation. NEE findings also excluded, especially if sensory symptoms are not
help exclude muscle disorders—another cause of apparent. Some features may indicate the lesion
weakness that may appear on nerve conduction is proximal to the femoral nerve, including weak-
studies as reduced motor responses, with sparing ness of hip flexion (iliopsoas involvement); obtu-
of sensory responses. rator nerve involvement via thigh adductor
weakness. Involvement of muscles distal to the
knee such as the tibialis anterior would suggest a
Proximal Lower Limb/Anterior Thigh more widespread process involving two or more
Weakness nerves, or alternatively and more likely, involve-
ment of the lumbar/lumbosacral plexus or even
Proximal lower limb/anterior thigh weakness more proximally at the nerve roots. An important
may be another common reason for consultation. clinical observation would be the presence of lat-
Frequently, the request is to exclude a proximal eral thigh sensory loss, which is usually indica-
myopathy, however other conditions must be tive of involvement of the lateral femoral
excluded such as anterior horn disease (MND), cutaneous nerve of the thigh (L2/L3—posterior
Diabetic lumbosacral radiculoplexus neuropathy plexus division).
(DLRPN) (also called diabetic amyotrophy), A suggested EDX assessment of anterior thigh
other lumbar plexus lesion, obturator and a femo- or proximal muscle weakness includes in addi-
ral neuropathies [14, 16]. The process of bipedal tion to the standard lower extremity assessment
motion is quite complex requiring stabilization of (vide supra) the performance of femoral motor
the hip, alternating anterior hip rotation, knee recording quadriceps (rectus femoris or vastus
locking, anterior heel placement and foot propul- lateralis), and saphenous sensory studies bilater-
sion. Among these, proximal leg weakness may ally (though these responses are technically dif-
manifest itself as inability/difficulty with getting ficult to obtain in most cases). The NEE must
out of a chair or a low car seat, or rising from a include, in addition to the standard muscles
squatting position, as well knee-buckling. When aforementioned, study of the rectus femoris (or
present, this latter phenomenon usually indicates vastus lateralis), iliacus, and adductor longus
quadriceps muscle weakness (muscle innervated (often useful to also study contralateral
by the femoral nerve-L2, L3, L4 roots/seg- muscles).
ments—posterior division lumbar plexus). The Femoral mononeuropathies, regardless of
obturator nerve—(L2–L3–L4 roots/segments— etiology often have a similar appearance on
anterior division lumbar plexus) supply the thigh EDX studies, i.e. low amplitude CMAP record-
adductor muscles, and the femoral (plus more ing quadriceps, and decreased recruitment on
direct/proximal) motor branches also innervate NEE, with fibrillation and positive sharp wave
the iliacus and psoas muscles—[(L1)L2–L3— potentials if there is active/ongoing denervation.
(via lumbar plexus) for thigh flexion]. The sciatic It is important to be mindful of the context in
nerve-[(L4)–L5–S1—lumbosacral trunk-which the suspected process presents to make
plexus—tibial/peroneal (fibular) nerves]—inner- the appropriate EDX correlation (for e.g. poten-
vate many muscles in the posterior thigh including tial focal demyelination/conduction block in a
patient with prolonged lithotomy position). It is 1. Partial or complete demyelinating block

also i mportant to sufficiently study several adja- across the fibular head (seen in 30% of the
cent non-femoral innervated muscles, for e.g. cases)
the tibialis anterior and adductor muscles and 2. Pure axonal loss, partial (with fascicular spar-
these should be normal to convincingly con- ing) or complete (either seen in 50% of the
clude on a femoral neuropathy. If these muscles cases)
are involved, then one has to make the appropri- 3. Mixed demyelinating and axonal loss features
ate conclusions of a plexus lesion, multifocal (seen in 15–20% of cases)
mononeuropathies or root involvement. An 4. A rare pure deep peroneal (fibular) neuropa-
example for these latter findings or combina- thy sparing the superficial peroneal (fibular) in
tions of them will be diabetic amyotrophy (lum- less than 5% of the cases.
bosacral radiculoplexus neuropathy).
In those with a focal demyelinating process at
the fibular head, they have normal peroneal (fibu-
Foot Drop/Weakness lar) motor responses (recording TA and EDB)
when stimulating below the fibular head, but sig-
An important muscle to assess when a patient nificant drop in or absent amplitude when stimu-
presents with foot drop is the tibialis anterior lating above the fibular head, and normal
muscle (the major foot/ankle dorsiflexor). In most superficial peroneal (fibular) SNAP. In those with
cases, the cause of foot drop is a L4/5 radiculopa- a pure axon loss lesion, there is decreased and/or
thy or common peroneal (fibular) neuropathy at absent motor responses along the peroneal (fibu-
the fibular head. However, this clinical deficit may lar) nerve path when stimulating above or below
also be seen in an anterior horn cell disorder such the fibular head, in addition to absent superficial
as a monomelic presentation of MND, a plexopa- peroneal (fibular) SNAP. Those with a mixed
thy, a sciatic mononeuropathy, an isolated deep lesion will demonstrate low amplitudes of the
peroneal (fibular) mononeuropathy, a distal poly- CMAP recording EDB and TA, partial or total
neuropathy, neuromuscular junction transmission block when stimulating above the fibular head
defect, or muscle disease [particularly distal and low/absent superficial peroneal (fibular)
myopathy or isolated TA myopathic involvement SNAP. When the rare pure deep peroneal (fibu-
(rare)]. Non PNS causes of foot drop include a lar) neuropathy is present, there is a low ampli-
focal cortical cerebral infarction, and musculo- tude of the CMAP recording EDB and TA, with
skeletal disorders/deformities. normal superficial peroneal (fibular) SNAP.
A suggested EDX assessment for foot drop In patients with an L5 radiculopathy, the most
in addition to the standard lower extremity common process is axon loss, usually manifest-
assessment includes peroneal (fibular) motor ing itself as a predominantly foot drop with
responses recording tibialis anterior, and the involvement of the tibialis anterior muscle. EDX
superficial peroneal (fibular) sensory responses findings will include a low to unelicitable (when
(bilaterally obtained, if necessary). On NEE, we very severe) CMAP responses recording TA and
add to the standard complement—the extensor EDB with normal response of the superficial
halluces longus, tibialis posterior, peroneous peroneal (fibular) sensory nerve. On NEE, given
longus, biceps femoris short head, and semiten- that the lesion is at the L5 nerve root/segment, all
dinous (with corresponding contralateral lower muscles within this myotome will be affected,
extremity muscles typically examined when including those below the knee (common pero-
abnormalities are found). neal (fibular) and L5-tibial innervated muscles)
Patients with common peroneal (fibular) neurop- and proximal L5 innervated muscles such as the
athy at the fibular head which commonly results in gluteus medius and/or tensor fascia lata.
foot drop may have, according to Katirji and When a high sciatic neuropathy is suspected
Wilbourn [17] four EDX presentations (see figure): as the cause for foot drop, the peroneal (fibular)
motor and sensory responses are usually more baseline). In addition, slow repetitive nerve stim-
affected than the derivatives of the tibial nerve. ulation of the peroneal (fibular) nerve recording
Most lesions are of the axon loss type, with TA, median nerve recording APB, spinal acces-
demyelinating lesions rarely being reported in sory nerve recording trapezius and facial nerve
this context. The NCS usually demonstrate recording nasalis (when indicated, as with crani-
decreased to unelicitable responses recording obulbar involvement) is usually performed. In
EDB and TA when stimulating the peroneal (fib- patients suspected to have a myopathic disorder,
ular) nerve and low to absent SNAP of the super- the NCS usually comprise one motor and sen-
ficial peroneal (fibular) sensory. The tibial nerve sory response stimulating the median nerve, one
response recording AH may be normal or motor response in the lower extremity (typically
decreased in amplitude. It is important to remem- peroneal (fibular) motor recording EDB or TA if
ber that the sural nerve receives fibers from both EDB abnormal) and a sural sensory response.
the common peroneal (fibular) and tibial nerves, F-waves of the tibial and median are also usually
therefore the sural response may be low to nor- performed. Additional studies must be informed
mal as the sural nerve may be predominantly by the initial findings on these responses. For
receiving tibial relatively unaffected sensory example, if the initial studies suggest the pres-
fibers. On NEE, the peroneal (fibular)-innervated ence of a polyneuropathy, then additional NCS
muscles will typically be more prominently to complete a polyneuropathy protocol is usually
involved—including those supplied by the super- pursued. The reader is referred to the Myopathy/
ficial peroneal (fibular) and deep peroneal (fibu- Muscle Disorders chapter for further detailed
lar) branches. discussion on this topic.
Generalized Weakness References
Patients with motor neuron disease, neuromuscular 1. Aminoff MJ. Electromyography in clinical practice.
3rd ed. Baltimore: Williams and Wilkins; 1998.
junction transmission defects and myopathies are 2. Wilbourn AJ. How can electromyography help you?
commonly referred to the EMG laboratory when Postgrad Med. 1983;73(6):187–95.
generalized weakness is the main symptom. The 3. Campbell WW. Essentials of electrodiagnostic medi-
clinical history and examination will usually guide cine. Baltimore: Williams & Wilkins; 1999.
4. Neal PJ, Katirji B. Nerve conduction studies: practi-
the electrodiagnostician to the proper studies. cal guide and diagnostic protocols. AANEM; 2015.
For those patients with suspected motor neu- www.aanem.org
ron disease, we recommend performing a “root 5. Wilbourn AJ. Nerve conduction studies. Types, com-
search” protocol of the upper and lower extrem- ponents, abnormalities, and value in localization.
Neurol Clin N Am. 2002;20:305–38.
ity (including NEE of muscles which may be a 6. Rubin DI. Needle electromyography. In: Rubin D,
part of a “split-hand” pattern), as well as NEE of Daube J, editors. Clinical neurophysiology. 4th ed.
the thoracic paraspinal muscles (usually mid and Oxford: Oxford University Press; 2016.
low levels). One may also perform post exercise 7. Ferrante MA. EMG what we measure and what we
do. AANEM 2012. www.aanem.org
testing recording APB or FDI to help exclude a 8. Kim DH, Midha R, Murovic JA, Spiner RH. Kline
neuromuscular junction transmission disorder. If and Hudson’s nerve injuries. 2nd ed. Philadelphia:
the history suggests a neuromuscular junction Saunders Elsevier; 2008.
transmission defect, we typically perform select 9. Sunderland, Sydney. Nerves and nerve injuries. 2nd
ed. New York: Churchill Livingstone; 1978.
motor/sensory studies in the upper and lower 10. Kline DG, Hudson AR. Nerve injuries. Operative
extremities such as sural, median motor and sen- results for major nerve injuries, entrapments and
sory, ulnar motor and sensory, and peroneal (fib- tumors. Philadelphia: W.B. Saunders; 1995.
ular) motor. All these motor nerve responses will 11. Ferrante MA. Comprehensive electromyography
with clinical correlations. Cambridge: Cambridge
include a pre- and post-10 s of isometric exercise University Press; 2018.
(particularly, if amplitudes are decreased at
12. Isley MR, Krauss GL, Levin KH, Litt B, Shieilds RW, 15. Kimura J. Electrodiagnosis in diseases of nerve and
Wibourn AJ. Electromyography/electroencephalogra- muscle. Principles and practice. 4th ed. Oxford:
phy. SpaceLabs Medical. 1993. Oxford University Press; 2013.
13. Dumitru D, Amato AA, Zwarts MJ. Electrodiagnostic 16. Wilbourn AJ. Focal weakness, numbness or pain in the
medicine. 2nd ed. Philadelphia: Hanley & Belfus, Inc; lower extremities. American Academy of Neurology.
2002. 49th Annual Meeting, April 12–17, 1997.
14. Preston DC, Barbara E. Shapiro. electromyog- 17. Katirji MB, Wilbourn AJ. Common peroneal mono-
raphy and neuromuscular disorders. Clinical- neuropathy: a clinical and electrophysiological study
electrophysiologic correlations. 3th ed. Philadelphia: of 116 lesions. Neurology. 1988;38:1723–8.
Elsevier Saunders; 2013.
Atlas of Nerve Conduction Studies
(NCS) 2
Alexandra Soriano, Karin Armstrong,
Melissa Goldberg, Lourdes Gonzalez,
and Dana Higginbotham
Introduction General Concepts
There may be some acceptable variability in cer- The performance of NCS is deceptively simple but
tain aspects of NCS, depending on the laboratory the importance of standardization in key aspects
performing the testing. What is described in this across laboratories cannot be overemphasized.
chapter follows the methodology utilized in our Accordingly, being consistent and attentive to
laboratory, which conforms to what is generally ensure that the studies are always performed in the
considered standard practice in the field of elec- same fashion is crucial for reliable NCS results.
trodiagnostic medicine. Most errors during NCS are caused by incorrect
or inconsistent technical components. Otherwise,
an anatomical variation may produce apparently
spurious NCS results as well, so knowledge of these
N. Galvez-Jimenez (*) ∙ A. Soriano
is also essential. Additionally, it is imperative to
Braathen Neurological Center, Cleveland Clinic
Florida, Weston, FL, USA maintain the tested limb/region at the recommended
temperature (above 32 °C for the upper extremities
Cleveland Clinic Lerner College of Medicine of Case
Western Reserve University, Cleveland, OH, USA and above 30 °C for the lower extremities, mea-
e-mail: galvezn@ccf.org; soriana@ccf.org sured at the dorsum of the hands and feet).
J. A. Morren Filter settings are also important, though typi-
Neuromuscular Center, Neurological Institute, cally preset in modern machines (e.g. 1 Hz–5 kHz
Cleveland Clinic, and Cleveland Clinic Lerner for compound muscle action potentials,
10 Hz–5 kHz for sensory nerve action potentials,
e-mail: morrenj@ccf.org 2 Hz–10 kHz for needle EMG, and 500 Hz–10 kHz
for single fiber EMG).
K. Armstrong · M. Goldberg · L. Gonzalez
Neurophysiology Lab, Cleveland Clinic Florida, E1: recording/active electrode. For motor
Weston, FL, USA NCS, this is on the motor point of the muscle (the
e-mail: armstrk@ccf.org; goldbem2@ccf.org; end plate region). Note: E1 used to be referred to
gonzall5@ccf.org
D. Higginbotham
Neurophysiology Lab, Neuromuscular Center,
Neurological Institute, Cleveland Clinic,
Cleveland, OH, USA
e-mail: higgind2@ccf.org

https://doi.org/10.1007/978-3-030-74997-2_2
as G1, but use of this term is now discouraged. Position: Patient is supine with the forearm
The “G” designation referred to “grid” derived and hand supinated, resting completely on the
from the classic electroencephalography litera- bed.
ture, but now obsolete. Recording electrode location:
E2: reference/inactive electrode. For motor
NCS, this is usually on the tendon of the muscle. E1: Second metacarpo-phalangeal joint.
Note: Similarly, E2 used to be referred to as E2: Second Distal interphalangeal joint, 3–4 cm
G2, but this term is now discouraged. distally to E1.
Recorded responses are those obtained from
E1 while E2 is silent. However, in some instances, Ground: Between stimulation and recording
E2 may be active due to inadvertent volume sites, at the dorsum of the hand.
conduction. Stimulation: At the wrist between the tendons
The ground electrode should always be of the flexor carpi radialis (FCR) and palmaris
between the stimulation site and recording sites. longus (PL), 13 cm proximal from E1.
As previously discussed in Chap. 1, the Caveats/Notes: make measurements with fin-
expected response is that of a negative (upward) gers extended and abducted.
potential. For motor NCS, if there is a positive
(downward) deflection preceding the negative edian Sensory Recording
M
deflection, is often because the E1 electrode is at the Thumb (See Fig. 2.2)
not adequately over the motor point/end plate Anatomy: brachial plexus (lateral cord ← upper
region and its position must be adjusted until a trunk) ← (mostly) C6 dorsal root ganglion
negative first potential response is obtained. (DRG).
Position: Patient is supine with the forearm
and hand supinated resting completely on the
Upper Extremities bed.
Recording electrode location:
Sensory NCS
E1: first metacarpo-phalangeal joint.
edian Sensory Recording at Index
M E2: first interphalangeal joint.
Finger (See Fig. 2.1)
Anatomy: brachial plexus (lateral cord ← upper Ground: Between stimulation and recording
and middle trunk) ← (mostly) C6–7 dorsal root sites, at the dorsum of the hand.
ganglia (DRG).
E2
E1
E2
E1
C
C G
G
Fig. 2.1 Median sensory response—stimulating at wrist, Fig. 2.2 Median sensory response—stimulating at wrist,
recording index. C = Cathode; G = Ground recording thumb. C = Cathode; G = Ground
2 Atlas of Nerve Conduction Studies (NCS) 27
C
E1 E2
C
G
E1
E2
Fig. 2.3 Median sensory response—stimulating at wrist, Fig. 2.4 Ulnar sensory response—stimulating at wrist,
recording middle finger. C = Cathode; G = Ground recording little finger (D5). C = Cathode; G = Ground
Stimulation: At the wrist between the tendons Position: Patient is supine with the forearm
of the flexor carpi radialis (FCR) and palmaris and hand supinated resting completely on the
longus (PL), 13 cm proximal from E1. bed.
Caveats/Notes: make measurements with fin- Recording electrode location:
gers extended and abducted.
E1: Fifth metacarpo-phalangeal joint.
edian Sensory Recording at Middle
M E2: Fifth Distal interphalangeal joint, 3–4 cm dis-
Finger (See Fig. 2.3) tally to E1.
Anatomy: brachial plexus (lateral cord ← middle
trunk) ← (mostly) C7 dorsal root ganglion Ground: Between stimulation and recording
(DRG). sites, at the dorsum of the hand.
Position: Patient is supine with the forearm Stimulation: At the medial wrist between ten-
and hand supinated resting completely on the dons of the flexor carpi ulnaris (FCU) and flexor
bed. digitorum profundus (FDP), 11 cm proximal to
Recording electrode location: E1.
Caveats/Notes: make measurements with fin-
E1: Third metacarpo-phalangeal joint. gers extended and abducted.
E2: Third Distal interphalangeal joint, 3–4 cm dis-
tally to E1. orsal Ulnar Cutaneous Sensory
D
Recording at Dorsum of the Hand (See
Ground: Between stimulation and recording Fig. 2.5)
sites, at the dorsum of the hand. Anatomy: brachial plexus (medial cord ← lower
Stimulation: At the wrist between the tendons trunk) ← (mostly) C8 dorsal root ganglion
of the flexor carpi radialis (FCR) and palmaris (DRG).
longus (PL), 13 cm proximal from E1. Position: Patient is supine with the forearm
Caveats/Notes: make measurements with fin- and hand pronated, resting completely on the
gers extended and abducted. bed.
Recording electrode location.
lnar Sensory Recording at Fifth Finger
U
(See Fig. 2.4) E1: dorsum of hand between the fourth and fifth
Anatomy: brachial plexus (medial cord ← lower finger web space.
trunk) ← (mostly) C8 dorsal root ganglion E2: 3–4 cm distal to E1, at the base of the fifth
(DRG). finger.

G
E1: At the anatomic “V” or web space formed
C E1 between the index finger and thumb
E2 metacarpals.
E2: First digit interphalangeal joint, 3–4 cm dis-
tal to E1.
Ground: Between stimulation and recording

sites, at the dorsum of the hand.
Stimulation: over the radius 10 cm proximal
Fig. 2.5 Ulnar sensory response—stimulating at wrist,
recording dorsum of hand (dorsal ulnar cutaneous sensory
to E1.
response). C = Cathode; G = Ground
edian Palmar Mixed Nerve (See
M
Fig. 2.7)
Anatomy: brachial plexus (lateral cord ← upper
and middle trunk) ← (mostly) C6–7 dorsal root
ganglia (DRG).
G
Patient position: Patient supine with arm rest-
C E1 ing comfortably completely on the bed. Palm fac-
E2
ing up and fingers abducted.
Important: This is an orthodromic nerve stim-
ulation study.
Fig. 2.6 Radial sensory response—stimulating at distal

E1: at the wrist crease between the flexor carpi radi-
forearm, recording at first web space. C = Cathode; alis and flexor pollicis longus.
G = Ground E2: 3 cm proximal to E1, in a straight line.
Ground: Between stimulation and recording Ground: dorsum of the hand.

sites, at the dorsum of the hand. Stimulation: in the palm, 8 cm distal to the
Stimulation: At the wrist, 10 cm proximal E1 in the space between the second and third dig-
from E1 recording electrode, stimulating between its (second metacarpal interspace).
the ulna and flexor carpi ulnaris (FCU) tendon,
proximal to the ulna styloid.
Caveats/Notes: Helpful in determining ulnar
neuropathy at the elbow, or most other lesions
proximal to the wrist, as it is typically spared in
an ulnar lesion at the wrist (Guyon’s canal).
C
adial Sensory Recording at Base

R E2 E1
of the Thumb (See Fig. 2.6) G
Anatomy: brachial plexus (posterior cord ←

upper and middle trunk) ← (mostly) C6–7 dorsal
root ganglia (DRG).
Position: Patient supine with forearm midway Fig. 2.7 Median palmar mixed nerve study—stimulating
between pronation and supination and resting the median nerve at the palm, recording at the wrist.
completely on the bed. C = Cathode; G = Ground
G
C
E2 E1
E2 E1
G
Fig. 2.9 Medial Antebrachial Cutaneous sensory

response—stimulating anteromedial elbow, recording
Fig. 2.8 Ulnar palmar mixed nerve study—stimulating medial forearm. C = Cathode; G = Ground
the ulnar nerve at the palm, recording at the wrist.
C = Cathode; G = Ground
ing up/forearm supinated and mildly flexed at the
Caveats/Notes: some laboratories use a stan- elbow.
dardized bar electrode (shown in picture). Recording electrode location:
lnar Palmar Mixed Nerve (See Fig. 2.8)

U E1: anteromedial forearm 12 cm distal to the
Anatomy: brachial plexus (medial cord ← lower stimulation site/cathode (that is, point between
trunk) ← (mostly) C8 dorsal root ganglion the biceps tendon and the medial epicondyle).
(DRG). E2: 3 cm distal to E1, in a straight line.
Patient position: Patient supine with arm rest-
ing comfortably completely on the bed. Palm fac- Ground: between the stimulation and record-
ing up and fingers abducted. ing sites.
Important: This is an orthodromic nerve stim- Stimulation: find the midpoint between the
ulation study. biceps tendon and medial epicondyle, 12 cm
Recording electrode location: proximal to E1.
Caveats/Notes: some laboratories use a stan-
E1: at the wrist crease between the flexor carpi dardized recording bar electrode (shown in
ulnaris and flexor digitorum profundus. picture).
E2: 3 cm proximal to E1, in a straight line.
ateral Antebrachial Cutaneous
L
Ground: dorsum of the hand. Sensory Recording Lateral Forearm
Stimulation: at the palm, 8 cm distal to the (See Fig. 2.10)
E1 in the space between the fourth and fifth digits Anatomy: musculocutaneous nerve ← brachial
(fourth metacarpal interspace). plexus (lateral cord ← upper trunk) ← (mostly)
Caveats/Notes: some laboratories use a stan- C6 dorsal root ganglion (DRG).
dardized bar electrode (shown in picture). Patient position: Patient supine with arm rest-
ing comfortably completely on the bed. Palm fac-
edial Antebrachial Cutaneous
M ing up/forearm supinated and mildly flexed at the
Sensory Recording Medial Forearm elbow.
(See Fig. 2.9) Recording electrode location:
Anatomy: brachial plexus (medial cord ← lower
trunk) ← (mostly) T1 dorsal root ganglion E1: anterolateral forearm 12 cm distal to the stim-
(DRG). ulation site, which is a point lateral to the biceps
Patient position: Patient supine with arm rest- tendon at the antecubital fossa.
ing comfortably completely on the bed. Palm fac- E2: 3 cm distal to E1, in a straight line.
E1
E1
E2
E2
C
Fig. 2.11 Median motor response recording the abductor

pollicis brevis, distal stimulation at the wrist. C = Cathode;
G = Ground
Fig. 2.10 Lateral Antebrachial Cutaneous sensory C
response—stimulating anterolateral elbow, recording lat-

eral forearm. C = Cathode; G = Ground E1
E2
Ground: between the stimulation and record-

ing sites.
Fig. 2.12 Median motor response recording the abductor
Stimulation: lateral to the biceps tendon at the pollicis brevis, proximal stimulation at the elbow.
antecubital fossa, 12 cm proximal to E1. C = Cathode; G = Ground
dardized recording bar electrode (shown in Ground: Between stimulation and recording
picture). sites—usually proximal dorsum of hand.
Proximal palm may be used instead.
Stimulation:
Motor NCS
Distal site: At the wrist between the tendons of
edian Motor Recording at Abductor
M the flexor carpi radialis (FCR) and palmaris
Pollicis Brevis (APB) longus (PL), 5 cm proximal from E1 (See
Anatomy/Innervation: Median nerve ← medial Fig. 2.11).
cord ← lower trunk ← C8-T1 spinal nerve roots. Proximal site: Antecubital fossa over pulse of
Position: Patient supine with forearm supi- brachial artery, just medial to the biceps ten-
nated, extended at the elbow and resting com- don (See Fig. 2.12).
pletely on the bed.
Recording electrode location: Caveats/Notes: If the recorded response when
stimulating at the antecubital fossa is larger in
E1: Motor point, belly of the ABP. amplitude than that recorded when stimulating at
E2: first metacarpophalangeal joint. the wrist, an anatomical variant/anomalous inner-
vation such as a Martin-Gruber anastomosis
(MGA) must be considered (this type of MGA

would involve cross-over median fibers innervat-
ing nearby thenar muscles which would typically
be ulnar-innervated, like the deep head of flexor C
pollicis brevis and the adductor pollicis). E1
E2
G
lnar Motor Recording at Abductor

U
Digiti Minimi (ADM)
Anatomy/Innervation: ulnar nerve ← medial cord
← lower trunk ← C8–T1 spinal nerve roots.
Fig. 2.13 Ulnar motor recording abductor digiti minimi,
Position: Patient supine with forearm supi-
with distal stimulation at wrist. C = Cathode; G = Ground
nated extended at the elbow and resting com-
pletely on the bed.
E1: Motor point, belly of the ADM.

E2: Mid-portion proximal phalanx fifth finger.
E2
sites—usually proximal dorsum of hand.
E1
Proximal palm may be used instead.
G
Stimulation:
Distal site: At the wrist medial to the tendon of

the flexor carpi ulnaris (FCU), 5 cm proximal
from E1 (See Fig. 2.13).
Proximal sites:
Below elbow: 4 cm distal to the ulnar groove/
medial epicondyle on the medial forearm C
(See Fig. 2.14).

Above elbow: 6 cm proximal to the ulnar
groove/medial epicondyle, at the medial
arm between biceps and triceps muscles
(See Fig. 2.15). with proximal stimulation at below-elbow. C = Cathode;
G = Ground
Accordingly, the total distance is 10 cm across
the elbow between these two proximal stimula-
tion sites. This measurement must be done fol- block between the elbow and the wrist, an ana-
lowing the contour of the medial aspect of the tomical variant/anomalous innervation such as a
forearm and arm, and the elbow must be in a 90 Martin-Gruber anastomosis must be considered
degrees flexed position. This is done to avoid (in this scenario, the crossover median-to-ulnar
“bunching up” or redundancy of the ulnar nerve fibers are stimulated at the wrist, but not at the
if the arm is extended, which could artifactually elbow sites).
produce a decreased distance measurement (since
this is done on the surface), resulting in spuri- lnar Motor Recording at First Dorsal
U
ously reduced motor conduction velocity. Interosseous (FDI)
Caveats/Notes: If the recorded response when Anatony/Innervation: ulnar nerve ← medial cord
stimulating at the elbow suggest a conduction ← lower trunk ← C8-T1 spinal nerve roots.
E2
E1
G
E1 C
E2
Fig. 2.16 Ulnar motor recording first dorsal interosse-

ous, with distal stimulation at wrist. C = Cathode;
G = Ground
with proximal stimulation at above-elbow. C = Cathode;
G = Ground
Accordingly, the total distance is 10 cm across
the elbow between these two proximal stimula-
Position: Patient supine with forearm supi- tion sites. This measurement must be done fol-
nated extended at the elbow and resting com- lowing the contour of the medial aspect of the
pletely on the bed. forearm and arm, and the elbow must be in a 90
Recording electrode location: degrees flexed position. This is done to avoid
“bunching up” or redundancy of the ulnar nerve
E1: Motor point, belly of the FDI. if the arm is extended, which could artifactually
E2: Midportion of the middle phalanx index produce a decreased distance measurement (since
finger. this is done on the surface), resulting in spuri-
ously reduced motor conduction velocity.
Ground: Between stimulation and recording Caveats/Notes: If the recorded response when
sites—usually proximal dorsum of hand. stimulating at the elbow suggest a conduction
Proximal palm may be used instead. block between the elbow and the wrist, an ana-
Stimulation: tomical variant/anomalous innervation such as a
Martin-Gruber anastomosis must be considered
Distal site: At the wrist slightly radial to the ten- (in this scenario, the crossover median-to-ulnar
don of the flexor carpi ulnaris (FCU), other- fibers are stimulated at the wrist, but not at the
wise site similar to that used when recording elbow sites).
the ADM (See Fig. 2.16).
Proximal sites: adial Motor Recording at Extensor
R
Below elbow: 4 cm distal to the ulnar groove/ Digitorum (Communis) [ED/EDC]
medial epicondyle on the medial forearm Anatomy/Innervation: posterior interosseous
(See Fig. 2.14—i.e. same stimulation site nerve ← radial nerve, posterior cord ← middle
as when recording ADM). and lower trunks ← C7–C8 spinal nerve roots.
Above elbow: 6 cm proximal to the ulnar Position: Patient supine with forearm pronated
groove/medial epicondyle, at the medial and elbow flexed and arm resting completely on
arm between biceps and triceps muscles the bed.
(See Fig. 2.15—i.e. same stimulation site Recording electrode location:
as when recording ADM).
E1: Motor point, belly of the EDC.

E2: posterior forearm about 5 cm proximal to
C
dorsum of wrist or ulnar styloid.
Ground: on the forearm between the recording

and stimulating sites. G
E1
Stimulation: E2
Distal site: At the elbow, at the groove between

biceps and brachioradialis muscles (See
Fig. 2.17). Fig. 2.19 Radial motor recording extensor digitorum,
Proximal sites: proximal stimulation at above-spiral groove. C = Cathode;
Below the spiral groove: between the biceps G = Ground
and triceps muscles, usually performed
only if there is a significant drop in ampli- Caveats/Notes: Do above spiral groove stimu-
tude when stimulating above the spiral lation site first, and then do the below spiral
groove recording EDC (See Fig. 2.18). groove stimulation site, only if there is a signifi-
Above the spiral groove, between the medial cant drop in amplitude (suggesting conduction
and lateral heads of the triceps (See block).
Fig. 2.19).
usculocutaneous Recording at Biceps
M
Brachii
Anatomy/Innervation: musculocutaneous nerve
← lateral cord ← upper trunk ← C5–C6 spinal
nerve roots.
Position: Patient supine with forearm supi-
nated and extended at the elbow and resting com-
C
E2 pletely on the bed.
E1
G
E1: Motor point, belly of the biceps.

E2: distal upper arm over the biceps tendon and
Fig. 2.17 Radial motor recording extensor digitorum, antecubital fossa.
distal stimulation at elbow. C = Cathode; G = Ground
sites.
Stimulation:
C
Distal: At the axilla beneath the tendon of the

G
short head of the biceps (See Fig. 2.20).
E1
Proximal: At Erbs point, in the supraclavicular
E2
fossa posterior to the sternocleidomastoid
muscle (See Fig. 2.21).
Caveat: Supramaximal stimulations may be

challenging at the Erb’s point due to patient dis-
Fig. 2.18 Radial motor recording extensor digitorum,
proximal stimulation at below-spiral groove. C = Cathode; comfort/pain intolerance. Important to compare
G = Ground
C
G E1
E2
G
E1
Fig. 2.20 Musculocutaneous motor recording biceps

brachii, distal stimulation at axilla. C = Cathode;
G = Ground
E2
C
E1 E2
G
Fig. 2.22 Axillary motor recording deltoid, stimulation

at Erb’s point. C = Cathode; G = Ground
Stimulation: At Erbs point, in the supracla-

Fig. 2.21 Musculocutaneous motor recording biceps
brachii, proximal stimulation at Erb’s point. C = Cathode; vicular fossa posterior to the sternocleidomastoid
G = Ground muscle (See Fig. 2.22).
Caveat: Supramaximal stimulations may be
challenging at the Erb’s point due to patient dis-
amplitude and latency of response to the contra- comfort/pain intolerance. Important to compare
lateral side. amplitude and latency of response to the contra-
lateral side.
xillary Recording at Deltoid Muscle
A
Anatomy/Innervation: Axillary nerve ← poste-
rior cord ← upper trunk ← C5-C6 spinal nerve Lower Extremity
roots.
Position: Patient is supine with forearm supi- Sensory NCS
nated, resting completely on the bed.
Recording electrode location: ural (Sensory) Recording Posterior
S
Distal Leg/Lateral Ankle (See Fig. 2.23)
E1: Motor point, belly of the deltoid (lateral Anatomy: the medial cutaneous branch from the
head). tibial nerve, and the lateral cutaneous branch from
E2: distal upper arm, above elbow. the common fibular nerve ← sciatic nerve ← lum-
bosacral plexus ← S1–2 dorsal root ganglia (DRG).
Ground: Between stimulation and recording Patient position: Patient in a lateral decubitus
sites, usually at the shoulder joint. position (contralateral limb down), with the knee
C
G
E2 E1
E1
E2 G
C
Fig. 2.23 Sural (sensory) recording at ankle/lateral mal-

leolus, stimulating at distal calf. C = Cathode; G = Ground Fig. 2.24 Superficial peroneal (fibular) sensory record-
ing dorsolateral aspect of ankle/proximal foot, stimulating
slightly flexed and leg resting comfortably, com- lateral distal leg. C = Cathode; G = Ground
pletely on the bed.
E1: postero-inferior to the lateral malleolus.

E2: placed on the side of the foot 3 cm distal to
E1 E2
E1. C G
Ground: Lateral lower leg, between stimula-

tion and recording sites.
Stimulation: Posterior aspect of distal leg,
with stimulation electrode 14 cm proximal to the Fig. 2.25 Saphenous (sensory) nerve recording medial
distal leg, stimulation at medial calf. C = Cathode;
E1.
G = Ground
dardized recording bar electrode (shown in
picture). Stimulation: Placing stimulating electrode
(cathode) in a straight line 10 cm (but may be up
uperficial Peroneal (Fibular) Sensory
S to 14 cm) proximal to E1.
Recording Dorsolateral Aspect Caveats/Notes: Some laboratories use a stan-
of Ankle/Proximal Foot (See Fig. 2.24) dardized recording bar electrode (shown in
Anatomy: peroneal (fibular) nerve ← sciatic picture).
nerve ← lumbosacral plexus, L5 (>S1) dorsal
root ganglion (DRG). aphenous Nerve Recording Medial
S
Patient position: Patient in supine with the leg Distal Leg (See Fig. 2.25)
resting comfortably, completely on the bed. Anatomy: femoral nerve ← lumbar plexus ←
Recording electrode location: L3–L4 dorsal root ganglia (DRG).
Patient position: patient supine with the leg
E1: lower lateral leg, dorsum of ankle with E1 resting comfortably, completely on the bed.
located halfway between lateral malleolus and Recording electrode location.
extensor digitorum longus tendon.
E2: placed 3 cm distal to E1. E1: medial leg/lower calf at ankle medial to the
tibialis anterior tendon.
Ground: Distal lower leg, between stimulation E2: 3 cm distal to E1, in the space between the
and recording sites. medial malleolus and the tibialis anterior mus-
cle tendon.
Ground: medial lower leg/lower calf, between picture). The lateral femoral cutaneous nerve
stimulation and recording sites. response is also often difficult to obtain with
Stimulation: cathode 10 cm (but may be up to consistency (especially in overweight/obese

14 cm) proximal to E1 between the medial gas- individuals). Therefore, one should be careful to
trocnemius and the tibia. interpret an unelicitable response as a pathologi-
Caveats/Notes: Some laboratories use a stan- cal finding, unless the contralateral response (in
dardized recording bar electrode (shown in pic- an unaffected limb) is obtained.
ture). The saphenous response is often difficult to
obtain with consistency. Therefore, one should be edial and Lateral Plantar Mixed Nerve
M
careful to interpret an unelicitable response as a Response Recording the Medial Ankle
pathological finding, unless the contralateral (See Figs. 2.27 and 2.28)
response (in an unaffected limb) is obtained. Anatomy: tibial nerve ← sciatic nerve ← lumbo-
sacral plexus, S1 (>S2, L4–5) dorsal root ganglia
ateral Femoral Cutaneous Nerve
L (DRG).
Recording Lateral Thigh (See Fig. 2.26) Patient position: Patient supine with the leg
Anatomy: lumbar plexus, L2–3 dorsal root gan- resting comfortably, completely on the bed.
glia (DRG). Orthodromic stimulation.
Patient position: patient supine with the leg Recording electrode location:
resting comfortably, completely on the bed.
Recording electrode location: E1: recording electrode is placed on the postero-
medial aspect of the distal leg/medial malleo-
E1: recording electrode is placed on the antero- lus in the hollow between the Achilles tendon
lateral aspect of the thigh 12 cm distal to the and medial malleolus.
stimulation site. E2: 3 cm proximal to E1.
E2: 3 cm distal to E1.
Ground: dorsum of foot, between stimulation
Ground: lateral thigh, between stimulation and recording sites.
and recording sites. Stimulation:
Stimulation: cathode is placed superior to the
inguinal ligament about 1 cm medial to the ante- Medial Plantar: cathode is placed 11–14 cm dis-
rior superior iliac spine (ASIS). tal to E1 on the medial aspect of the sole of the
Caveats/Notes: Some laboratories use a foot.
standardized recording bar electrode (shown in
E1
E2 E2
G E1
C
Fig. 2.26 Lateral femoral cutaneous nerve recording lat- Fig. 2.27 Medial plantar mixed nerve response record-
eral thigh, with stimulation above inguinal ligament. ing the medial ankle, stimulation at the medial sole.
C = Cathode; G = Ground C = Cathode; G = Ground
division of the Sacral Plexus ← L5-S1 spinal

nerve roots.
Patient position: Patient supine with the leg
G
E2
E1 C
E1: recording electrode is placed on the motor
point, belly of the extensor digitorum brevis.
E2: distal to E1, at the fifth metatarsophalangeal
joint.
Fig. 2.28 Lateral plantar mixed nerve response record- Ground: dorsum of foot, between stimulation
ing the medial ankle, stimulation at the lateral sole. and recording sites.
Stimulation:
Lateral Plantar: cathode is placed 13–14 cm dis- Distal Site: cathode is place on a straight line up
tal to E1 on the lateral aspect of the sole of the 6–8 cm (usually 7 cm) proximal to E1, stimu-
foot. lating at the distal ankle crease over the pero-
neal (fibular) nerve (See Fig. 2.29).
Caveats/Notes: Some laboratories use a stan- Proximal stimulation is performed at two sites:
dardized recording bar electrode (shown in Below the fibular head: 2–4 cm below the fib-
picture). Commonly, the plantar mixed nerve ular head in the lateral calf (See Fig. 2.30).
responses (especially the lateral response) may be Above the fibular head: in the lateral popliteal
unobtainable secondary to technical factors, espe- fossa adjacent to the biceps femoris ten-
cially if the patient is older than 50 years and/or don, about 10–12 cm proximal to the
has evidence of thickened skin of the sole of the below-fibular head stimulation site (See
foot. Therefore, one should be careful to interpret Fig. 2.31).
an unelicitable response as a pathological finding,
unless the contralateral response (in an unaffected Caveats/Notes: Some laboratories only per-
limb) is obtained. When these technical factors are form a below-fibular head site stimulation when
less likely (especially in those less than 50 years there is evidence of a conduction block between
old), absent plantar mixed nerve responses may the ankle and above-fibular head stimulation sites.
be the earliest electrodiagnostic manifestation
of a length-dependent large fiber polyneuropa-
thy. However, the complete set of routine lower
extremity studies should be performed and plantar
mixed nerve responses interpreted in the context
of other electrodiagnostic findings obtained and
the clinical presentation.
G
E2
Motor NCS E1 C
eroneal (Fibular) Motor Recording

P
at Extensor Digitorum Brevis (EDB)
Anatomy/Innervation: Deep Peroneal (fibular) Fig. 2.29 Fibular motor recording at extensor digitorum
nerve ← Common Peroneal (fibular) nerve ← brevis, distal stimulation at ankle. C = Cathode;
Sciatic nerve ← Lumbosacral trunk and Posterior G = Ground
G
E2
G E2
E1 E1
C
C
Fig. 2.32 Fibular motor recording at tibialis anterior, dis-

Fig. 2.30 Fibular motor recording at extensor digitorum tal stimulation at below-fibular head. C = Cathode;
brevis, proximal stimulation at below-fibular head. G = Ground
G
E2 E1
C
E2
E1 G
C
Fig. 2.33 Fibular motor recording at tibialis anterior,

proximal stimulation at popliteal fossa/above-fibular
Fig. 2.31 Fibular motor recording at extensor digitorum head. C = Cathode; G = Ground
brevis, proximal stimulation at above-fibular head.
Ground: between stimulation and recording
sites.
If the amplitude of the CMAP is reproducibly Stimulation:
higher at the below and above-fibular head stimu- Stimulation is performed at two sites:
lation sites (compared to that at the distal ankle
stimulation site), then an accessory peroneal (fib- Distal stimulation: 2–4 cm below the fibular head
ular) nerve variant must be considered. This is in the lateral calf (See Fig. 2.32).
typically confirmed by eliciting a significant Proximal stimulation: in the lateral popliteal
response with stimulation at the posterior aspect fossa adjacent to the biceps femoris tendon,
of the lateral malleolus, while recording the EDB. about 10–12 cm proximal to the below-fibular
head stimulation site (See Fig. 2.33).
eroneal (Fibular) Motor Recording
P
at Tibialis Anterior (TA) Caveats/Notes: Amplitude and configuration
Anatomy/Innervation: Deep Peroneal (fibular) of the motor response may vary considerably
nerve ← Common Peroneal (fibular) nerve ← depending on location of E1. Unless there is evi-
Sciatic nerve ← Lumbosacral trunk and Posterior dence of conduction block between the standard
division of the Sacral Plexus ← L4, L5 spinal nerve distal and proximal stimulation sites outlined,
roots. there is usually no need to stimulate further
Patient position: Patient supine with the leg between these sites in the popliteal fossa.
Recording electrode location: ibial Motor Recording at Abductor
T
Hallucis (AH)
E1: recording electrode is placed on the muscle Anatomy/Innervation: Medial Plantar nerve ←
belly of the tibialis anterior muscle. Tibial nerve ← Sciatic nerve ← Anterior division
E2: placed anterior/top of ankle. of the Sacral Plexus, S1 > S2 spinal nerve roots.
Patient position: patient supine with the leg

resting comfortably, completely on the bed. E2
Recording electrode location: G
C E1
E1: recording electrode is placed on AH muscle

belly on the medial aspect of the plantar arch,
1 cm distal to the prominence of the navicular
bone.
E2: base of great toe, at the first metatarsophalan- Fig. 2.35 Tibial motor recording abductor hallucis, prox-
geal joint. imal stimulation at popliteal fossa. C = Cathode;
G = Ground
and recording sites. abundant popliteal fat pad, hence firm pressure
Stimulation: and higher stimulation intensity may be needed
(sometimes associated with marked discomfort).
Distal site: 8 cm proximal to E1 at the hollow Commonly, a significant drop in amplitude from
space between the medial malleolus and the proximal stimulation site (compared to that
Achilles tendon (See Fig. 2.34). obtained at the distal/ankle stimulation site) is
Proximal site: lateral aspect of the popliteal fossa, noted. Accordingly, caution must be applied to
at the level that corresponds to the lower bor- not overcall a partial/incomplete conduction
der of the kneecap (See Fig. 2.35). block in this scenario (typically, an amplitude
drop of up to 50% may be dismissed).
Caveats/Notes: Proximal stimulation may be
difficult to perform in some individuals with an ibial Motor Recording at Abductor
T
Digiti Quinti Pedis (ADQP)
Anatomy/Innervation: Lateral Plantar nerve ←
Tibial nerve ← Sciatic nerve ← Anterior division
of the Sacral Plexus, S1 > S2 spinal nerve roots.
Patient position: Patient supine with the leg
E2
G E1: recording electrode is placed on ADQP mus-

cle belly—about mid-distance between the
E1
lower edge of the lateral malleolus and the lat-
eral border of the foot.
E2: little toe, at the fifth metatarsophalangeal
joint.
C

and recording sites.
Stimulation:
Distal site: 8 cm proximal to E1 at the hollow

space between the medial malleolus and
Achilles tendon (See Fig. 2.36).
Fig. 2.34 Tibial motor recording abductor hallucis, distal Proximal site: lateral aspect of the popliteal fossa,
stimulation at medial ankle. C = Cathode; G = Ground at the level that corresponds to the lower bor-
G E1
C
E2
E2
E1
Fig. 2.37 Femoral motor recording at rectus femoris,

G stimulation at just below inguinal ligament. C = Cathode;
G = Ground
approximately at mid-point between the hip

C
and knee joints.
E2: tendinous portion just above the knee.
Ground: proximal thigh, between stimulation

and recording sites.
Stimulation: cathode is place below the ingui-
nal ligament at the inguinal crease, just lateral to
Fig. 2.36 Tibial motor recording abductor digiti quinti the femoral pulse point (See Fig. 2.37).
pedis, distal stimulation at medial ankle (proximal stimu-
lation at popliteal fossa is identical to that for abductor Caveat: Effective stimulation may be difficult
hallucis). C = Cathode; G = Ground to perform in some larger individuals due to tis-
sue impediment (including difficulty palpating
the femoral pulse). Hence firm pressure may be
der of the kneecap (See Fig. 2.35—i.e. same required. Observing the contraction of the rectus
proximal stimulation site when recording femoris is more important in this scenario. If no
AH). observable rectus femoris contraction is noted, or
other muscles (e.g. vastus medialis) respond to
Caveats/Notes: Proximal stimulation may be stimulation instead, the cathode must be
difficult to perform in some individuals with an repositioned.
abundant popliteal fat pad, hence firm pressure
and higher stimulation intensity may be needed ibial H-Reflex Recording at Soleus
T
(sometimes associated with marked Anatomy/Innervation: Tibial nerve ← Sciatic
discomfort). nerve ← Anterior division of the Sacral Plexus,
S1 > S2 spinal nerve roots.
emoral Motor Recording at Rectus
F Patient position: The patient should be prone
Femoris on the bed, using a pillow or similar item to help
Anatomy/Innervation: Femoral nerve ← Posterior keep the limb comfortable during the study.
division of the Lumbar Plexus ← (L2)L3-L4 spi- Recording electrode location:
nal nerve roots.
Patient position: Patient supine with the leg Recording: E1 is placed at soleus muscle, just
resting comfortably, completely on the bed. over the point in between the medial and lat-
Recording electrode location: eral heads of the gastrocnemius muscle. It is
helpful to have the patient plantar flex the foot
E1: recording electrode is placed over the belly to help with delineating space just below the
of the rectus femoris in the anterior thigh, separation of the two heads of the gastrocne-
mius muscle. The E1 electrode must be placed Although a bilaterally absent tibial H-reflex
over this space. response may confer less diagnostic yield in the
Reference: E2 is placed in distal leg, typically work-up of a focal lesion/process, reduction or
above or at the Achilles tendon (usually absence of the response with preservation on the
10–15 cm distal to E1). contralateral/unaffected side is diagnostically
valuable.
Ground: Proximal to E1 on the leg below the The lack of proper positioning and patient
knee, between stimulation and recording sites. relaxation commonly results in an absent/subop-
Stimulating: mid-popliteal fossa (over the timal response.
popliteal pulse), with the cathode positioning
reversed/polarity of the stimulator reversed, so pinal Accessory Motor Recording at
S
that the cathode is effectively proximal to the Trapezius
anode in the popliteal fossa (See Fig. 2.38). Anatomy/Innervation: spinal accessory nerve ←
Caveats/Notes: The tibial H-reflex response C3&C4 spinal nerves.
usually has a latency between 25 and 35 ms. Patient position: Patient supine with arm rest-
The H-response/reflex begins to be observed ing comfortably, completely on the bed.
before the “M” or muscle response. Recording electrode location:
As the intensity of the H-reflex stimulation
increases, the M response increases and the E1: Belly of the (upper) trapezius muscle.
H-response decreases until the H-response is no E2: placed on top of the shoulder (over glenohu-
longer obtainable. meral joint).
The tibial H-reflex is commonly absent after
age 60, after lumbosacral spine surgeries, proxi- Ground: upper back, between stimulation and
mal (e.g. root-level) demyelination injury, axon- recording sites.
loss radiculopathies, and large fiber Stimulation: lateral to the sternocleidomastoid
polyneuropathies [loss of the sensory (afferent) muscle.
and/or motor (efferent) volley]. Therefore, this This setup can be used during the repetitive
response provides a very sensitive evaluation of nerve stimulation protocol, in the work-up of a
those S1 > S2/tibial sensory fibers that pass neuromuscular junction transmission disorder
through the popliteal fossa. (See Fig. 2.39).
The tibial H-reflex is affected by both axon Caveats/Notes: Some laboratories may use a
loss and demyelination processes along the standardized bar electrode connected to the hand-
S1 > S2/tibial nerve fiber pathway from the pop- held stimulator prongs via an adapter (shown in
liteal fossa to the spinal cord, including the pre- picture).
ganglionic sensory root segment.
acial Motor Recording at Nasalis
F
Anatomy/Innervation: Facial nerve (cranial
nerve VII) originates from the union of the
axons coming from the facial motor nucleus
E2 (primarily motor fibers for facial expression
E1
G
muscles) and the nervus intermedius (giving
C parasympathetic, taste, and non-taste sensory
fibers). The zygomatic branch innervates the
nasalis muscle.
Patient position: Patient supine,
Fig. 2.38 Tibial H-Reflex recording at Soleus. C = Cathode; semi-recumbent.
G = Ground
Ground: under the chin (shown in picture), or

forehead.
Stimulation: cathode is place just below the
ear and anterior to the mastoid process (See
C Fig. 2.40).
Caveat: Disposable electrodes may be used
G
for facial nerve conduction studies. In a similar
E1
manner, other facial muscles may be used for
recording purposes. However, each laboratory
E2
must ensure technique consistency for results
reliability, reproducibility, and comparison
purposes.
The nasalis (shown), frontalis, zygomaticus,
orbicularis oris, orbicularis oculi, buccinators or
quadratus labii superioris (levator labii superi-
oris), and mentalis muscles may all be target
Fig. 2.39 Spinal accessory motor recording at trapezius, muscles from which the facial CMAP response
stimulation lateral to the sternocleidomastoid muscle. may be recorded.
C = Cathode; G = Ground The chosen muscle will depend on the clinical
context and indication, determined on a case-by-
case basis.
Suboptimal placement of the stimulating elec-
trode may result in an initial positive deflection in
the motor response. The appropriate motor
E2 E1
response consists of an initial negative
deflection.
C
G
Suggested Reading
1. Kubhare D, Robinson L, Buschbacher R, editors.
Buschbacher’s manual of nerve conduction studies.
3rd ed. New York: Demos Medical; 2016.
2. Neal PJ, Katirji B. Nerve conduction studies. Practical
guide and diagnostic protocols. AANEM. 2011.
3. Ferrante M. Comprehensive electromyography
with clinical correlations. Cambridge: Cambridge
Fig. 2.40 Facial motor recording at nasalis, stimulation University Press; 2018.
at anterior mastoid process. C = Cathode; G = Ground 4. Training Programs in Electromyography Manual.
Rochester, MN: Mayo Clinic; 1980.
Recording electrode location: 5. Wilbourn AJ. Training program in electromography
special nerve conduction studies. Manual. Cleveland,
OH: Cleveland Clinic; 1992.
E1: recording electrode is placed on the nasalis 6. Hammer K. Nerve conduction studies. Springfield:
muscle (immediately lateral to mid-nose) Charles C. Thomas Pub; 1981.
bilaterally. 7. Lee HJ, DeLisa JA. Manual of nerve conduction study
and surface anatomy of needle electromyography. 4th
E2: placed at the same location contralaterally. ed. New York: Lippincott Williams & Wilkins; 2005.
Atlas of Needle Electrode
Examination (NEE) 3
and Alexandra Soriano
The needle electrode examination (NEE) requires electrical grounding, and puncture site compres-
that electromyographers employ a bedside manner sion post needle removal. Depending on the mus-
and skill set that minimize patient anxiety and dis- cle being studied, there may be several specific
comfort, as well as maximize patient cooperation. caveats, confounders, precautions and tips to
With experience and the appropriate diligence, the keep in mind to ensure successful examination.
NEE will model a successful combination of art
and science in electrodiagnostic medicine.
It is necessary to have a replete knowledge of Upper Extremity
musculoskeletal anatomy as it pertains to “sur-
face localization” which facilitates correct needle Abductor Pollicis Brevis (APB)
insertion point positions (using several surface
landmarks, including bony prominences). Innervation: Median nerve ← medial cord ←
However it is also important to be versed in cross- lower trunk ← C8–T1 spinal nerve roots.
sectional anatomy to aid in the safe and adequate Activation: With forearm and hand supinated
placement of the needle (particularly the record- have patient abduct the thumb.
ing tip) during the study. Providing clear, precise Needle placement: Into the thenar eminence,
instructions to the patient, with appropriate body anterolateral to the mid-point of first metacarpal
and limb positioning are also key to successful (See Fig. 3.1).
NEE, as are several measures to reduce compli- Indications include: Carpal tunnel syndrome,
cations like appropriate skin cleaning/antisepsis, proximal median neuropathies, lower trunk/
medial cord plexopathy, thoracic outlet syn-
drome, C8–T1 radiculopathy.
N. Galvez-Jimenez (*) ∙ A. Soriano
Braathen Neurological Center, Cleveland Clinic Notes and precautions/confounders: Best
Florida, Weston, FL, USA muscle to sample distal to the carpal tunnel. This
Western Reserve University, Cleveland, OH, USA
e-mail: galvezn@ccf.org; soriana@ccf.org
J. A. Morren
Neuromuscular Center, Neurological Institute,

https://doi.org/10.1007/978-3-030-74997-2_3
Fig. 3.2 Needle insertion site (black dot in center of red

circle) for needle electrode examination of the opponens
Fig. 3.1 Needle insertion site (black dot in center of red pollicis
circle) for needle electrode examination of the abductor
pollicis brevis
muscle is spared in anterior interosseous syn-

drome. Tends to be difficult to tolerate due to
exquisite tenderness in some.
Opponens Pollicis
Innervation: Median nerve ← medial cord ← circle) for needle electrode examination of the flexor pol-
lower trunk ← C8–T1 spinal nerve roots. licis brevis
Activation: With forearm and hand supinated
have patient oppose the thumb to the little finger. Needle placement: Just lateral (superficial
Needle placement: Into the lateral thenar emi- head) or just medial (deep head) to the mid-point
nence (lateral to the site for abductor pollicis bre- of the first metacarpal bone in the thenar emi-
vis), with oblique needle insertion, almost nence (See Fig. 3.3).
parallel to the palm (See Fig. 3.2). Indications include: Generally avoided in
Indications include: Carpal tunnel syndrome, most routine work-ups (especially due to dual
proximal median neuropathies, lower trunk or innervation). May have some utility in cases of
medial cord plexopathies, thoracic outlet syn- lower trunk or medial cord plexopathies, or C8–
drome, or C8–T1 radiculopathy. T1 radiculopathy.
Notes and precautions/confounders: This Notes and precautions/confounders: Has
muscle is right below (deep to) the abductor pol- mixed ulnar (deep head) and median (superficial
licis brevis muscle, so avoid inserting needle too head) innervation, with potential variability in
superficially. normal subjects. Accordingly, interpretation of
abnormalities should be done with caution.
Flexor Pollicis Brevis

Pronator Quadratus (PQ)
Innervation: Median (superficial head) and ulnar
(deep head) nerves ← medial cord ← lower trunk Innervation: Anterior interosseous nerve
← C8–T1 spinal nerve roots. (branch) ← median nerve ← lateral and medial
Activation: Flexion of thumb at the metacar- cords, middle ← lower trunk ← C7–C8–T1 spi-
pophalangeal joint. nal nerve roots.
3 Atlas of Needle Electrode Examination (NEE) 45

Fig. 3.4 Needle insertion site (black dot in center of red circle) for needle electrode examination of the flexor pol-
circle) for needle electrode examination of the pronator licis longus
quadratus
up from the lateral wrist toward the lateral elbow

Activation: with elbow slightly flexed, have (see Fig. 3.5).
the patient pronate the hand. Indications include: Anterior interosseous
Needle placement: Three fingerbreadths prox- nerve syndrome or proximal median neuropa-
imal to the mid-point of an imaginary line drawn thies, C8–T1 radiculopathy.
from the ulnar to radial styloid, insertion via the Notes and precautions/confounders: If the
dorsal forearm with slight angulation of the nee- needle is inserted or oriented too medially, it may
dle laterally (towards radius) ensuring depth suf- enter the radial artery and potentially lead to a
ficient to pierce interosseous membrane, this with large hematoma from arterial (high-pressure)
hand in mid-position between supination and blood loss.
pronation (See Fig. 3.4).
Indications include: Anterior interosseous
nerve syndrome, proximal median neuropathies. lexor Digitorum Profundus to Digits
F
Notes and precautions/confounders: The mus- 2,3 (FDP)
cle is deep to the finger and thumb extensor ten-
dons and muscles, and the needle must be inserted Innervation: anterior interosseous nerve
sufficiently deep, through the interosseous mem- (branch) ← median nerve ← lateral and medial
brane (usually one detects confirmatory tissue cord ← middle and lower trunk ← C7–C8 spinal
resistance changes from the needle when this is nerve roots.
achieved). Activation: flexion of digits 2 or 3 at the distal
interphalangeal (DIP) joint.
Needle placement: three to four fingerbreadths
Flexor Pollicis Longus distal to the olecranon and about 1 fingerbreadth
medial to the ulna. The penetration depth should
Innervation: anterior interosseous nerve be about 3–4 cm (twice as deep as the ulnar-
(branch) ← median nerve ← lateral and medial innervated counterpart of this muscle going to
cords ← middle and lower trunks ← (C7)C8–T1 digits 4,5). (see Fig. 3.6)
spinal nerve roots. Indications include: Anterior interosseous
Activation: Flexion of the thumb at the inter- nerve syndrome or proximal median
phalangeal joint. neuropathies.
Needle placement: with hand supinated, over Notes and precautions/confounders: Due to
the radius and proximally one third the distance the depth of this muscle, the needle tip may come
Fig. 3.6 Needle insertion site (black dot in center of red Fig. 3.7 Needle insertion site (black dot in center of red
circle) for needle electrode examination of the flexor digi- circle) for needle electrode examination of the flexor digi-
torum profundus to digits 2,3 torum superficialis
quite close to the main trunk of the ulnar nerve,

so insertion while the patient is gently activating
the muscle may provide assurance that adequate
depth is attained and not surpassed. If insertion is
too superficial, the FDP to digits 4,5 (ulnar-
innervated) will be sampled erroneously.
Flexor Digitorum Superficialis (Sublimis)
Innervation: median nerve ← medial and lat-
eral cord ← middle and lower trunks ← C7–C8–
(T1) spinal nerve roots.
Activation: flexion of the digits at the proxi- Fig. 3.8 Needle insertion site (black dot in center of red
mal interphalangeal (PIP) joints. circle) for needle electrode examination of the flexor carpi
Needle placement: Volar aspect, about 2–3 cm radialis
medial to the mid-point between the biceps ten-
don and the mid-wrist, with the forearm supi- Activation: flexion of the wrist with radial
nated (see Fig. 3.7). deviation.
Indications include: Proximal median neurop- Needle placement: About four fingerbreadths
athies, but not in the anterior interosseous nerve distal to the mid-point between the biceps tendon
syndrome. and medial epicondyle, with forearm supinated.
Notes and precautions/confounders: This Indications include: Proximal median neurop-
muscle is more superficial than the FDP. The athies, pronator teres syndrome, but not in ante-
median nerve can be close if needle is inserted in rior interosseous nerve syndrome. Also used in
the midline and too deep. If insertion is too lat- the demonstration of a C6–C7 radiculopathy or a
eral, the needle may enter the flexor carpi radialis brachial plexopathy affecting the lateral cord (see
(also median-innervated). Fig. 3.8).
Notes and precautions/confounders: If needle
is placed too deeply it can inadvertently contact
Flexor Carpi Radialis (FCR) the median nerve. Insertion too laterally may
encounter the brachioradialis (radial-innervated);
Innervation: median nerve ← lateral cord ← insertion too laterally and proximally may
upper and middle trunks, C6–C7 spinal nerve encounter the pronator teres (also median-
roots. innervated); insertion too medially may encoun-

circle) for needle electrode examination of the first dorsal
Fig. 3.9 Needle insertion site (black dot in center of red interosseous
circle) for needle electrode examination of the pronator
teres
First Dorsal Interosseous (FDI)
ter the palmaris longus or flexor digitorum
superficialis (both median-innervated). Innervation: ulnar nerve ← medial cord ← lower
trunk ← C8–T1 spinal nerve roots.
Activation: Abduction of index finger (e.g.
Pronator Teres (PT) asking the patient to make a fist, then have index
finger point upwards).
Innervation: median nerve ← lateral cord ← Needle placement: in the dorsal hand, halfway
upper and middle trunks ← C6–C7 spinal nerve between the first and second metacarpophalan-
roots. geal (MCP) joints, with the needle inserted
Activation: With elbow extended, have patient obliquely more towards the second MCP joint
pronate the hand (“like turning a door knob”) (Fig. 3.10).
against some resistance from the examiner. Indications include: Ulnar neuropathy, lower
Needle placement: about three fingerbreadths trunk or medial cord brachial plexopathy, tho-
distal to the mid-point between biceps tendon racic outlet syndrome or C8–T1 radiculopathy.
and medial epicondyle, with forearm supinated Notes and precautions/confounders: Generally
(see Fig. 3.9). easy muscle to access reliably by needle exami-
Indications include: Proximal median neurop- nation, without significant potential for errors or
athies, but typically spared in pronator teres syn- confounders.
drome; also spared in the anterior interosseous
nerve syndrome. Also used to demonstrate
involvement in C6–C7 radiculopathy. Abductor Digiti Minimi (ADM)
Notes and precautions/confounders: If needle
is placed too deeply it can inadvertently contact Innervation: ulnar nerve ← medial cord ← lower
the median nerve. Needle insertion too laterally trunk ← C8–T1 spinal nerve roots.
may penetrate the brachioradialis (radial- Activation: Abduction of the little finger (for
innervated); insertion too medially may penetrate ease, the patient may spread all fingers during
the flexor carpi radialis (median-innervated as activation).
well); insertion too deeply may penetrate the Needle placement: In the medial aspect of the
flexor digitorum superficialis (median-innervated hand (hypothenar eminence), mid-point of the
as well). fifth metacarpal (See Fig. 3.11).
circle) for needle electrode examination of the abductor circle) for needle electrode examination of the flexor digi-
digiti minimi torum profundus to digits 4,5
Indications include: ulnar neuropathy (may be

spared in some ulnar lesions at Guyon’s canal),
lower trunk or medial cord brachial plexopathy,
thoracic outlet syndrome, C8–T1 radiculopathy.
lexor Digitorum Profundus (FDP)

F
to Digits 4,5
Innervation: ulnar nerve ← medial cord ← lower

trunk ← C8–T1 spinal nerve roots. Fig. 3.13 Needle insertion site (black dot in center of red
Activation: flexion of digits 4,5 at the distal circle) for needle electrode examination of the flexor carpi
ulnaris
interphalangeal joints.
Needle placement: three to four fingerbreadths
distal to the olecranon and about 1 fingerbreadth Activation: flexion or wrist with ulnar
medial to the ulna. The penetration depth should deviation.
be about 1.5 to 2 cm (half as deep as the median- Needle placement: with the forearm supi-
innervated counterpart of this muscle going to nated, the needle is inserted at about 4–5 fin-
digits 2,3) (See Fig. 3.12). gerbreadths distal to the medial epicondyle,
Indications include: ulnar neuropathy at the along the medial aspect of the ulna (see
elbow; lower trunk or medial cord brachial Fig. 3.13).
plexopathy, thoracic outlet syndrome, C8–T1 Indications include: ulnar neuropathy at the
radiculopathy. elbow (though fascicular sparing may be seen);
Notes and precautions/confounders: The more lower trunk or medial cord brachial plexopathy,
superficial muscle (to digits 4,5) is the one inner- thoracic outlet syndrome, C8–T1 radiculopathy.
vated by the ulnar nerve, which makes it relatively Notes and precautions/confounders: insert the
easy to study (compared to the median-innervated needle superficially as the muscle tends to be
FDP to digits 2,3, which is deeper). very thin. Deeper insertions may penetrate the
flexor digitorum superficialis (median-
innervated), or even flexor digitorum profundus
Flexor Carpi Ulnaris (FCU) (median- and ulnar-innervated). Too lateral an
insertion may end up in the palmaris longus or
Innervation: ulnar nerve ← medial cord ← lower flexor carpi radialis (both of these being
trunk ← C8–T1 spinal nerve roots. median-innervated).
Extensor Indicis [Proprius] (EI/EIP) Needle placement: on the lateral aspect (radial
side) of the mid-point of the ulnar, with forearm
Innervation: posterior interosseous nerve ← pronated (see Fig. 3.15).
radial nerve, posterior cord ← middle and lower Indications include: radial neuropathy includ-
trunks ← C7–C8 spinal nerve roots. ing posterior interosseous neuropathy, lower
Activation: extension of index finger. trunk or posterior cord brachial plexopathy, tho-
Needle placement: towards the radial (lateral) racic outlet syndrome, C7–C8 radiculopathy.
aspect of the ulna, three to four fingerbreadths Notes and precautions/confounders: too lateral
proximal to the ulnar styloid, with forearm and an insertion may lead to penetrating the extensor
hand pronated (see Fig. 3.14). digiti minimi (quinti) or extensor digitorum (both
Indications include: radial neuropathy includ- radial/posterior interosseous-innervated).
ing posterior interosseous (branch) neuropathy,
lower > middle trunk or posterior cord brachial
plexopathy, thoracic outlet syndrome, C8 > C7 xtensor Digitorum [Communis]
E
radiculopathy. (ED/ EDC)
Notes and precautions/confounders: If the
needle is inserted too superficially and/or too Innervation: posterior interosseous nerve ←
proximally it may be in the extensor carpi ulna- radial nerve, posterior cord ← middle and lower
ris, extensor digiti minimi, or extensor digitorum trunks ← C7–C8 spinal nerve roots.
(all radial/posterior interosseous-innervated). If Activation: extension of the middle finger (see
insertion is performed too laterally, it may pen- Fig. 3.16).
etrate the extensor pollicis longus (still radial/
posterior interosseous-innervated).
Extensor Carpi Ulnaris (ECU)
Innervation: posterior interosseous nerve ←

radial nerve ← posterior cord ← middle and
lower trunks ← C7–C8 spinal nerve roots.
Activation: extension of wrist with ulnar
deviation.
circle) for needle electrode examination of the extensor
carpi ulnaris
circle) for needle electrode examination of the extensor circle) for needle electrode examination of the extensor
indicis indicis
Needle placement: with the forearm pronated, Anconeus

the needle is inserted mid-forearm, at the mid-
point between the ulna and radius. Innervation: radial nerve ← posterior cord ←
Indications include: radial neuropathy includ- upper, middle and lower trunks ← C6–C7–C8
ing posterior interosseous neuropathy, C7–8 spinal nerve roots.
radiculopathy. Activation: Extension of the elbow.
Notes and precautions/confounders: If needle Needle placement: Two to three fingerbreadths
is inserted too medially, it may enter the extensor distal to the olecranon, on the radial aspect of the
digiti minimi or the extensor carpi ulnaris (both ulna, with the forearm pronated (see Fig. 3.18).
posterior interosseous-innervated); insertion too Indications include: radial neuropathies above
laterally may penetrate the extensor carpi radialis the spiral grove, since it is the only muscle in the
(radial-innervated). This muscle is commonly forearm that receives its radial innervation from
sampled during single fiber EMG studies. above the spiral grove. Accordingly, the anco-
neus is spared in radial neuropathy at (or distal
to) the spiral grove.
Brachioradialis Notes and precautions/confounders: placing
the needle on the medial aspect of the ulna may
Innervation: radial nerve ← posterior cord ← lead to inadvertently recording the flexor digito-
upper trunk ← C5–C6 spinal nerve roots. rum profundus (mixed median and ulnar innerva-
Activation: flexion of elbow with wrist in mid tion). If insertion is too lateral, it may penetrate
position between pronation and supination. the extensor carpi ulnaris (posterior
Needle placement: three to four fingerbreadths interosseous-innervated).
distal to mid-point between the biceps tendon
and lateral epicondyle (see Fig. 3.17).
Indications include: radial neuropathy (but Triceps Brachii
unaffected in posterior interosseous neuropathy),
upper trunk brachial plexopathy, C5–C6 Innervation: Radial nerve ← posterior cord ←
radiculopathy. upper, middle and lower trunks ← C6–C7–C8
Notes and precautions/confounders: placing spinal nerve roots.
the needle too laterally may lead to penetrating Activation: Elbow extension.
the extensor carpi radialis muscle (although also Needle placement: Mid-point between lateral
radial-innervated). epicondyle and shoulder (to access the lateral

circle) for needle electrode examination of the Fig. 3.18 Needle insertion site (black dot in center of red
brachioradialis circle) for needle electrode examination of the anconeus

Fig. 3.19 Needle insertion site (black dot in center of red circle) for needle electrode examination of the biceps
circle) for needle electrode examination of the triceps brachii
brachii
Notes and precautions/confounders: inserting

head of the triceps), with forearm pronated and the needle on the medial side of the muscle can
elbow flexed (see Fig. 3.19). encroach the brachial artery, the median or ulnar
Indications include: C7(>C6,C8) radiculopa- nerve and major veins (e.g. basilic vein) in the
thy. Not typically affected in radial neuropathy at area. Needle insertion too deeply may enter the
the spiral groove as it receives its innervation brachialis (also musculocutaneous-innervated);
from above this segment. insertion too proximally may enter the anterior
Notes and precautions/confounders: Approach head of the deltoid (axillary-innervated), or the
from the lateral head will minimize risk of inad- pectoralis major (medial and lateral
vertently contacting vascular and nervous struc- pectoral-innervated).
tures (e.g. the brachial artery and the radial nerve
trunk) in the area. If the needle is inserted too
anteriorly, it may encroach onto the biceps bra- Pectoralis Major
chii or the brachialis muscle (musculocutaneous-
innervated); insertion too proximally runs the Innervation: Medial and lateral pectoral nerves
risk of entering the deltoid muscle ← medial and lateral cords ← upper-middle and
(axillary-innervated). lower trunks ← C5–C6 spinal nerve roots (cla-
vicular portion) and C7–C8–T1 spinal nerve
roots (sternocostal portion).
Biceps Brachii Activation: Shoulder adduction.
Needle placement: In the anterior lower shoul-
Innervation: musculocutaneous nerve ← lateral der, at the anterior axillary line, just medial to the
cord ← upper trunk ← C5–C6 spinal nerve roots. anterior axillary fold (see Fig. 3.21).
Activation: flexion of the elbow with hand Indications: infrequently studied (isolated lat-
supinated. eral or medial pectoral neuropathy is very rare),
Needle placement: just lateral to the mid-point but may be abnormal in brachial plexopathy or
in the muscle between the anterior shoulder and cervical radiculopathy at multiple levels-C5–C6
biceps tendon/mid-antecubital fossa (see if testing clavicular portion, or C7–C8–T1 if test-
Fig. 3.20). ing sternocostal portion.
Indications include: C5 or C6 radiculopathies, Notes and precautions/confounders: Placing
brachial plexopathies involving upper trunk or the needle more medially and deeply may inadver-
lateral cord. tently penetrate the intercostal space and pleura,
circle) for needle electrode examination of the pectoralis circle) for needle electrode examination of the deltoid
major
with risk of pneumothorax, or the needle may

cause penetration injury to components of the bra-
chial plexus, or major blood vessels (e.g. subcla-
vian artery) in the area. Placing the needle too
laterally may misdirect it into the deltoid muscle.
Deltoid
Innervation: Axillary nerve ← posterior cord ←

upper trunk ← C5–C6 spinal nerve roots.
Activation: Shoulder abduction.
Needle placement: (for middle head) Outer
aspect of the shoulder, about three to four finger-
breadths below the lateral aspect of the acromion
(see Fig. 3.22). Fig. 3.23 Needle insertion site (black dot in center of red
Indications include: Axillary neuropathy, circle) for needle electrode examination of the teres minor
upper trunk or posterior cord brachial plexopa-
thy, C5–C6 radiculopathy.
Notes and precautions/confounders: Fairly the needle is placed about one fingerbreadth lat-
easy muscle to sample and away from major eral to the outer border of the middle third of the
nerves and vessels, especially if the medial head scapula (see Fig. 3.23).
is examined. Indications include: axillary neuropathy,
upper trunk and posterior cord brachial plexopa-
thy, C5–C6 radiculopathy.
Teres Minor Notes and precautions/confounders: placing
the needle too medially and/or superiorly can
Innervation: axillary nerve ← posterior cord ← get it into the infraspinatus (suprascapular-
upper trunk ← C5–C6 spinal nerve roots. innervated), and placing the needle too later-
Activation: external rotation of the arm. ally and/or superiorly may get it into the
Needle placement: With the patient in the lat- posterior head of the deltoid (also
eral decubitus position (contralateral side down), axillary-innervated).
Upper Trapezius Activation: Turning head and neck to the con-

tralateral side.
Innervation: spinal accessory nerve ← C3–C4 Needle placement: mid-point of the muscle
spinal nerve roots. (between the mastoid process and sternal
Activation: Shrugging/elevating of the shoulder. head), ensuring the muscle is well-delineated
Needle placement: With the patient in the lat- by palpation as well. The needle should enter
eral decubitus position (contralateral side down), the muscle at an angle so that it is almost paral-
the needle is inserted just lateral to the junction of lel to its fibers when being advanced (see
the shoulder and the neck (see Fig. 3.24). Fig. 3.25).
Indications include: spinal accessory nerve Indications include: spinal accessory nerve
injury, C3–4 radiculopathy. injury, C3–4 radiculopathy.
Notes and precautions/confounders: Insert the Notes and precautions/confounders: If the
needle superficially, as going too deep may place needle insertion is too anteriorly and/or medi-
it in the rhomboids or paraspinal muscles (espe- ally, it may cause penetration injury to the
cially if too medial as well), or it may enter the carotid artery or jugular vein. Unlike the trape-
apical pleura (risk for pneumothorax). zius, this muscle is often spared in iatrogenic
injury of the spinal accessory nerve with lymph
node dissection in the posterior triangle of the
Sternocleidomastoid neck.
Innervation: spinal accessory nerve ← C3–C4

spinal nerve roots.

circle) for needle electrode examination of the
circle) for needle electrode examination of the upper
sternocleidomastoid
trapezius
Supraspinatus

circle) for needle electrode examination of the
Fig. 3.26 Needle insertion site (black dot in center of red infraspinatus
circle) for needle electrode examination of the supraspinatus
Innervation: suprascapular nerve ← upper trunk Activation: external rotation of the arm (more
← C5–C6 spinal nerve roots. easily done with elbow semi-flexed).
Activation: shoulder abduction. Needle placement: With the patient in the lat-
Needle placement: With the patient in the lateral decubitus position (contralateral side down),
eral decubitus position (contralateral side down), insert the needle in the infraspinous fossa, about
insert the needle in the supraspinous fossa, just one to two fingerbreadths below the mid-point of
medial to the mid-point of the scapular spine, gen- the medial third of the scapular spine (see
tly advancing the needle to the bone, then slightly Fig. 3.27).
retracting the needle to ensure penetration is Indications include: suprascapular neuropa-
deeper than overlying trapezius (see Fig. 3.26). thy, upper trunk brachial plexopathy, C5–C6
Indications include: suprascapular neuropa- radiculopathy.
thy, upper trunk brachial plexopathy, C5–C6 Notes and precautions/confounders: A super-
radiculopathy. ficial insertion may place the needle in the trape-
Notes and precautions/confounders: The muscle zius muscle (innervated by the spinal accessory
will typically be unaffected in cases of suprascapular nerve, C3–4 spinal nerve roots). Too lateral an
neuropathy at the spinoglenoid notch (but typically insertion may place the needle into the posterior
affected with a lesion at the suprascapular notch). head of the deltoid (axillary-innervated). Too
A superficial needle insertion may place it at inferior a needle insertion may enter the latissi-
the trapezius muscle (innervated by the spinal mus dorsi (thoracodorsal-innervated).
accessory nerve, C3–4 spinal nerve roots).
Infraspinatus Rhomboids
Innervation: supra-scapular nerve ← upper trunk Innervation: Dorsal scapular nerve ← C5 spinal
← C5–C6 spinal nerve roots. nerve root (pre-brachial plexus).

Fig. 3.28 Needle insertion site (black dot in center of red circle) for needle electrode examination of the latissimus
circle) for needle electrode examination of the rhomboid dorsi
major
Latissimus Dorsi
Activation: Ask the patient to retract (draw Innervation: thoracodorsal nerve ← posterior
back) the shoulder blade toward the spine. cord ← upper, middle and lower trunks ← C6–
Needle placement: With the patient in the lat- C7–C8 spinal nerve roots.
eral decubitus position (contralateral side down). Activation: Extension (pushing back) of the
Rhomboid major (illustrated in Fig. 3.28 arm, with the arm also being internally rotated
below): about one to two fingerbreadths medial and abducted.
to the medial border of the scapula, at the mid- Needle placement: With the patient in the lat-
point between the scapular spine and the infe- eral decubitus position (contralateral side down),
rior angle. insert the needle about one fingerbreadth lateral
Rhomboid minor: about one to two finger- to the inferior angle of the scapula, within the
breadths medial to the medial border of the scap- posterior axillary fold (see Fig. 3.29).
ular spine. Indications include: brachial plexopathy, C6–
Indications include: C5 radiculopathy. C7–C8 radiculopathies.
Notes and precautions/confounders: Avoid Notes and precautions/confounders: Too supe-
deep needle insertion due to risk of pneumotho- rior a needle insertion runs the risk of inadver-
rax if the needle traverses a posterior intercostal tently sampling the teres major (lower
space. subscapular-innervated).
However, too superficial an insertion will put
the needle in the trapezius muscle (innervated by
the spinal accessory nerve, C3–4 spinal nerve Serratus Anterior
roots). Too inferior a needle insertion (for the
rhomboid major) may enter the latissimus dorsi • Innervation: Long thoracic nerve ← C5–C6–
(thoracodorsal-innervated). C7 spinal nerve roots
The rhomboids are typically not involved in • Activation: Forward flexion of the arm, reach-
brachial plexopathies of the upper trunk (inner- ing anteriorly, then pushing forward (e.g.
vated directly by C5 spinal nerve roots). against a wall)

circle) for needle electrode examination of the (low) cer-
vical paraspinal muscles
useful as a reference level], and place the nee-

circle) for needle electrode examination of the serratus dle about two to four centimeters laterally,
anterior with the tip of the electrode oriented medially
towards the deeper muscle layers (the verte-
• Needle placement: In the mid-axillary line, bral lamina being deep to this) (see Fig. 3.31).
identify and isolate a rib at about the mid- • Indications include: To differentiate between
thoracic level, placing two fingers in between an intraspinal canal process (e.g. radiculopa-
adjacent intercostal spaces, and then inserting thy or anterior horn cell disorder) versus a
the needle perpendicular to the skin until gen- lesion distal to the dorsal root ganglia (which
tly abutting the bony rib (see Fig. 3.30). will affect corresponding sensory nerve action
• Indications include: Long thoracic neuropa- potentials as well). These muscles may also be
thy, brachial plexopathy, C5–C7 radiculopa- affected in many proximal myopathies, having
thy (especially in conditions associated with high diagnostic yield for certain disorders
medial scapular winging) (e.g. Pompe disease).
• Notes and precautions/confounders: If the • Notes and precautions/confounders: Needle
needle is inserted in the intercostal space, it examination that is too deep may irritate the
may pierce the pleura and lead to pneumotho- bony vertebral laminae with resultant exces-
rax. If the needle insertion is too close to the sive pain. It is not necessary to always have
inferior border of the rib, it may injure the the patient activate these muscles, as it is gen-
adjacent neurovascular bundle. erally uncomfortable/difficult to execute and
the evaluation of recruitment and motor unit
morphology is seldom further helpful in most
Cervical Paraspinal Muscles studies, except in patients with myopathies,
and motor neuron diseases.
• Innervation: Posterior/dorsal rami of the cer-
vical spinal nerves. Overlap innervation of C4
through C8/T1 spinal nerve root segments Lower Extremity
(overlap may occur for up to 4–6 contiguous
segments/levels). Extensor Digitorum Brevis
• Activation: Neck extension (e.g. pushing head
back against the examiner’s hand). Innervation: Deep Peroneal (fibular) nerve ←
• Needle placement: Identify the spinous pro- Common Peroneal (fibular) nerve ← Sciatic nerve
cess at the level of interest [the vertebra prom- ← Lumbosacral trunk and Posterior division of
inens (C7) at the base of the neck may be the Sacral Plexus ← L5–S1 spinal nerve roots.
the tibialis anterior; if inserted too laterally it will

be in the peroneus tertius.
Peroneus Tertius
Innervation: Deep Peroneal (fibular) nerve ←

Common Peroneal (fibular) nerve ← Sciatic
nerve, Lumbosacral trunk and Posterior division
of the Sacral Plexus, L5 > S1 spinal nerve roots.
Fig. 3.32 Needle insertion site (black dot in center of red Activation: Patient to dorsiflex and evert the
circle) for needle electrode examination of the extensor foot.
digitorum brevis
Needle placement: one handbreadth above the
bimalleolar line of the ankle and two finger
Activation: Patient to extend the toes. breaths lateral to the tibia (see Fig. 3.34).
Needle placement: Three finger breadths Indications include: L5 > S1 radiculopathies,
distal to the lower border of the lateral malleo- deep or common peroneal (fibular) neuropathy.
lus parallel to the border of the foot (see Potential Confounders: If electrode inserted
Fig. 3.32). too medially will be in the extensor hallucis lon-
Indications include: entrapment/injury of the gus, if inserted too proximally it will be in the tibi-
common peroneal (fibular) nerve (e.g. at the fibu- alis anterior, or the extensor digitorum longus.
lar head), Anterior tarsal tunnel syndrome, L5, S1
radiculopathies.
Potential Confounders: Interpretation of
abnormalities from this muscle needs to be taken
with care as it is common to find features of
chronic denervation in normal subjects without
any symptoms. This may be attributable to the
effects of chronic local trauma, as can be seen
with footwear/shoewear.
Extensor Hallucis Longus Fig. 3.33 Needle insertion site (black dot in center of red
circle) for needle electrode examination of the extensor
hallucis longus
nerve, Lumbosacral trunk and Posterior division
of the Sacral Plexus, L5 > S1 spinal nerve roots.
Activation: Patient to extend the big toe.
Needle placement: Three to five finger-
breadths above the bimalleolar line of the ankle
just lateral to the crest of the tibia (see Fig. 3.33).
Indications include: L5 > S1 radiculopathies,
deep or common peroneal (fibular) nerves, often
abnormal in peripheral neuropathies.
Potential Confounders: If electrode inserted Fig. 3.34 Needle insertion site (black dot in center of red
circle) for needle electrode examination of the peroneus
too superficially and too proximal, it will be in tertius
Tibialis Anterior

nerve ← Lumbosacral trunk and Posterior divi-
sion of the Sacral Plexus ← L4, L5 spinal nerve
roots.
Activation: Patient to dorsiflex the foot.
Needle placement: Four fingerbreadths below
the tibial tuberosity and one fingerbreadth lateral Fig. 3.36 Needle insertion site (black dot in center of red
to the tibial crest (see Fig. 3.35). circle) for needle electrode examination of the peroneus
longus
Indications include: L4, L5 radiculopathies,
deep or common peroneal (fibular) or sciatic
neuropathies.
Potential Confounders: If electrode inserted
too laterally and to deeply will be in the extensor
digitorum longus.
Peroneus Longus
Innervation: Superficial Peroneal (fibular) nerve

← Common Peroneal (fibular) nerve ← Sciatic
nerve ← Lumbosacral trunk and Posterior divi- Fig. 3.37 Needle insertion site (black dot in center of red
sion of the Sacral Plexus, L5 > S1 spinal nerve circle) for needle electrode examination of the abductor
roots. hallucis
Activation: Patient to evert the foot.
Needle placement: Three fingerbreadths
below the fibular head (see Fig. 3.36). Abductor Hallucis
Indications include: L5 > S1 radiculopathies,
superficial or common peroneal (fibular) or sci- Innervation: Medial Plantar nerve ← Tibial nerve
atic neuropathies. ← Sciatic nerve ← Anterior division of the Sacral
Potential Confounders: If electrode inserted Plexus, S1 > S2 spinal nerve roots.
too posterior it will be in the soleus, if inserted Activation: Patient to spread the toes.
too anteriorly it will be in the extensor digitorum Needle placement: One fingerbreadth below
longus. the navicular bone on mid portion of the medial
aspect of the foot (see Fig. 3.37).
Indications include: peripheral neuropathy,
medial plantar nerve lesions, tarsal tunnel syn-
drome, other tibial neuropathies, sciatic neuropa-
thy, sacral plexopathy, S1 > S2 radiculopathies.
too distally it will be in the flexor hallucis brevis,
if inserted too deep it will be in the flexor digito-
rum brevis. Interpretation of abnormalities from
this muscle needs to be taken with care as it is
circle) for needle electrode examination of the tibialis common to find features of chronic denervation
anterior in normal subjects without any symptoms. This
may be attributable to the effects of chronic local

trauma, as can be seen with footwear/shoewear.
Flexor Hallucis Brevis
Innervation: Medial Plantar nerve ← Tibial nerve

← Sciatic nerve ← Anterior division of the Sacral
Plexus, S1, S2 spinal nerve roots.
Activation: Patient to flex the great toe.
Needle placement: Proximal and medial to the Fig. 3.39 Needle insertion site (black dot in center of red
tendon of the flexor hallucis longus (see Fig. 3.38). circle) for needle electrode examination of the flexor digi-
Indications include: peripheral neuropathy, torum longus
medial plantar nerve lesions, tarsal tunnel syn-
drome, other tibial neuropathies, sciatic neuropa- Activation: Patient to flex the toes without
thy, sacral plexopathy, S1, S2 radiculopathies. flexing the ankle.
Potential Confounders: If electrode inserted Needle placement: Just posterior of the medial
too laterally it will be in the adductor hallucis, if edge of the tibia at midpoint (see Fig. 3.39).
inserted too medially it will be in the abductor Indications include: tibial neuropathy, sciatic
hallucis. Interpretation of abnormalities from this neuropathy, sacral plexopathy, L5 > S1 radicu-
muscle needs to be taken with care as it is com- lopathies. Useful in the evaluation of a foot drop
mon to find features of chronic denervation in to differentiate either a sciatic neuropathy or an
normal subjects without any symptoms. This intraspinal process (particularly at L5) from a
may be attributable to the effects of chronic local peroneal (fibular) neuropathy.
trauma, as can be seen with footwear/shoewear. Potential Confounders: If electrode inserted
too superficial it will be in the soleus, if inserted
too deep it will be in the tibialis posterior
Flexor Digitorum Longus (although this is also tibial/L5 > S1-innervated).
Innervation: Tibial nerve ← Sciatic nerve ←

Anterior division of the Sacral Plexus, L5 > S1 Iliopsoas or Iliacus
spinal nerve roots.
Innervation: Femoral nerve ← Posterior division
of the Lumbar Plexus ← L2, L3 (L4) spinal nerve
roots.
Activation: Patient to flex the hip.
Needle placement: Two fingerbreadths lateral
from the femoral artery pulse and one to two fin-
gerbreadths below the inguinal ligament (see
Fig. 3.40).
Indications include: high/proximal femoral
neuropathy, posterior division lumbar plexopa-
thy, L2, L3, L4 radiculopathies, myopathies
(proximal muscle typically with high yield for
myopathic changes in affected patients).
Potential Confounders: If electrode inserted too
circle) for needle electrode examination of the flexor hal- medially it will contact the neurovascular bundle,
lucis brevis if inserted too laterally it will be in the sartorius.
if inserted too laterally it will be in the long head

of the biceps femoris.
Long Head of Biceps Femoris
Innervation: Sciatic nerve (Tibial divi-

sion) ← Anterior division of the Sacral Plexus ←
S1 > L5 spinal nerve roots.
Activation: Patient to flex the knee.
Fig. 3.40 Needle insertion site (black dot in center of red Needle placement: Insert the needle at mid-
circle) for needle electrode examination of the iliopsoas/ point between the lateral knee and the ischial
iliacus tuberosity (see Fig. 3.42).
Indications include: sciatic neuropathy,
Anterior division sacral plexopathy, S1 > L5
radiculopathies.
Potential Confounders: if needle electrode is
inserted too medially, it will be in the short head
of the biceps femoris.
Semimembranosus
Innervation: Sciatic nerve (Tibial divi-

sion) ← Anterior division of the Sacral Plexus ←
Fig. 3.41 Needle insertion site (black dot in center of red L5 > S1 spinal nerve roots.
circle) for needle electrode examination of the short head
Activation: Patient to flex the knee and inter-
of biceps femoris
nally rotate the tibia.
Needle placement: Three finger breadths
Short Head of Biceps Femoris proximal to the medial knee and medial (though
can be lateral as well) to the semitendinosus ten-
Innervation: Sciatic nerve (Peroneal (fibular) don (see Fig. 3.43).
division) ← Posterior division of the Sacral
Plexus ← S1 > L5 spinal nerve roots.
Activation: Patient to flex the knee.
Needle placement: Three finger breadths
proximal to the lateral knee and medial to the
long head of biceps femoris tendon (see
Fig. 3.41).
Indications include: sciatic neuropathy,
S1 > L5 radiculopathies.
Of note, this is considered the only muscle
above the knee innervated by the peroneal (fibu-
lar) division of the sciatic nerve. Important to
check in suspected peroneal (fibular) Fig. 3.42 Needle insertion site (black dot in center of red
neuropathy. circle) for needle electrode examination of the long head
Potential Confounders: If electrode inserted of biceps femoris
too medially it will be in the semimembranosus,
Indications Include: sciatic neuropathy, Indications include: sciatic neuropathy,

Anterior division sacral plexopathy, L5 > S1 Anterior division sacral plexopathy, L5 > S1
radiculopathies. radiculopathies.
Potential Confounders: If electrode inserted Potential Confounders: If electrode inserted
too laterally it will be in the semitendinosus and too lateral it will be in the long head of biceps
if inserted further laterally it will be in the short femoris, if inserted too medial or too deep it will
head of the biceps femoris; if inserted too deeply be in the semimembranosus.
will be in the adductor magnus.
Tensor Fascia Lata

Semitendinosus
Innervation: Superior gluteal nerve ← Posterior
Innervation: Sciatic nerve (Tibial divi- division of the Sacral Plexus ← L5 > S1 spinal
sion) ← Anterior division of the Sacral Plexus ← nerve roots.
L5 > S1 spinal nerve roots. Activation: Patient to internally rotate thigh
Activation: Patient to flex the knee and inter- (this muscle is also a weak hip abductor).
nally rotate the tibia. Needle placement: Two finger breadths ante-
Needle placement: Midpoint between the rior to the greater trochanter (see Fig. 3.45).
ischial tuberosity and the medial knee (superfi- Indications include: Superior gluteal neuropa-
cial to the semimembranosus) (see Fig. 3.44). thy, Posterior division sacral plexopathy, L5 > S1
radiculopathies.
too anterior it will be in the sartorius or rectus
femoris, if inserted too deep it will be in the
vastus lateralis, if inserted too posteriorly it will
be in the gluteus medius (although this is also
superior gluteal/L5 > S1-innervated).
Gluteus Medius
Fig. 3.43 Needle insertion site (black dot in center of red Innervation: Superior gluteal nerve ← Posterior
circle) for needle electrode examination of the division of the Sacral Plexus ← L5 > S1 spinal
semimembranosus nerve roots.
circle) for needle electrode examination of the circle) for needle electrode examination of the tensor fas-
semitendinosus cia lata
circle) for needle electrode examination of the gluteus circle) for needle electrode examination of the gluteus
medius maximus
Activation: Patient to abduct the thigh. Potential Confounders/Pitfalls: Iatrogenic

Needle placement: With the patient in the lat- injury to the sciatic nerve is the major issue to
eral decubitus position, contralateral side down, avoid. This tends to occur if needle insertion is
the needle is inserted about two finger breadths too lateral or inferior to the site recommended
posterior to the mid-point between the summit of above.
the iliac crest and the greater trochanter (see
Fig. 3.46).
Indications include: Superior gluteal neuropa- Vastus Lateralis
thy, Posterior division sacral plexopathy, L5 > S1
radiculopathies. Innervation: Femoral nerve ← Posterior division
Potential Confounders: If the needle is inserted of the Lumbar Plexus ← (L2)L3–L4 spinal nerve
too anteriorly, it may be in the tensor fascia lata roots.
(although this is also superior gluteal/ Activation: Patient to extend and gently lock
L5 > S1-innervated). the knee, tightening and pressing the knee
towards the examination table/bed, or your hand,
which may be placed underneath the knee (patient
Gluteus Maximus being in supine position). If necessary, may ask
the patient to elevate the heel off the bed.
Innervation: Inferior gluteal nerve ← Posterior Needle placement: lateral aspect of the upper
division of the Sacral Plexus ← S1(S2) > L5 spi- thigh, anterior to the groove formed between the
nal nerve roots. outer portion of the hamstrings and vastus latera-
Activation: Patient to extend the hip/thigh lis (see Fig. 3.48).
with the knee flexed; accentuation may also be Indications include: Femoral neuropathy,
produced by asking the patient to tighten the but- Lumbar plexopathy (especially posterior divi-
tock muscles. sion), (L2)L3–L4 radiculopathies.
Needle placement: Superior external buttock Potential Confounders/Pitfalls: If the needle is
quadrant (as shown), or about 2 finger breadths placed too laterally, it may go into the iliotibial
lateral to the midpoint between the posterior band (which is in the groove between the vastus
superior iliac spine (at sacral dimple) and the top lateralis and the hamstrings, on the lateral aspect
of the natal cleft (see Fig. 3.47). of the thigh), and this may cause avoidable pain/
Indications include: Inferior gluteal neuropa- discomfort. If the needle is inserted too medially,
thy, Posterior division sacral plexopathy, S1 > L5 it may enter the rectus femoris (although this is
radiculopathies. also femoral/(L2)L3–4-innervated).

circle) for needle electrode examination of the rectus
circle) for needle electrode examination of the vastus
femoris
lateralis
Rectus Femoris
Innervation: Femoral nerve ← Posterior division

of the Lumbar Plexus ← (L2)L3–L4 spinal nerve
roots.
Activation: Patient to extend the knee and flex
at the hip (this muscle spans both the knee and
hip joints.
Needle placement: Anterior aspect of the
thigh, at the midpoint between the knee and ante-
rior superior iliac spine (see Fig. 3.49).
Indications include: Femoral neuropathy, Fig. 3.50 Needle insertion site (black dot in center of red
Lumbar plexopathy (especially posterior divi- circle) for needle electrode examination of the adductor
sion), (L2)L3–L4 radiculopathies. longus
Confounders: If too distal of a needle insertion,
the muscle become thin and more tendinous and thy (affecting the anterior division) from a femo-
tends to be painful, with risk of abutting the distal ral mononeuropathy.
femoral bone/periosteum. If needle insertion is too Activation: Patient to adduct the thigh.
lateral or too medial, the vastus lateralis or vastus Needle placement: Proximal medial thigh,
medialis (respectively) may be entered—although about three to four finger breadths distal to the
both are also femoral/(L2)L3–4-innervated. pubic tubercle (see Fig. 3.50).
Indications include: Obturator neuropathy,
Lumbar plexopathies (especially anterior divi-
Adductor Longus sion), L2–L4 radiculopathies.
Potential Confounders: If the needle is placed
Innervation: Obturator nerve ← Anterior division too distally, the adductor longus is no longer
of the Lumbar Plexus ← L2–L4 spinal nerve superficial, and the adductor magnus may be
roots. entered. The latter muscle is partially innervated
Note: Needle examination of this muscle is by the sciatic nerve (tibial division) as well, and
often helpful in differentiating a lumbar plexopa- may provide misleading data.
Lumbar Paraspinal Muscles
Innervation: Posterior/dorsal rami of the lumbar

spinal nerves. Overlap innervation of L1 through
S1 spinal nerve root segments (overlap may occur
for up to 4–6 contiguous segments/levels).
Activation: Hip extension. Alternatively, may
ask the patient to arch the trunk backwards (spine
extension).
Needle placement: Identify the spinous pro-
cess (using as a reference, the L3–L4 level in
between the posterior superior iliac crest on
either side), and place the needle about two to
four centimeters laterally, with the tip of the elec-
trode oriented medially towards the deeper mus-
cle layers (the vertebral lamina being deep to
this) (see Fig. 3.51). Fig. 3.51 Needle insertion site (black dot in center of red
Indications include: To differentiate between circle) for needle electrode examination of the (low) lum-
an intraspinal canal process (e.g. radiculopathy bar paraspinal muscles
or anterior horn cell disorder) versus a lesion dis-
tal to the dorsal root ganglia (which will affect except in patients with myopathies, and motor
corresponding sensory nerve action potentials as neuron diseases.
well). These muscles may also be affected in
many proximal myopathies, having high diag-
nostic yield for certain disorders (e.g. Pompe Suggested Reading
disease).
Potential Confounders/Pitfalls: Needle 1. Leis AA, Trapani VC. Atlas of electromyography.
Oxford: Oxford University Press; 2000.
examination that is too deep may irritate the 2. Perotto A, Delagi EF. Anatomical guide for the electro-
bony vertebral laminae with resultant excessive myographer: the limbs and trunk. 5th ed. Springfield,
pain. It is not necessary to always have the IL: Charles C. Thomas Publisher; 2011.
patient activate these muscles as it is generally 3. Lee HJ, DeLisa JA, Lee HJ. Manual of nerve conduc-
tion study and surface anatomy for needle electro-
uncomfortable/difficult to execute and the eval- myography. In: Surface anatomy for clinical needle
uation of recruitment and motor unit morphol- electromyography. 4th ed. Philadelphia: Lippincott
ogy is seldom further helpful in most studies, Wilkins and Williams; 2005.
Introduction to Needle
Electromyography 4
Bryan Tsao
What Do We Measure resolve with 10 s of onset, is termed “snap,

with the Needle EMG? crackle, pop”. This is more often found in
younger, healthy, muscular males, more fre-
A single motor unit includes one anterior horn quently in the lower limbs than upper limbs, and
cell (AHC), its axon process and terminal most commonly in the medial gastrocnemius
branches, neuromuscular junction, and muscle muscle [3]. Abnormal increased insertional activ-
fibers. The electrical activity of motor units ity includes trains of positive sharp waves and
recorded with a needle electrode or motor unit fibrillation potentials, sometimes irregular in
action potentials (MUAPs), is the sum of action their firing frequency, that last more than 300 μsec
potentials generated by those muscle fibers that but are non-sustained.
fire singly or in groups near the electrode [1]. In Opposite of increased insertional activity is
contrast to the NCS which assess both motor and decreased insertional activity, identified when
sensory nerves, the needle EMG only assesses the needle is moved through electrically inactive
the integrity of the motor unit, but is a more quan- tissue, e.g., subcutaneous adipose, edema, or
titative method for doing so (Table 4.1). necrotic or fibrotic muscle. Certain neuromuscu-
The needle EMG search contains two por- lar conditions associated with disorders of glyco-
tions: searching at rest for spontaneous activity gen metabolism (i.e., myophosphorylase,
and assessing with muscle activation MUAP phosphofructokinase deficiency) as well as ion
appearance and recruitment. When the needle is channel defects during episodes of periodic
moved within resting muscle, muscle fiber paralysis can also result in decreased insertional
discharges are induced that result in normal activity or electrical silence [3].
insertional activity, recognized by its sharp, Spontaneous activity is defined as discharges
distinct, and brief sound. Normal insertional that occur without being triggered by needle
activity lasts less than 200–300 μsec after needle movement and continue longer than 200–
movement stops [3–5] (Fig. 4.1). 300 μsec or indefinitely. Normal increased spon-
A benign variant of normal insertional activity taneous activity is seen when the needle tip
comprised of irregularly firing discharges, often approximates the neuromuscular junction gener-
in the form of positive sharp waves that typically ating end-plate spikes (from the terminal axon)
and end-plate noise (from the release of mini
B. Tsao (*) end-plate potentials); this is interpreted by
Department of Neurology, Loma Linda University patients as a particularly strong aching or painful
School of Medicine, Loma Linda, CA, USA sensation (Figs. 4.2 and 4.3).
e-mail: btsao@llu.edu

https://doi.org/10.1007/978-3-030-74997-2_4
66 B. Tsao
Table 4.1 Advantages and limitations of nerve conduc-

tion studies and the needle EMG [2]
Advantages Subclinical detection of demyelinating
lesions
Less uncomfortable, requires less
cooperation
Highly sensitive in differentiating axon
loss from demyelination
Can locate focal demyelinating lesions
Limitations Routine studies primarily assess the
distal nerves
Certain sensory responses may be lost
with age
Less sensitive for axon loss
Needle EMG
Advantages Subclinical detection of axon loss
lesions
Allows for more widespread
examination of the peripheral nervous
system
Can diagnose myopathy
Limitations Requires patient cooperation and is
generally more uncomfortable Fig. 4.2 End-plate spikes (raster plot)—the baseline is
Does not evaluate sensory fibers normal and the initial negative (upward) deflection of the
Insensitive for demyelinating lesions potential distinguishes it from spike fibrillation potentials
(which have an initial positive or downward deflection)
Fig. 4.1 Normal insertional activity—short duration dis- Fig. 4.3 Endplate spikes and endplate noise—note
charges triggered by needle movement (indicated by blue increased baseline “hiss” or low amplitude waveforms
arrows), associated with a sharp, discrete, and brief sound. compared to a normal smooth baseline as well as the
Normally, this activity lasts less than 200–300 μsec after initial negative (upward) deflection of the endplate spike
the cessation of needle movement potential
4 Introduction to Needle Electromyography 67
Fasciculation potentials are MUAPs that fire During low levels of muscle contraction,
in a spontaneous manner singly or in groups and MUAPs are assessed for amplitude (peak-to-
are characterized by their irregular rate. It is often peak), duration, number of phases (baseline
said that “fasciculations are only as bad as the crossings plus one; normal is four or less), and
company they keep”. Accordingly, fasciculation serrations or turns (changes in waveform
potentials are normal when they occur in isola- deflection without baseline crossing) (Fig. 4.14a,
tion, and even when abundant are most often seen b). Each muscle has its own morphology or
with benign fasciculation or the benign cramp- characteristic MUAP appearance related to the
fasciculation syndrome. While fasciculations ratio of the muscle fibers innervated by a single
potentials may be the initial manifestation of motor neuron/AHC and to the way the muscle’s
amyotrophic lateral sclerosis or grouped into end plate zone is laid out in the muscle belly [3].
myokymic potentials as part of focal or general- For example, MUAPs in normal gluteus maximus,
ized myokymic conditions, it is only when they biceps, brachioradialis, iliacus, frontalis,
are accompanied by other abnormal findings in obicularis oris, obicularis oculi, and paraspinal
sufficient distribution (such as evidence of wide- muscles tend to have MUAPs with shorter mean
spread denervation and reinnervation), should duration and increased number of phases, with up
they be considered abnormal (Fig. 4.4). to 10–30% of normal MUAPs having more than
Abnormal spontaneous activity spontaneous five phases [4]. In contrast, MUAPs in the triceps,
comes in many forms and includes fibrillation vastus lateralis, and tibialis anterior tend to have
potentials, positive sharp waves, myotonia, myoky- a slightly longer duration. Normal duration
mia, neuromyotonia, complex repetitive discharges, ranges from ≤10–15 msec and MUAP amplitude
cramps, tremor, and electrical artifact, each is typically ≤2–3 millivolts.
described in Table 4.2 and illustrated in Figs. 4.5, Age is another factor that affects MUAP dura-
4.6, 4.7, 4.8, 4.9, 4.10, 4.11, 4.12 and 4.13. tion such that broad MUAPs of slightly increased
Fig. 4.4 Complex fasciculation potential on a background of small positive sharp wave potentials (shown on the lower
right rastered screen)
68 B. Tsao
Table 4.2 Types of spontaneous activity [4] Table 4.2 (continued)

Type Generator Characteristics Type Generator Characteristics
End-plate Terminal Biphasic with initial Artifact Pacemaker Small very regular
spikes axon negative deflection, (among spikes (pacemaker)
irregular other types)
End-plate noise Mini High-pitched hissing AHC anterior horn cell, CNS central nervous system, CRD
end-plate complex repetitive discharge, MUAP motor unit action
potentials potential
Fasciculation AHC, nerve Simple or polyphasic
potentials > muscle MUAP appearance,
irregular/random
rates varying from patient. The regulation of surface temperature
0.005 Hz to many per during the EDX study is essential not only for
minute NCSs but also needle EMG. Cooling results in
Fibrillation Muscle Triphasic (positive- delayed inactivation of sodium channels in nerve
potentials fiber negative-positive)
and muscle and increased duration of action
potentials. Rarely
irregular but not as potentials so that an increase in MUAP amplitude
irregular as end-plate and duration is expected [3]. Cooling of the mus-
spikes cle will increase the amplitude and duration of
Positive sharp Muscle Mono or biphasic waveforms while cooling of the nerve may inhibit
waves fiber wave, fires regularly or
irregularly spontaneous firing and reduce the discharge fre-
Myotonia Muscle Brief biphasic or quency of spontaneous neuronal discharges [6].
fiber triphasic spikes that Thus, it is imperative that the limbs be main-
fire between tained within the same temperature range as
20–100 Hz with a
desired for NCS (>32 °C for the upper and
waxing and waning
(crescendo and >30 °C for the lower limbs, per American
decrescendo) pattern; Association of Neuromuscular and
positive sharp positive Electrodiagnostic Medicine laboratory
wave form induced by
accreditation guidelines).
needle insertion
Myokymia Groups of Regular or semi-
The theory behind MUAP recruitment is
motor units regular bursts of straightforward but the ability to consistently
normal MUAPs at judge MUAP recruitment takes considerable
0.1–10 Hz experience and is one of the more difficult EDX
Neuromyotonia Motor units High frequency (up to
skills to acquire. MUAPs are recruited in an
300 Hz) discharges
with characteristic orderly manner based on the Henneman size
‘pinging’ sound principle which refers to the orderly successive
CRD Muscle Groups of simple or activation of MUAPs such that small, “weak”
complex spike patterns type I motor units are activated first in early or
(via ephaptic
transmission) that
minimal contraction, and sequentially larger,
regularly repeat at “stronger” type II motor units are called up to
0.3–150 Hz deliver an increase in muscle power [3]. Initial
Cramp Multiple Fire synchronously MUAP recruitment is best assessed with mini-
motor units between 40 and 60 Hz,
mal activation when most MUAPs represent the
rarely up to
200–300 Hz smaller motor units that comprise type I muscle
Tremor Motor Correlates with the fibers [7] (Fig. 4.15). With minimal volitional
units/CNS type of tremor contraction, a single MUAP begins to fire at a
origin frequency of around 5 Hz. With increased effort
and when the firing frequency of the first MUAP
duration in a 75 year old may be normal for the reaches 10 Hz, a second MUAP is recruited.
patient’s age but may be abnormal for a younger With continued increased effort, when the firing
Fig. 4.5 Fibrillation potentials (positive wave form)—note the low amplitude (~200 μV), short duration (~8 ms), reg-
ularly-firing potentials
Fig. 4.6 Many positive sharp wave potentials—note the initial positive or downward deflection from baseline
frequency of the first potential reaches 15 Hz, a motor units for the rate of firing frequency and
third MUAP is recruited, and so forth. Thus, for force produced, implying reduced recruitment,
every 5 Hz increase in firing frequency of the most commonly due to neurogenic disease in the
original MUAP, an additional MUAP is recruited. form of axon loss (Fig. 4.16) or demyelinating
This is referred to as the 5:1 recruitment ratio or conduction block. The sound differences
the rule of 5’s. When the recruitment ratio is between normal MUAP recruitment or severely
increased, especially to 10:1, there are too few reduced recruitment is easily recognized by the
70 B. Tsao
Fig. 4.7 Myotonic potentials—note the positive sharp arrow) and then recurrence of myotonic potentials (white
wave morphology with a transition comprising reduced outline arrow), hence the typical waxing and waning
frequency and amplitude of the waveform (solid white profile
Fig. 4.8 Myokymic potentials—note the semi-rhythmic firing of grouped motor unit potentials occurring in bursts,
typically producing a “marching soldiers” sound
Fig. 4.9 Neuromyotonia—note the spontaneous, brief, very high-frequency discharge (~180 Hz) which tapers off in
amplitude to create the characteristic “pinging” sound
Fig. 4.10 Complex repetitive discharges (CRDs)—note the regularly repeating potentials with inter-potential duration
of ~10 ms (~100 Hz frequency, particularly fast in this example). These have typical abrupt onset and cessation
Fig. 4.11 Cramp potentials—note MUAPs firing syn- Fig. 4.12 Tremor tracing—normal MUAPs activated in a
chronously (typically 40–60 Hz), correlating with invol- very intermittent but regular manner, corresponding with
untary painful contraction of the muscle being examined the frequency of the underlying tremor
72 B. Tsao
examiner can feel and judge the amount of force

that is being sustained by the patient while assess-
ing the number of displayed MUAPs.
Incomplete MUAP recruitment can also result
from poor volitional muscle contraction, most
often in the setting of pain-limited effort or func-
tional weakness. It can also be seen in the pres-
ence of pre-existing upper motor neuron disorders
(e.g., with myelopathy, prior stroke, multiple
sclerosis, etc.) when patients are unable to acti-
vate muscles effectively on command. Incomplete
MUAP recruitment from poor effort or an upper
motor neuron disorder can be indistinguishable
and appear as either intermittent firing, or firing
at slow frequencies despite maximal effort,
referred to as “slow firing MUAPS”. Whenever
Fig. 4.13 Artifact from an implanted cardiac pacemaker
this is present, the electromyographer can only
device (detected while recording spontaneous activity in attest that recruitment of the number of MUAPs
the lumbar paraspinal muscles)—note the small, very present is appropriate to the degree the muscle is
regular spikes corresponding to the pacemaker setting of activated [8].
60 beats per minute
seasoned EDX medicine consultant. However, it How Do We Perform These

becomes increasingly difficult to judge progres- Measurements?
sively less severe degrees of reduced MUAP
recruitment. One commonly used method of During needle EMG the typical display sweep
MUAP recruitment defines four grades of speed is set at 10 msec/division. The sensitivity is
recruitment, where 4R = only a single MUAP set to 50 uV/division when searching for sponta-
(severely or profoundly reduced); 3R = 2–3 neous activity and 200 uV when assessing for
MUAPs (markedly reduced); 2R = 4 or more MUAP appearance and recruitment. To better
MUAPs (moderately reduced); and 1R = just visualize abnormally large MUAPs, the sensitiv-
less than normal but not as reduced as the 2R ity setting may have to be temporarily adjusted to
designation, i.e., mildly reduced. Another anno- 1000 uV or 1 mV [4]. Needle EMG may employ
tation for 4R is single MUAP (SMU). In prac- either of two types of needles: concentric or
tice, most EDX medicine consultants do not monopolar (Table 4.3 and Fig. 4.18a, b). For con-
routinely calculate recruitment ratios or the fir- centric needles with a range of 23–25 gauge, any-
ing frequency of MUAPS. Instead, the degree of where from 8 to 20 muscle fibers belonging to
abnormal recruitment is judged by a combina- same motor unit contribute to the MUAP [3].
tion of visual and auditory recognition. Hence The ideal time frame in which to perform
the semi-quantitative nature of the needle EMG. NCS and needle EMG depends on the suspected
With muscle disorders there is a drop out of pathophysiology and condition at hand (see
muscle fibers and a reduction in contractile force Chap. 2). For any peripheral condition causing
per motor unit. This results in a decreased recruit- weakness, the needle EMG can performed from
ment ratio (below 4:1) or the activation of too the moment weakness is present. It is, however,
many MUAPs for the degree of muscle contrac- unable to differentiate between axon loss and
tion, termed early (sometimes also referred to as demyelinating conduction block as the caus-
“rapid”) recruitment [4] (Fig. 4.17). The best ative pathophysiology when done too early. For
way to determine early recruitment is if the EDX example, if we perform the needle EMG in a
a b
Turns
Amplitude
Amplitude
Baseline
crossing
Phase
Baseline
crossings
Duration
Duration
Fig. 4.14 (a) Normal motor unit action potential boxes or 10.2–10.3 ms (normal is generally <15 ms). (b)
(MUAP) at 200 μV sensitivity and 10 ms sweep speed. Complex MUAP with increased duration and phases, at
Phases equal the number of baseline crossings (single 200 μV sensitivity and 10 ms sweep speed. The duration
white arrow) +1. In this MUAP, there are 2 baseline is over two boxes wide or >20 ms, while the number of
crossings +1 = 3 phases. Amplitude measures the entire phases is at least six baseline crossings (solid white arrow)
vertical dimension of the MUAP, and in this illustration +1 = 7 total (normal generally 4 or less). Turns or
spans about just under six boxes or just under 1200 μV or serrations are changes in deflections without baseline
1.2 mV (normal is <2–3 mV). MUAP duration is measured crossings and are seen pointed out with the open arrow.
from the onset of initial baseline deflection to the final The amplitude of this MUAP spans nearly eight boxes or
return to baseline, measuring about one and a quarter 1600 μV or 1.6 mV, still within normal limits
muscle immediately after an acute axon lesion or In this setting, NCS may determine if there is a
immediately after a focal demyelinating lesion focal demyelinating conduction block as long as
appears, the needle EMG will show a reduction proximal and distal stimulation (with the lesion
in MUAP recruitment that is proportional to the in between these sites) is possible, and 5–7 days
degree of motor axon loss or conduction block. have passed to allow for Wallerian degeneration
74 B. Tsao
Fig. 4.15 Normal motor unit action potentials (MUAPs) within a normal recruitment pattern
Fig. 4.16 Chronic motor axon loss/neurogenic recruit- number of recruited MUAPs (i.e. <4–5 MUAPs total, with
ment pattern with complex MUAPs of increased duration recruitment ratio >5)
and phases, high firing frequency of >30 Hz, with reduced
Fig. 4.17 Typical EMG findings in myopathy—note MUAPs at relatively low levels of contraction effort is
MUAPS of decreased duration, reduced amplitude, and consistent with “rapid” or “early” MUAP recruitment
increased phases (polyphasia). The large number of
Table 4.3 Comparison of concentric and monopolar needle EMG alone will not be able to differenti-
needle electrodes [9] ate between acute axon loss and demyelinating
Concentric Monopolar conduction block and would have to be repeated
Recording 20–100 μm2 100–500 μm2 after at least 3 weeks has passed in order to
surface appreciate the development of fibrillation poten-
Active On beveled edge Larger needle
electrode of needle tip tip surface
tials. Waiting until 4–5 weeks have passed since
Reference Needle shaft Surface the onset of symptoms (in particular weak-
electrode electrode ness) increases the yield of the study as certain
Patient Lower Higher patients may not manifest significant fibrilla-
tolerance tion potentials at precisely 21 days. In short, a
MUAP Lower Higher few conditions are amendable to needle EMG
amplitude
and NCS in under 3 weeks from target symp-
MUAP Shorter Longer
duration toms onset, including acquired demyelinating
LFF setting 10 Hz 20 Hz polyneuropathies and other focal demyelinating
HFF setting 10–20 kHz 20 kHz conditions, e.g., acute demyelinating polyradic-
Cost Higher Lower uloneuropathy, radial nerve compression at the
μm micrometers, MUAP motor unit action potential, LFF spiral groove (and similar entrapment mono-
low frequency filter (high-pass), HFF high frequency neuropathies), and differentiating demyelinat-
filter (low-pass)
ing conduction block from axon loss in facial
neuropathy due to Bell’s palsy.
to occur. This would exclude acute axon loss The art of conducting the needle EMG relies
effects which may transiently mimic a conduc- on anticipating whether or not all the muscles
tion block (what constitutes a so-called “acute that ideally need to be examined can be examined.
discontinuity lesion”). However, the acute-stage The EDX consultant must factor patient tolerance
76 B. Tsao
a b
Fig. 4.18 (a) Picture of a concentric EMG needle of (darker) insulation material. (b) Picture of a monopolar
(75 mm × 0.6 mm)—note, as seen in the magnified view EMG needle (25 mm × 0.35 mm)—note, as seen in the
in the inset (right lower corner), there is an outer cannula magnified view in the inset (right lower corner), there is
or “sleeve” of metal (E2 electrode) that is the external an outer “sleeve” of green insulation material on the
needle shaft, and the bevel tip discloses an internal metal surface of the needle shaft and the “trocar” tip exposes a
wire shaft (E1 electrode) separated by an interposed layer single metallic electrode (E1) surface
and prioritize which muscles to study based on You may withdraw the needle to the
their diagnostic yield. Here are some guidelines subcutaneous layer and then reinsert into the
for performing the optimal needle EMG: muscle during contraction and withdraw the
needle similarly prior to muscle relaxation.
1. Educate the patient on what is about to take 5. If the muscle is difficult to localize on the sur-
place, preferably using the term pin instead of face, first assess for MUAP configuration and
needle. recruitment before spontaneous activity so
2. Position the patient comfortably—they may that you know you’re in the desired muscle.
need extra pillows; the room must be warm, This method is also preferred when searching
slightly darkened, and quiet; and the limb muscles near vital structures, e.g., first have
positioned where maximum muscle relaxation the patient activate to reliably localize the
can occur. serratus anterior (to avoid pneumothorax) or
3. Start with high-yield and accessible muscles the flexor pollicis longus (to avoid radial
e.g., the triceps if cervical radiculopathy is arterial puncture/hematoma).
suspected or tibialis anterior if lumbosacral 6. Always finish the EDX examination with
radiculopathy is suspected. It may also be brief post-study instructions (covering typi-
prudent to not start with muscles that are well- cal post-EMG sequelae, which are usually
known to be rather sensitive/painful (e.g. the benign and self-resolving), letting the patient
abductor pollicis brevis). You may routinely know when results are expected to be posted
assess various muscles in a specific order, but to the ordering provider, help the patient sit
be ready to adapt the study if it looks like up, and offer to assist with dressing (or call
patient tolerance is wearing thin. in a gender appropriate assistant) and safe
4. While inserting the needle, some EDX medi- departure.
cine consultants like to say, “Here comes a
little pinch” or other verbal clues to alert the Additional recommendations are listed in
patient and either simultaneously pinch, tap, Table 4.4.
or stretch the skin as a sensory distraction The needle EMG examination typically
(pain-gating theory). Avoid having the length includes a single insertion into the muscle of
of the muscle significantly change (e.g. by choice, followed by 4–6 brief needle movements
having the patient fully contract or relax) or searches that are divided into four quadrants of
while the needle is considerably intramuscular. each muscle. There should be at least at least 2 s
Table 4.4 Additional guidelines on performing the needle EMG

Upper limb
– For extensor indicis proprius or other finger/wrist extensors, gently support the volar surface of the wrist in
pronation to produce a ‘limp’ hand
– Palpate each muscle with contraction prior to inserting the needle no matter how obvious their location,
particularly in patients with excess subcutaneous/adipose tissue
– Study the biceps brachii by inserting lateral or just medial of the midline (to avoid the intramuscular septum)
– The anconeus, although oftentimes tender, is a high-yield C7-innervated muscle and useful with radiculopathy
work-ups when the triceps is uninvolved
– When assessing cranial-innervated muscles, always study the genioglossus last, and never insert a needle used to
assess this muscle (when employing the intraoral/transmucosal approach) into another muscle. This minimizes
bacterial translocation and infection risk
Lower limb
– Study the flexor digitorum longus instead of the posterior tibialis or have the patient co-contract both (ask patient
to dorsiflex the toes while internally rotating the ankle)
– Save the intrinsic foot muscles, if indicated, for last
– The tensor of fascia lata may be more accessible than gluteus medius in patients with large hips
Either
– Activate the antagonist muscle if necessary to produce transient relaxation
– Support the neck and knees with pillows with slight neck and knee flexion and have the patient gently contract
the abdominal/anterior neck muscles (or push the spine backwards) to obtain paraspinal muscle relaxation
between each search to distinguish between Table 4.5 Safety guidelines for the needle EMG
normal insertional activity induced by needle Physician safety guidelines
movement and abnormally increased insertional – Never recap the needle using both hands
– Always recap the needle when moving the patient or
activity. The amount of needle searches may be
performing any task that requires both hands
increased or decreased, depending on the level of – The physician should always recap and dispose of
suspicion for abnormalities and how the patient is the needle immediately after the study is complete
tolerating the examination. To assess for MUAP – Always dispose of the needle after studying the
tongue/genioglossus muscle (when employing the
recruitment, the patient is first asked to perform a
intraoral/transmucosal approach) and use a new needle
minimal voluntary contraction with specific if additional muscles need to be studied
directions on how to activate the muscle against – Always use two pairs of gloves when assessing
resistance. Analyze single MUAPs before patients with known transmissible infections
(including hepatitis, HIV, and any other potential
requesting full muscle contraction which is
blood-borne pathogens)
usually reserved for the end of the search. With – Remove gloves when leaving the room and replace
maximal contraction in a normal muscle, the with new gloves prior to continuing the needle EMG
screen should be filled with overlapping MUAPs – Always provide patient pre- and post-needle EMG
instructions
such that analysis of the firing frequency and
– If a contaminated needle stick occurs, ask the patient
configuration of individual MUAPs is difficult, if to remain available for consent for blood draw/
not impossible (thus, this is referred to as a “full potential blood draw as it pertains to ruling out
interference pattern”). transmissible infections
Ensure that you and the personnel in the EDX
laboratory are well-versed on needle EMG safety
guidelines. An example of physician safety extensive needle EMG on multiple limbs or large,
guidelines is presented in Table 4.5. Growing evi- deep muscle groups in patients with coagulopa-
dence supports that performing the needle EMG thy. Note is also made of the risks of needle EMG
in anticoagulated patients is relatively safe [10, in patients with lymphedema. In such scenarios
11]. However, we still leave it to the discretion of judicious muscle selection is also required to
individual electrodiagnostic consultants on minimize risk of protracted oozing (of serous
whether or not they feel comfortable performing fluid) and infection.
78 B. Tsao
hat Do the Measurements Mean?

W atrophy, or (less typically) chronic
How Do Different Diseases Affect radiculopathy.
These Measurements? With disorders of neuromuscular junction
(NMJ) transmission, the needle EMG reflects find-
MUAP abnormalities correlate with the location ings that may be similar to myopathic diseases,
of pathology along the peripheral neuro-axis including MUAPs of short duration, small ampli-
(Table 4.6). Neurogenic changes, e.g., fibrilla- tude, and increased phases or turns. Specifically,
tion potentials and MUAPs of increased ampli- the MUAPs seen with NMJ diseases reflect the
tude, duration, polyphasia, and reduced variability in NMJ transmission as evident by a
recruitment are present with disorders of the change in the morphology of individual MUAPs.
AHC, nerve root, plexus, or peripheral nerve. When assessed using conventional concentric or
Normal MUAP duration varies with each muscle monopolar needle electrodes, this finding is also
tested but a general rule of thumb is that duration referred to as moment-to-moment amplitude varia-
ranges from ≤10–15 msec and MUAP amplitude tion (MMAV) or jiggle (Fig. 4.19a, b), in contrast
≤2–3 millivolts [3]. With reinnervation, an to jitter which is seen on single fiber electromyog-
increased MUAP duration is typically correlated raphy. The presence of unstable MUAPs is an
with an increase in phases but not necessarily a abnormal but non-specific finding and can be seen
proportional increase in amplitude. Moreover, an with early re-innervation, muscle or NMJ trans-
interpretation of a study being abnormal should mission disorders, and segmental demyelinating
not rely on visualization of increased polyphasic polyneuropathies [3]. Use of the term “myopathic
MUAPs alone without correlative increases in MUAPs” is discouraged since there are multiple
duration or amplitude or a reduction in MUAP causes for MUAPs of short duration, low ampli-
recruitment [8]. Markedly increased MUAP tude, and increased polyphasia (e.g. nascent units
amplitude of 8–10 millivolts or greater invari- seen in some cases of re-innervation). Thus, a
ably represents chronic neurogenic states in description of the MUAP configuration in the nee-
which reinnervated has occurred over years, e.g., dle EMG results section with a separate statement
remote poliomyelitis, late-onset spinal muscular that the findings are consistent with myopathy
Table 4.6 Patterns of abnormality seen with the needle EMG

Disorder MUAP duration Recruitment Variation/MMAV
Anterior horn cell (ALS) Decreased/increased Reduced Yes/No
Acute radiculopathy Normal Variable/reduced No
Chronic radiculopathy Increased Variable/reduced No
Acute PN Normal Reduced No
– Axon loss Normal Reduced Yes/No
– Demyelinating
Chronic PN Increased Reduced No
– Axon loss Normal Increased/Reduced Yes/No
– Demyelinating
Myasthenia gravis Normal or decreased Normal Yes
LEMS Normal or decreased Normal Yes
Botulism Normal or decreased Normal Yes
Early myopathy Decreased Normal No
Late to severe myopathy Decreased/increased Early/reduced No/Yes
MUAP motor unit action potential, ALS amyotrophic lateral sclerosis, PN polyneuropathy, LEMS Lambert-Eaton
Myasthenic Syndrome, MMAV moment-to-moment amplitude variation
Adapted from [1]
Fig. 4.19 (a) Complex MUAP disclosing instability MUAP from top-to-bottom. Note the variability of the
(also referred to as moment-to-moment amplitude MUAP complexity each time it fires. This is also
variation, MMAV) on the left (5 ms sweep speed), rastered highlighted in the superimposed tracings of the same
on the right (2 ms sweep speed) displaying the same MUAP shown in (b)
under the interpretation portion of the EDX exam- As with radiculopathy, the electrodiagnostic
ination report is more appropriate than simply stat- diagnosis of myopathy primarily relies on the
ing that “myopathic” MUAPs are present. needle EMG. However, the sensitivity of the nee-
80 B. Tsao
dle EMG for diagnosing muscle disorders is vari- ow Do These Measurements

H
able and the specificity is low. Correlate with Motor NCS?
With myopathy, the earliest MUAP change
due to muscle fiber loss is a reduction in dura- Both needle EMG and motor NCS assess motor
tion, followed by increased polyphasia or turns fibers, but the needle EMG is more sensitive in
and reduced amplitude. Early or increased the detection of motor axon loss, i.e., loss of a
recruitment becomes apparent when there is single motor axon will yield fibrillation potentials
functional loss of muscle fibers within a motor if the needle is adjacent to the denervated muscle
unit so that more muscle fibers and contraction fibers, whereas it is estimated that approximately
is required to generate a given force. Although 50% of motor axons within a motor unit must be
early recruitment is one of the most reliable fea- lost before there is an appreciable reduction in
tures of myopathy, it is often only present with compound muscle action potential (CMAP)
moderate to severe disease and, thus, is not an amplitude. With increasing severity of motor
early EDX manifestation [3]. With myopathy, axon loss, there is an increase in fibrillation
fibrillation potentials indicate there is loss of potentials and reduction of MUAP recruitment.
muscle fiber connectivity to its end plate and When reinnervation occurs either in the form of
supports the presence of inflammation or necro- collateral sprouting or axonal regeneration
sis of the muscle fiber. However, the presence of (usually after ~3 months have passed), MUAPs
fibrillation potentials do not always mean that with increased duration, amplitude and phases
inflammation will be found on muscle biopsy appear. MUAPs with greatly increased amplitude
due to sampling and the patchy nature of some (of >3–4 millivolts) signify a very long-standing
inflammatory myopathies [3]. Similarly, the process and are typically seen in patients with
absence of fibrillation potentials does not indi- remote poliomyelitis or other AHC or root level
cate that inflammation or necrosis is absent due disease.
to needle sampling, the non-uniform nature Whenever focal demyelination is present and
of inflammatory myopathies, and the fact that the stimulating electrode can be placed proximal
inflammatory changes may be obscured by treat- and distal to the site of demyelination, the motor
ment with steroids or other immunomodulating NCS can localize the focal conduction defect
therapy. Myotonic potentials are the next most with a good degree of accuracy. However, if focal
common spontaneous activity seen with myopa- demyelination disrupts nerve conduction
thy, yet are non-specific, being compatible with propagation to a sufficient degree that weakness
a wide range of myopathies [1]. results and the stimulator can only be placed
Certain myopathies may result in a combina- distal and not proximal to the block, then the
tion of both neurogenic-appearing and myo- distal CMAP will be normal despite clinical
pathic-appearing MUAPs. A classic example is deficits. Thus, when NCS are conducted after
inclusion body myositis, a chronic myopathy in 5–7 days—the amount of time it typically takes
which local inflammation results in denervation Wallerian degeneration of the distal nerve
and reinnervation of the muscle fibers as well as segment to occur after focal axonal injury—there
desychronization and slowing of distal terminal is weakness of the recorded muscle and the distal
nerve branches [3]. As a result, there are MUAPs CMAP is of normal amplitude, then the likely
of small duration, short amplitude, and increased pathophysiology is demyelinating conduction
phases intermixed with MUAPs of increased block proximal to the stimulation site. Assuming
duration, high amplitude, and increased phases. there is sufficient demyelinating conduction
The MUAP firing pattern may also be com- along the nerve to the weak muscle, the needle
prised of a mixture of early and reduced EMG in muscles innervated by that nerve
recruitment. segment will demonstrate MUAPs that have
normal appearance but are reduced in number in ment and the analysis of MUAP configuration—
proportion to the number of blocked motor nerve including duration, amplitude, and phases. The art
fibers. This combination of a normal CMAP in a of performing needle EMG to maximize patient
weak muscle and reduced MUAP recruitment tolerance and diagnostic yield includes using a
allows for the EDX medicine consultant to deter- variety of patient education, sensory/pain distrac-
mine indirect evidence of proximal demyelinat- tors, and prioritizing studied muscles. The study
ing conduction block (so-called “inferred” can be performed safely in virtually all patients
block). This has both diagnostic and treatment but should be used with caution in patients with
implications as incorrectly diagnosing motor lymphedema and anti-coagulation.
axon loss carries a poorer prognosis while persis-
tent proximal demyelinating conduction block
can be associated with treatable acquired poly- References
neuropathies (e.g., multifocal motor neuropathy
with conduction block). 1. Daube JR. Assessing the motor unit with needle
electromyography. In: Clinical neurophysiology.
Another scenario in which the needle EMG is Philadelphia: FA Davis Co; 1996. p. 257–281.
useful is when there is no clinical weakness in a 2. Chemali KR, Tsao B. Electrodiagnostic testing of
muscle which has a low or reduced CMAP ampli- nerves and muscles: when, why, and how to order.
tude yet the needle EMG reveals normal MUAP Cleveland Clin J Med. 2005;72:37–48.
3. Levin KH. Needle electrode examination. In: Levin
appearance and recruitment. In this instance, the KH, Luders HO, editors. Comprehensive clinical
best alternative explanation is that the muscle is neurophysiology. Philadelphia: WB Saunders; 2000.
receiving its innervation from another nerve p. 122–39.
which should clue the EDX medicine consultant 4. Dumitru D. Needle electromyography. In: Dumitru
D, Amato AA, Zwarts MJ, editors. Electrodiagnostic
to perform additional NCS for anomalous medicine. 2nd ed. Philadelphia: Hanley & Belfus;
innervations. 2002. p. 257–91.
Finally, when there is chronic motor axon loss 5. Preston D, Shapiro B. Basic electromyography: anal-
followed by adequate reinnervation, the CMAP ysis of spontaneous activity. In: Electromyography
and neuromuscular disorders. 2nd ed. Boston:
may be normal in amplitude despite prior loss of Butterworth-Heinemann; 2005. p. 199–213.
motor unit function. When muscle fibers recorded 6. Rutkove SB. AANEM minimonograph #14: the
on motor NCS include those re-innervated by effects of temperature in neuromuscular electrophysi-
surrounding motor units, the amplitude is normal ology. Muscle Nerve. 2001;24:867–82.
7. Preston D, Shapiro B. Basic electromyogra-
despite varying degrees of reduced MUAP phy: analysis of motor unit action potentials. In:
recruitment and large polyphasic MUAPs seen Electromyography and neuromuscular disorders.
on needle EMG. 2nd ed. Boston: Butterworth-Heinemann; 2005.
p. 215–29.
8. Dumitru D. Electrodiagnostic medicine pitfalls.
In: Dumitru D, Amato AA, Zwarts MJ, editors.
Summary Electrodiagnostic medicine. 2nd ed. Philadelphia:
Hanley & Belfus; 2002. p. 541–77.
The needle EMG relies on the assessment for 9. Campbell WW. Needle electrode examination. In:
Essentials of electrodiagnostic medicine. Baltimore:
spontaneous activity and MUAP recruitment and Williams & Wilkens; 1999. p. 93–116.
appearance to evaluate the integrity of the motor 10. Lynch SL, Boon AJ, Smith J, Harper CM Jr, Tanaka
unit and together with NCS, can localize and diag- EM. Complications of needle electromyogra-
nose any number of neuromuscular disorders from phy: hematoma risk and correlation with antico-
agulation and antiplatelet therapy. Muscle Nerve.
the intraspinal canal, nerve roots, plexus, periph- 2008;38:1225–30.
eral nerve, neuromuscular junction, and muscle. 11. Boon JA, Bertken JT, Watson JC, Laughlin
Recognition of both normal and abnormal sponta- RS, Strommen JA, Mauermann ML, Sorenson
neous activity is imperative as is the advanced skill EJ. Hematoma risk after needle electromyography.
Muscle Nerve. 2012;45:9–12.
of semi-quantitative assessment of MUAP recruit-
Mononeuropathies
5
Ramon Lugo and Alexandra Soriano
Mononeuropathies, especially those seen with In the upper extremity, the most common
entrapment are among the most common condi- mononeuropathies include median neuropathy at
tions investigated with the use of electrodiagnostic the wrist (carpal tunnel syndrome), and ulnar neu-
(EDX) studies. These studies not only help to iden- ropathy at the elbow. In the lower extremity, it is
tify the single peripheral nerve involved, but also the peroneal (fibular) neuropathy at the fibular head.
site of the lesion, the type of fibers involved (sen- Causes of mononeuropathies include mechanical
sory and/or motor), the character of the lesion (axo- injury caused by compression or trauma like fractures
nal vs demyelinating), and the age/chronicity of the or joint dislocations, repetitive activities like those
lesion. There is also the potential to obtain prognos- seen in typists or carpenters, endocrine disorders like
tic information, including features of ongoing rein- diabetes and hypothyroidism, local tumors like
nervation in axon loss lesions. The most distinctive Schwannomas, pregnancy, prolonged limb posture
localizing features occur with focal demyelination during surgical procedures, among many others.
(as seen in many entrapment mononeuropathies). Most mononeuropathies involve sensory and
These include the presence of conduction block motor fibers (potentially affecting corresponding
and/or focal conduction slowing, with or without sensory and motor responses on NCS), but some
concomitant axon loss features. However, these fea- are purely motor including the anterior interos-
tures may not always be found, and in some cases seus neuropathy, posterior interosseus neuropa-
lesions are not able to be precisely localized with thy, long thoracic neuropathy and spinal
only findings of axonal loss on nerve conduction accessory neuropathy. Others may be purely sen-
studies (NCS) and needle electromyography sory, like lateral femoral cutaneous neuropathy
(EMG) in muscles innervated by the affected single (meralgia paresthetica), and superficial radial
nerve. However, knowledge of the anatomy of the sensory neuropathy (cheiralgia paresthetica).
course of individual peripheral nerves, and their The EDX study is also very helpful to differ-
branches which supply specific muscles allows the entiate between mononeuropathies and other pat-
electromyographer to at least localize to a particular terns of injury affecting the peripheral nervous
segment of the nerve in most cases. system including radiculopathies, plexopathies,
and polyneuropathies.
R. Lugo (*)
Cleveland Clinic Florida, Weston, FL, USA
e-mail: lugor@ccf.org
Median Nerve
A. Soriano
Florida, Weston, FL, USA The sensory fibers of the median nerve come
from the cervical root levels 5 and 6 while the
Western Reserve University, Cleveland, OH, USA motor fibers come from the cervical root levels
e-mail: soriana@ccf.org

https://doi.org/10.1007/978-3-030-74997-2_5
84 R. Lugo and A. Soriano
of C6–T1. This separation of the sensory fibers motor upper and middle trunk components
continue in the brachial plexus with the sensory entering into the lateral cord as well). Then,
fibers traveling only through the lateral cord. these median lateral and medial cord fibers join
The motor fibers travel through the lower trunk to form the median nerve proper. It passes
and medial cord for the C8–T1 components, and through the upper arm without branching or
follow along with the C6 median sensory fibers innervating any muscle. At the antero-cubital
through the upper trunk, with the C7 component area, it is adjacent to the brachial artery. Then it
arising from the middle trunk (these median passes in between both heads of the pronator
Fig. 5.1 Median nerve:

course and branches to
key muscles of
electrodiagnostic
importance
Median n.
Anterior
Interosseous n.
Pronator teres
Flexor digitorum Flexor carpi radialis
profundus
Palmaris longus
Flexor pollicis
longus Flexor digitorum
superficials
Pronator quadratus
Abductor pollicis
brevis
Flexor pollicis brevis
(superficial head)
Opponens pollicis
First lumbrical
Second lumbrical
5 Mononeuropathies 85
teres to the forearm (see Fig. 5.1). As it pierces Common risk factors for median neuropathy
the pronator teres it innervates this muscle. Then at the wrist include diabetes mellitus, hypothy-
it branches to innervate several muscles in the roidism, obesity, wrist trauma, pregnancy, and
anterior portion of the forearm before branching repetitive mechanical wrist movement/hand use
to originate the anterior interosseous nerve (a [1, 8–12]. Other causes include congenital small
pure motor branch), which innervates deeper caliber of carpal tunnel, use of vibrating tools,
forearm muscles (flexor pollicis longus, prona- renal failure, amyloidosis, and arthritis.
tor quadratus, and the lateral half of flexor digi- Electrodiagnostic studies can help confirm the
torum profundus). Before getting in the carpal diagnosis and assess the severity of the median
tunnel it gives the cutaneous palmar branch, that neuropathy. The nerve conduction study findings
supply cutaneous innervation to the thenar emi- include:
nence. After passing through the carpal tunnel it
gives sensory innervation to the first 3 digits and 1. Focal slowing across the carpal tunnel, mani-
the lateral half of the fourth finger. The motor fested by a prolonged peak latency of the sen-
branch innervates the first 2 lumbricals and the sory nerve action potential first, and then
thenar muscles including the opponens pollicis, decreased amplitude of the sensory response.
abductor pollicis brevis, and superficial part of The motor responses abnormalities include
flexor pollicis brevis (muscles supplied by the first prolonged onset distal latency of the com-
recurrent branch of the median nerve). pound motor action potential across the wrist,
The most common sites of entrapment are at and later on decrease amplitude of the motor
the carpal tunnel (wrist) [1–4] and while piercing response. Marked decreased amplitude may
the pronator teres muscle (pronator teres suggest a poorer prognosis.
syndrome).
The routine electrodiagnostic studies for a
median neuropathy at the wrist should include:
edian Neuropathy at the Wrist
M
(Carpal Tunnel Syndrome) 1. Motor nerve conduction of the median nerve
at the wrist and at the elbow while recording
It affects around 3–5% of Americans [1, 2, 5–7], at the abductor pollicis brevis.
with a peak incidence between 40 and 60 years 2. Ulnar motor studies with stimulation at the
old, and more frequent in females than male (3:1). wrist, below the elbow and above the elbow
Dominant hand tends to be affected first and more while recording at the abductor digiti
severely. The clinical syndrome is known as car- minimi.
pal tunnel syndrome and may include symptoms 3. Sensory nerve studies of the median and ulnar
of pain or paresthesia located in the wrist, hand, nerves must be done. Sometimes, in milder
fingers, at times in forearm (and even as proximal cases, comparison studies are necessary,
as the shoulder), this pain is usually worst with including comparison of latency between the
repetitive hand use, and at night, often awaking median palmar and ulnar palmar mixed nerve
patients. Paresthesia and sensory loss features are response, among others.
appreciated especially in the first three digits, and 4. Needle electromyography must include at
sometimes the lateral half of the fourth finger. least 2 C6–C7 innervated muscles, abductor
Weakness of the hand can also develop, particu- pollicis brevis, and 2 median-innervated
larly involving the median-innervated muscles of muscles proximal to the wrist and 2 C8–T1
the thenar eminence, with atrophy of these mus- non-median-innervated muscles.
cles seen in more severe cases.
nterior Interosseous Nerve (AIN)

A 1. Median motor and sensory responses which
Syndrome are either reduced or absent. A normal median
peak/distal latency or conduction velocity at
This is a predominantly motor syndrome, with the wrist segment can help distinguish this
presence of weakness of the thumb and index fin- from carpal tunnel syndrome.
ger with difficulty pinching items between these 2. On needle examination, there may be features
two fingers due to weakness of the lateral half of of denervation of all the median-innervated
flexor digitorum profundus and flexor pollicis muscles distal to the pronator teres, with rela-
longus muscles (patient unable to make the “OK tive sparing of the pronator teres.
sign” with fingers). Pain can be present, but sen-
sory loss should not. The place of entrapment of
this nerve is variable, but mostly at the forearm. Ulnar Nerve
Nerve conduction studies may be normal, or
show reduced amplitudes of median motor The sensory and the motor fibers of the ulnar
responses. EMG will show denervation changes nerve originate at the level of C8 and T1 nerve
in the flexor pollicis longus, flexor digitorum pro- roots, then traveling through the inferior/lower
fundus to second and third digits, and/or pronator trunk and in the medial cord before forming the
quadratus. ulnar nerve proper (see Fig. 5.2). In the arm
(above elbow) it does not provide any innervation
and travels together with the brachial artery, vein
Median Mononeuropathy and the median nerve. Then passing through the
at the Proximal Medial Forearm ulnar groove at the elbow between the medial epi-
(Pronator Teres Syndrome) condyle of the humerus and the olecranon process
of the ulna. It enters the forearm through the apo-
When the median nerve passes in between the neurotic arcade known as the cubital tunnel. There
two heads of the pronator teres muscle it can be it provides innervation to the flexor carpi ulnaris
compressed at this level due to various reasons muscle, and travels in its belly, exiting later to
including, hypertrophied pronator teres muscle, innervate the flexor digitorum profundus to the
repetitive pronation/supination motion, acute ring and little fingers. At about the mid-forearm it
forceful pronation, anomalous arteries, postoper- gives the dorsal cutaneous branch to the dorsal
ative scarring, and compression on the proximal surface of the medial one and a half fingers, and
forearm by heavy bag with narrow handles the associated dorsal hand area, separate from the
(grocery-bag neuropathy). Compression of the palmar cutaneous branch to the medial aspect of
median nerve in the proximal forearm can also the palm including the proximal aspect of the ring
occur at the edge of the flexor digitorum sublimis and little fingers. The final sensory component is
(flexor digitorum superficialis) arch. the superficial branch which arises in the hand
The clinical symptoms with entrapment of the itself and innervates the palmar surface of the
median nerve at these proximal forearm sites are medial one and a half fingers. There is no consis-
like those of carpal tunnel syndrome with pares- tent sensory innervation from the ulnar nerve to
thesias mostly in the second and third digits, but the forearm itself. When the ulnar nerve reaches
lack of nocturnal worsening of the symptoms, the wrist, it passes through the Guyon’s canal,
with also dull ache around the forearm that is formed by the pisiform medially and the hook of
exacerbated with forced arm pronation. Physical the hamate laterally. There it divides in to superfi-
exam findings may include tenderness on palpa- cial and deep branches to intrinsic hand muscles.
tion of the pronator teres, increased pain and par- The most common sites of compression of the
esthesias with pronation of the arm, weakness and ulnar nerve are at the elbow (retrocondylar
atrophy of forearms flexors (typically sparing pro- groove and/or ulnar groove and cubital tunnel),
nator teres). Nerve conduction studies can show: and at the wrist (Guyon’s canal) [13–15].
Fig. 5.2 Ulnar nerve:

key muscles of
electrodiagnostic
importance
Ulnar n.
Flexor carpi ulnaris
Flexor digitorum profundus

(digits 4 and 5)
Adductor pollicis
Flexor pollicis brevis Digiti minimi:

(deep head)
-Abductor
First dorsal -Opponens
interosseous -Flexor
First palmar Fourth lumbrical

interosseous
Third lumbrical
Ulnar Neuropathy at the Elbow dislocation) or 1–2 cm distally at the cubital tun-
nel. At the retrocondylar grove this can be caused
Compression of ulnar nerve at the elbow can occur by repeated trauma such as leaning on the elbow,
either at the retrocondylar grove and/or ulnar sustained hyperflexion of the elbow, distal humerus
groove (between the medial epicondyle and the fractures or elbow dislocation, degenerative joint
olecranon, especially with nerve subluxation or disease, immobilization during surgery.
Patients may present with sensory symptoms chronic mechanical compression due to use of
and pain in the sensory distribution at the hand, crutches, compression in bicycle bars, etc.
including dorsum of the hand, with pain or ten- Compression at and around this site may
derness at the elbow, and when motor symptoms involve specific branches of the nerve and pro-
present: weakness and muscle wasting of the first duce various patterns as follows:
dorsal interosseous (FDI), abductor digiti minimi
(ADM) and/or other ulnar innervated muscles –– Type 1 (only subtype where lesion is actually
(including those in the forearm like the flexor in Guyon’s canal) involves compression of
carpi ulnaris and the flexor digitorum profundus both the deep and superficial branches of the
to digits 4&5). ulnar nerve. There is weakness in the intrinsic
The electrodiagnostic evaluation of an ulnar hand muscles and sensory loss in digits 4&5,
neuropathy must include motor nerve conduc- though there is sparing in the distributions of
tion studies of the ulnar nerve when recording at the palmar and dorsal ulnar cutaneous
the abductor digiti minimi and, if needed, the branches.
first dorsal interosseus and with stimulations at –– Type 2 is manifested by exclusive involve-
the wrist, below and above the elbow (with the ment of the deep branch, affecting the distal
elbow flexed at 90 degrees). Median nerve con- components of both the hypothenar branch
duction studies should be done as well. Median and the terminal branch (to the interossei and
and ulnar F-waves should be evaluated as well lumbricals). Accordingly, sensory deficits are
sensory responses of the median and ulnar absent. Compression in this scenario occurs
nerves. Dorsal ulnar and medial antebrachial distal to Guyon’s canal but proximal to the
cutaneous sensory responses may be beneficial hypothenar branch.
as well, depending on the need for further lesion –– Type 3 occurs when there is compression of
localization refinement. The needle electromy- the deep branch distal to the hypothenar
ography should include ulnar-innervated mus- branch, thereby sparing the hypothenar mus-
cles distal to the wrist, the forearm cles (the interossei and third and fourth lum-
ulnar-innervated muscles, other C8–T1 non- bricals are affected). Sensory function is not
ulnar innervated muscles, and C8–T1 (low cervi- affected.
cal) paraspinals. –– Type 4 is rare and comprises compression dis-
Electrodiagnostic findings may show: tal to Guyon’s canal involving the superficial
branch exclusively, so motor function is
1. Slowing of conduction velocity or conduction spared (exception being the palmaris brevis
block at the culprit segment (typically between muscle).
the above-elbow and below-elbow stimulation
sites). The role of electrodiagnostic studies is
2. Absent or reduced superficial sensory +/− essentially to rule out more common ulnar
dorsal ulnar cutaneous responses (both usu- nerve lesions, such as at the elbow. If there is an
ally affected when ulnar lesion is at the elbow, abnormality of the dorsal ulnar sensory
but fascicular sparing may occur). response, this may suggest a process/lesion
3. EMG abnormalities may be found in the FDI, proximal to the wrist. If the ulnar sensory
ADM, as well as the flexor carpi ulnaris and response, when recorded at the fifth finger is
flexor digitorum profundus (to digits 4&5)— affected and there is a normal dorsal ulnar sen-
ulnar-innervated intrinsic hand and forearm sory response, this favors an ulnar lesion distal
muscles usually affected when ulnar lesion is to the mid-forearm and most likely at the wrist.
at the elbow, but fascicular sparing may Sometimes to further assess the possibility of a
occur). palmar process affecting the ulnar nerve, com-
paring the onset latencies of the ulnar motor
Ulnar neuropathy at the wrist (Guyon’s canal). response when recorded at the abductor digiti
Entrapment at the wrist can be associated with minimi versus the first dorsal interoseous with
mass lesions such as ganglion cyst, lipomas, or wrist stimulation may be performed. More than
2 milliseconds in difference may be a discrimi- Radial Nerve

nating feature. On needle electromyography,
there may be signs of denervation in ulnar- The radial nerve receives fibers from C5 to C8.
innervated hand muscles with sparing of ulnar- These fibers arise from the posterior cord where
innervated forearm muscles. However, the terminal branch becomes the radial nerve
sometimes this pattern may also be seen in (remaining fibers after the axillary nerve
ulnar neuropathy at the elbow with fascicular branches off)—see Fig. 5.3. It travels posterior
sparing and close analysis of the nerve conduc- to the axillary artery and goes across the back of
tion studies will usually help with distinguish- the humerus through the spiral grove. In the arm
ing localization. it gives innervation to the triceps, anconeous
Fig. 5.3 Radial nerve:

key muscles of
electrodiagnostic
importance
Radial n.
Triceps
(long head)
Triceps
(lateral head)
Triceps
Brachioradialis (medical head)
Extensor carpi
radialis longus
Extensor carpi
radialis brevis Anconeus
Supinator Posterior
Extensor carpi ulnaris Interosseous n.
Extensor digitorum
Extensor digiti minimi
Abductor pollicis longus
Extensor pollicis longus
Extensor pollicis brevis
Extensor indicis Superficial radial n.
and brachioradialis. There it come across the teres, and flexor carpi radialis) is needed to
elbow and divides into the superficial and deep exclude a C7 radiculopathy.
branch of the radial nerve. The superficial
branch is purely sensory to the dorsum of the
hand lateral to the ring finger. The deep motor Radial Neuropathy at the Elbow
branch/posterior interosseous nerve (PIN) sup-
plies multiple muscles including the supinator, Compression of the radial nerve at the elbow hap-
extensor carpi ulnaris, extensor digitorum (com- pens when the posterior interosseus branch of the
munis), extensor digiti minimi, abductor polli- radial nerve pass in between the two heads of the
cis longus, extensor pollicis longus and brevis supinator muscle in the arcade of Frohse. This
and extensor indicis. may be due to repetitive supinator use with mus-
The radial nerve can be injured at multiple cle hypertrophy, fracture of the proximal radius,
levels, including the brachio-axillary angle due to nearby lipomas or other mass lesions. The symp-
use of crutches or proximal humeral fractures; at toms are purely motor, affecting mostly finger
the level of the spiral grove due to compression extension and sparing supination (with partial
or humeral fractures as well; at the level of the sparing of wrist extension).
supinator as it crosses this muscle, specifically Electromyography studies will show:
affecting the deep motor branch/PIN; or more
distally due to use of handcuffs or complications 1. Normal radial sensory response.
from IV infusions affecting mainly the superfi- 2. Abnormal radial motor nerve responses, with
cial sensory branch. possible reduction in conduction velocity with
motor amplitude.
3. Needle EMG with evidence of denervation in
adial Neuropathy at the Spiral
R all muscles supplied by the PIN, with normal
Groove triceps, anconeous, brachioradialis, extensor
carpi radialis which are supplied by the (more
Radial compression mononeuropathy at the spi- proximal) radial nerve.
ral grove can happen secondary to fracture of the
humeral shaft at the spiral groove, or by pro-
longed external compression of the nerve when Radial Neuropathy at the Wrist
the arm is over a chair or bed, including while
sleeping, this classically occurring in sedated/ When compressed at the wrist, the radial neuropa-
intoxicated patients (“Saturday night palsy”). thy is purely sensory (cheiralgia paresthetica), this
The clinical features of a radial compression typically caused by use of handcuffs, tight watches
at this level may include, weakness with wrist or bracelets. Patients present with sensory changes
and finger extension, weakness of supination, in the radial dorsum of the hand (mostly around
mild weakness with elbow flexion, with spearing first web space), with no motor involvement.
of elbow extension (triceps) and sensory loss of The electrodiagnostic studies typically dis-
the dorsum of the hand mostly in the vicinity of close a reduced or absent superficial radial sen-
the first web space. sory response, with normal radial motor studies
Electromyography findings include: and normal needle EMG findings.
1. Reduced or absent radial sensory responses.

2. On needle electromyography denervation fea- Peroneal (Fibular) Nerve
tures may be found in the brachioradialis,
wrist and finger extensors, with sparing of the The peroneal (fibular) nerve derives mostly from
triceps and anconeus. Needle EMG of non- the L5 and (to a lesser extent) S1 spinal nerve
radial-innervated C7 muscles (e.g. pronator roots. Parent fibers travel through the lumbosa-
cral plexus and becomes part of the sciatic nerve. superficial peroneal (fibular) nerve innervates
While passing through the thigh it supplies what mainly the foot evertors, also supplying sensa-
may be considered the only peroneal (fibular)- tion to the lateral calf and the dorsum of the foot.
innervated muscle proximal to the fibular head, The most common site of peroneal (fibular)
the short head of bicep femoris (alternatively compression neuropathy is at the knee, right at the
considered to be innervated by the peroneal (fib- fibular neck [16, 17]. At this location, both the deep
ular) division of the sciatic nerve). The sciatic and superficial peroneal (fibular) nerves are
nerve divides into the common peroneal (fibular) involved, and patients present with weakness in toes
and the tibial nerves behind the knee, above the and foot dorsiflexion, resulting in foot drop, and
popliteal fossa. It gives out the lateral cutaneous weakness in foot eversion. There are also sensory
nerve of the knee just before passing through the symptoms in the lateral aspect of the calf, and in the
fibular tunnel. It passes through the fibular tun- dorsum of the foot including the first web space.
nel and then branch into the superficial peroneal Peroneal (fibular) neuropathy at the fibular
(fibular) and deep peroneal (fibular) branches neck occurs secondary to direct trauma, stretch
(see Fig. 5.4). The deep peroneal (fibular) nerve injuries, or prolonged inadvertent compression in
innervates mainly the foot dorsiflexors while the patients following heavy sedation or anesthesia.
Ganglion cyst (intraneural) or a nerve sheet
tumor, repetitive leg crossing or repetitive squat-
ting can also be a cause/contributor.
Electrodiagnostic findings include:
1. In peroneal (fibular) motor studies there can

be focal slowing or conduction block across
the fibular neck. Any slowing 10 m/s or more,
or a reduction in amplitude of 20% or more
(with proximal/above fibular neck site stimu-
Common peroneal n. lation) may be considered significant.
Deep peroneal n. 2. Reduction of motor amplitudes are present
Superficial peroneal n.
when axonal loss occurs, typically in conjunc-
Tibialis anterior
Peroneus longus tion with reduction in the amplitude of the
Extensor
Peroneus brevis digitorum longus superficial peroneal (fibular) sensory nerve
action potential.
Extensor
hallucis longus
3. Focal slowing or conduction block in peroneal
Cutaneous branches (fibular) motor studies recording at the tibialis
to dorsum of foot anterior (TA) muscle, if this is not present
Peroneus tertius when doing motor studies at the extensor digi-
torum brevis (EDB).
4. Needle electrode evidence of denervation in
Extensor
digitorum brevis muscles supplied by the deep and superficial
peroneal (fibular) nerves. Other non-peroneal
(fibular) innervated muscles supplied by the
L5–S1 roots should be examined to rule out the
possibility of a more proximal sciatic neuropa-
thy, lumbosacral plexopathy or L5–S1 lumbar
radiculopathy. As part of this approach, perti-
nent muscles above the fibular neck (in particu-
Fig. 5.4 Peroneal (fibular) nerve: course and branches to lar, the short head of biceps femoris) should be
key muscles of electrodiagnostic importance tested to exclude a sciatic neuropathy.
5. Prolonged or absent peroneal (fibular) Fwave significant asymmetry of findings (when the
response may be seen. lesion is unilateral).
2. Needle EMG may show motor axon loss
changes in the tibial/plantar-innervated
Tibial Nerve intrinsic foot muscles, with sparing of the calf
muscles and the peroneal (fibular)-innervated
Most of the fibers from the tibial nerve come intrinsic foot muscles (like the extensor digi-
from the L5, S1 and some S2 roots. They travel torum brevis). Needle EMG of foot muscles
through the lumbosacral plexus and become the can be difficult to be tolerated by the patient,
tibial division of the sciatic nerve. Behind the but again, contralateral studies are strongly
knee, the sciatic nerve gives rise to the tibial recommended in order to increase diagnostic
nerve after the peroneal (fibular) division certainty (including the assurance that there
branches off (see Fig. 5.5). The tibial nerve travel are motor axon loss features on the affected
behind the calf innervating the plantar flexors and side beyond chronic denervation changes
foot invertor muscles before passing through the often seen in intrinsic foot muscles secondary
tarsal tunnel. It runs distal to the medial malleo- to chronic local trauma, as attributable to
lus beneath the flexor retinaculum on the medial footwear).
side of the ankle. In the tarsal tunnel, the tibial
nerve travels with the tibial artery, tendon of the
flexor hallucis longus, tibialis posterior and flexor
digitorum longus. Then the tibial nerve divides in
to the medial and lateral calcaneal sensory nerves,
providing sensation to the heel of the sole, and
the medial and lateral plantar mixed nerves pro- Tibial n. Common peroneal n.
viding innervation to medial and lateral sole of
the foot. These plantar mixed nerves innervate
muscles of the foot that can be tested during Gastrocnemius Gastrocnemius
EMG, such as the abductor hallucis (AH), flexor (medical head) (lateral head)
Popliteus
digitorum brevis (FDB), abductor digiti quinti
Soleus
pedis (ADQP) muscles. Tibialis posterior
Compression of the tibial nerve under the Flexor hallucis
Flexor digitorum longus
flexor retinaculum at the medial ankle, poten- longus
tially results in tarsal tunnel syndrome, which
can follow trauma (sprains or fractures), but has
also been attributed to inflammation or tumors
[18, 19].
The clinical picture in tarsal tunnel syndrome
is perimalleolar pain, ankle and sole burning pain Medial plantar n. Lateral plantar n.
that is worse with weight bearing, especially at

Abductor hallucis Flexor digitorum
night. Ankle jerk is spared as well as sensationin accessorius
the dorsum and lateral aspect of the foot. Flexor digitorum
Abductor digiti minimi
brevis
The electrodiagnostic findings include: Flexor digiti minimi
Abductor hallucis
1. Reduction in the tibial compound motor
First lumbrical Interossei and
action potentials when recording the abductor lumbricals
hallucis and abductor digiti quinti pedis mus-
cles. Contralateral examination is strongly Fig. 5.5 Tibial nerve: course and branches to key mus-
recommended in these cases to demonstrate cles of electrodiagnostic importance
Sciatic Nerve Accordingly, the clinical and electrodiagnos-

tic characteristics of sciatic mononeuropathies
The sciatic nerve is derived from the L4–S2 represent a combination of the individual features
nerve roots. It is essentially composed of com- seen with common peroneal (fibular) and tibial
partmentalized common peroneal (fibular) and mononeuropathies, with additional hamstring
tibial parent nerve fibers along its course, com- muscle involvement (muscles innervated by the
prising the lateral and medial divisions, respec- superior and inferior gluteal nerves, as well as the
tively. After exiting the pelvis at the sciatic femoral and obturator nerves spared).
notch, the nerve passes under the piriformis
muscle (see Fig. 5.6). As the sciatic nerve courses
through the posterior thigh, the tibial nerve com- Femoral Nerve
ponent fibers innervate several hamstring mus-
cles (semimembranosus, semitendinosus, and The femoral nerve comes from the lumbar
the long head of biceps femoris). The short head plexus and receives branches from L2, L3 and
of bicep femoris is considered the only ham- L4 nerve roots. Parent nerve fibers cross the pel-
string muscle innervated by the common pero- vis behind the psoas muscle, deep and above the
neal (fibular) nerve. The adductor magnus iliacus muscle, which it also supplies (see
(hamstring portion) is also innervated by the Fig. 5.7). It enters the thigh together with the
tibial component of the sciatic nerve (the adduc- femoral artery and vein. It divides into the ante-
tor portion of this muscle is supplied by the obtu- rior division which gives the medial and inter-
rator nerve). Of note, most lesions of the mediate cutaneous nerves of the thigh, and
proximal sciatic nerve (for example at the upper motor branches to sartorius and pectineus mus-
thigh and hip) tend to affect the lateral division/ cles; the posterior division innervates the quad-
common peroneal (fibular) nerve fibers. riceps femoris muscle, continuing then as the
saphenous (sensory) nerve.
Causes of femoral neuropathy include prolonged
compression during pelvic or abdominal surgeries,
Piriformis or during labor or delivery. Trauma to the nerve may
also occur in total hip replacement surgeries.
Retroperitoneal hemorrhages or hematomas of the
inguinal region may also injure the femoral nerve.
Sciatic n.
Patients typically present with symptoms of
sensory changes in the anterior/medial thigh, and
Semitendinosus medial calf, with weakness of the quadriceps and
Biceps femoris iliopsoas muscles, usually manifesting with knee
(long head) buckling/weakness of knee extension and weak-
Semimembranosus Biceps femoris
(short head) ness of hip flexion.
Adductor magnus Electrodiagnostic findings include:
Common peroneal n.
Tibial n. 1. Reduced femoral nerve motor amplitude (in
cases of axonal loss) recording at the quadri-
ceps muscle (rectus femoris or vastus latera-
lis). Side to side comparison is strongly
recommended to demonstrate significant
asymmetry and increase diagnostic certainty.
2. Reduced saphenous sensory nerve ampli-
Fig. 5.6 Sciatic nerve: course and branches to key mus- tudes. Similarly, side-to-side comparison
cles of electrodiagnostic importance studies are needed in these cases, with iso-
cerebellopontine angle, next to cranial nerve VIII

and both nerves enter the internal auditory
lliacus (iliopsoas)
meatus. Then the facial nerve (sensory compo-
nent) fibers pass through the geniculate ganglion
Femoral n.
Sartorius Pectineus before getting into the facial nerve canal. Some
of the parasympathetic fibers branches out to the
greater and lesser petrosal nerves to get to the
Quadriceps femories:
-Rectus femoris pterygopalatine and otic ganglia. The next fibers
-Vastus lateralis to branch out are the motor fibers which inner-
-Vastus intermedius vate the stapedius muscle in the ear. Next, the
-Vastus medialis
chorda tympani carry special sensory fibers for
taste supplying the anterior 2/3 of the tongue, and
parasympathetic fibers to the submandibular and
sublingual glands. The facial nerve then exits the
cranium through the stylomastoid foramen. After
Saphenous n. exiting, it innervates the posterior belly of the
digastric and the stylohyoid muscles. It then
gives the posterior auricular branch. At this point,
the facial nerve continues towards the face to
branch out into the five main branches (temporal,
zygomatic, buccal, mandibular and cervical).
The most common facial neuropathy is idio-
pathic Bell’s palsy. The etiology is still unclear,
Fig. 5.7 Femoral nerve: course and branches to key mus- although it is believed to be a neuritis caused by a
cles of electrodiagnostic importance viral infection, possibly herpes viruses. Other
possible etiologies for facial neuropathy include
lated bilaterally absent saphenous responses tumor, trauma or infectious [20, 21]. Bilateral
considered insufficient for diagnostic pur- facial neuropathies are less common but may be
poses (often technically difficult to obtain, seen with, Guillain-Barré syndrome or other
especially in overweight or obese patients). inflammatory/infectious processes affecting the
3. Needle EMG of the iliacus (for lesions above base of the brain such as Lyme disease, sarcoid-
the inguinal ligament) and two heads of the osis, or leptomeningeal lymphomatosis/
quadriceps muscle (usually rectus femoris and carcinomatosis.
vastus lateralis) typically shows motor axon The clinical manifestation of facial neuropa-
loss changes. Other L2–L4-innervated mus- thy depends on what level is the pathology.
cles must be sampled to exclude the presence Processes affecting the nerve as it exits the brain
of a lumbar plexopathy, or L2–L4 motor stem may affect not only the muscles of facial
radiculopathy. expression, but also taste in the anterior 2/3 if the
tongue, hearing (hyperacusis) and tearing (lacri-
mation) ipsilaterally. Processes affecting the
Facial Nerve nerve distal to the chorda tympani will affect only
the motor fibers.
The facial nerve (cranial nerve VII) originates Electrodiagnostic studies can be used to deter-
from the union of the axons coming from the mine the amount of axon loss associated with
facial motor nucleus (primarily motor fibers for facial nerve lesions, which may help in prognos-
facial expression muscles) and the nervus inter- tication. These studies typically employed are
medius (giving parasympathetic, taste, and non- facial nerve conduction studies with needle elec-
taste sensory fibers). It exits the brainstem at the tromyography and blink reflexes.
The muscle typically recorded for facial motor entrapment. If entrapment occurs at the supra-
nerve conduction studies is the nasalis. This mus- scapular notch, patients may develop shoulder
cle may also be examined by needle electrode, pain that may worsen with shoulder movement,
together with the frontalis, orbicularis oculus, weakness with shoulder abduction and external
orbicularis oris, and the mentalis muscles. rotation. Atrophy may be noted in the supraspi-
The nerve conduction studies should be done natus and infraspinatus muscles. If entrapment
at least 10 days after the onset of the neuropathy occurs distally at the spinoglenoid notch,
to allow for Wallerian degeneration to take place patients may develop relatively painless weak-
(to reduce the likelihood of false negative/con- ness and atrophy of the infraspinatus muscle
founding results). The blink reflexes may help to alone.
assess the portion of the facial nerve proximal to Differential diagnosis of suprascapular neu-
the stylomastoid foramen. The reader is referred ropathy includes cervical (C5–C6) radiculopathy,
to Chap. 1 for further details about blink reflex upper brachial plexopathy including neuralgic
testing. amyotrophy, rotator cuff tear and other musculo-
skeletal shoulder injuries.
Electrodiagnostic studies may help confirm
Less Common Mononeuropathies the diagnosis and assess the severity of the mono-
neuropathy. In these studies, needle EMG may be
Suprascapular Neuropathy useful in confirming and localizing the lesion,
and excluding a cervical radiculopathy. Nerve
The suprascapular nerve fibers arise from the conduction studies are particularly useful in
C5–C6 anterior horn cells and its spinal nerve excluding a brachial plexopathy (for e.g. by dem-
roots/segments passing through the upper trunk onstrating an intact lateral antebrachial cutaneous
of the brachial plexus. The nerve itself passes sensory response).
posterior to the trapezius muscle, then through Studies of nerves that pass through the upper
the suprascapular notch of the scapula (space trunk of the brachial plexus should be done
located in the superior border of the scapula cov- including the lateral antebrachial cutaneous
ered by the superior transverse scapular ligament. nerve, as well as the median and radial sensory
The nerve first supplies the supraspinatus muscle, nerves recording from the thumb. Other than
then traverses the spinoglenoid notch to innervate “routine” motor nerve conduction studies of the
the infraspinatus muscle. upper extremity, motor nerve conduction studies
Entrapment is more common at the suprascap- of the supraspinatus and infraspinatus can be
ular notch, and less common at the spinoglenoid done using a monopolar needle electrode in either
notch. Some causes for entrapment include repet- muscle for recording, and stimulating at the Erb’s
itive movement of the shoulder and scapula, point.
commonly observed in weight lifters, prolonged Needle EMG of the upper extremity including
positioning during surgical procedures, volley- the supraspinatus and infraspinatus should be
ball players, baseball pitchers and dancers. The done. Motor axon loss needle EMG abnormali-
nerve can also be involved in cases of neuralgic ties will typically be found in both the supraspi-
amyotrophy. Some masses such as ganglion natus and infraspinatus muscles if the nerve is
cysts, sarcomas or metastatic carcinomas may entrapped at the suprascapular notch. However,
cause entrapment of this nerve; rotator cuff tear these abnormalities will only be found in the
can potentially cause nerve compression due to infraspinatus muscle if the lesion is at the spino-
medial retraction of the tendons of the supraspi- glenoid notch. Other C5–C6 innervated muscles
natus and infraspinatus muscles. and the cervical paraspinals, should be sampled
Patients may present with various symptoms to rule out a cervical motor radiculopathy and/or
and exam findings, depending on the site of other intraspinal processes.
Axillary Neuropathy Musculocutaneous Neuropathy
The posterior cord of the brachial plexus gives From the lateral cord of the brachial plexus,
rise to the axillary nerve, with fibers coming with fibers coming from the C5–C6(+/-C7) ante-
from the C5–C6 anterior horn cells and spinal rior horn cells and spinal nerve roots/segments,
nerve roots/segments, passing through the passing through the upper trunk, the musculocu-
upper trunk. The axillary nerve then exits the taneous nerve innervates the brachialis, coraco-
axilla though a space delineated by the brachialis and biceps brachii muscles. Coursing
humerus, teres minor, teres major and long below the elbow it ends as the lateral antebrachial
head of the triceps muscles, called the quadri- cutaneous sensory nerve.
lateral space. Then it divides into two trunks, Mononeuropathies of this nerve are uncom-
the posterior trunk supplying the teres minor mon and can occur in cases of direct trauma, sur-
muscle as well as the posterior head of the del- gery, compression (e.g. prolonged resting head
toid muscle, terminating in the lateral brachial during sleep), or stretch injury (lifting a particu-
cutaneous nerve conveying sensation over the larly heavy weight). The lesion of this nerve can
lateral shoulder. The anterior trunk supply the cause symptoms of paresthesia in the lateral fore-
middle and anterior heads of the deltoid mus- arm, and weakness of elbow flexion. Rarely, the
cle and gives deep sensory branches to the lateral antebrachial cutaneous sensory nerve may
shoulder joint. be entrapped at the elbow between the biceps ten-
Common causes of axillary neuropathy don and the brachialis muscle, this leading to
include direct trauma, shoulder dislocations or increased pain or paresthesia in lateral forearm
humeral fractures. Symptoms include numbness with pronation and arm extension.
over the lateral shoulder and weakness of shoul- Electrodiagnostic studies are used to exclude or
der abduction and external rotation. The extent of isolate a mononeuropathy of this nerve, differenti-
clinical deficits may be quite variable across ating from a more widespread lesion like a bra-
cases. Additionally, brachial plexopathy can chial plexopathy, or cervical radiculopathy. For
present with axon loss features predominantly in this, motor neve conduction studies can be done by
the axillary nerve distribution. stimulating the musculocutaneous nerve at the
Electrodiagnostic studies should include sen- axilla and Erb’s point while recording at the biceps
sory nerves that run through the posterior cord muscle. For sensory studies, testing the lateral
and upper trunk of the brachial plexus, including antebrachial cutaneous response is essential. Side-
radial and lateral antebrachial cutaneous sensory to-side comparisons of these motor and sensory
nerves, and the median nerve recording at the responses is highly recommended.
thumb. Motor studies of the axillary nerve are In proximal lesions of the musculocutaneous
done by stimulating at the Erb’s point and record- nerve, needle EMG would typically show motor
ing with a surface electrode over the deltoid mus- axon loss changes in the biceps muscle. Other
cle. Reduction in the CMAP can be found, and muscles innervated by the upper trunk and lateral
side-to-side comparison should be done. A drop cord (e.g. pronator teres, flexor carpi radialis, del-
of more than 50% in CMAP amplitude (com- toid, brachioradialis, supraspinatus and infraspi-
pared to the contralateral/normal side) is consid- natus, and cervical paraspinals) should be tested
ered abnormal. as well to exclude a more widespread lesion e.g.
Needle EMG should be performed in the teres brachial plexopathy, or C5–C6 cervical motor
minor and deltoid muscles, as well as other mus- radiculopathy.
cles innervated by the upper trunk and posterior
cord of the brachial plexus (e.g. supraspinatus,
infraspinatus, biceps, triceps, brachioradialis, Long Thoracic Neuropathy
cervical paraspinals) to exclude a more wide-
spread lesion like a brachial plexopathy or a C5– This nerve arises directly from the cervical spinal
C6 cervical motor radiculopathy. nerve roots C5–C6–C7 and strictly speaking, does
not enter the brachial plexus. It innervates the ser- Lesion to this nerve occurs most commonly at
ratus anterior muscle only. This muscle produces the posterior cervical triangle, due to external
scapular protraction and stabilization, together with compression, stretch or following local surgery
augmenting upward rotation of the scapula. Damage such as cervical lymph node biopsy. This causes
to this nerve alone is relatively uncommon, but has weakness of the trapezius muscle resulting in
been described in cases of trauma to the cervical shoulder drop by affecting shoulder abduction. A
spine and chest wall, or stretch injuries as may shoulder drop may result in traction of the bra-
occur in a variety of sports and recreational activi- chial plexus leading to shoulder pain and pares-
ties, or even during surgical procedures/positioning. thesia, which may lead to the clinical suspicion
In some cases of neuralgic amyotrophy, it may also of brachial plexopathy. If the lesion is more prox-
be the only nerve involved. imal, then weakness of the sternocleidomastoid
Symptoms may include pain around the muscle is seen with difficulty turning head and
shoulder and weakness around the shoulder, neck to contralateral side (as well as having some
particularly affecting scapular stabilization weakness in neck flexion).
and producing scapular winging. This is best The electrodiagnostic studies of this nerve can
seen when the arm is placed (flexed) in front of be performed by stimulating posterior to the mid-
the body and the hand is pushed firmly against dle of the sternocleidomastoid, recording over
a wall, producing medial winging of the the upper trapezius. Comparison to the contralat-
scapula. eral side is recommended. Sensory nerves travel-
Needle EMG is most helpful when studying ing through the upper trunk of brachial plexus
this nerve, as there is no specific or reliable way [i.e. lateral antebrachial cutaneous, median
to stimulate this nerve exclusively. Abnormalities (recording at the thumb) and radial sensory]
should only be found in the serratus anterior mus- should be studied to exclude a more widespread
cle with isolated lesions, always using abundant process.
caution when inserting the needle to prevent iat- The EMG portion is useful in studying the tra-
rogenic pneumothorax with pleural puncture pezius and sternocleidomastoid muscles. When
(recommended to insert over the rib and not in studying the trapezius, caution should be taken
the interspace). One should always sample other not to insert the needle too deep and sample
C5–C6 and C7 muscles, as well as paraspinal underlying muscles, such as rhomboids or supra-
muscles to exclude a cervical motor radiculopa- spinatus. Other muscles (especially those inner-
thy or a brachial plexopathy. Nerve conduction vated by C5–C6 spinal nerve roots/segments)
studies should be aimed at ruling out a more should be tested including deltoid, infraspinatus,
widespread process involving the brachial plexus, supraspinatus and rhomboids and cervical para-
especially studying nerves that travel though the spinals, to rule out a more widespread process.
upper and middle brachial plexus and are derived
from the same roots [i.e. lateral antebrachial
cutaneous, median (recording at the thumb) and Sample Cases
radial sensory nerves].
Median Neuropathy (at the Wrist)
Spinal Accessory Neuropathy istory of Presentation and Exam

H
Findings
This pure motor nerve comes from the C1 to C4 A 72-year-old man presented with right hand and
cervical roots and does not enter the brachial finger numbness of 1 year duration. Symptoms
plexus. It passes through the foramen magnum are intermittent and made worse by hand use,
and then though the jugular foramen supplying such as holding a book to read, using mechanical
the sternocleidomastoid muscle, and finally tools or driving. At times, he has been awakened
through the posterior cervical triangle to inner- during sleep with pain and numbness of the entire
vate the trapezius muscle. right hand, finding relief by changing arm posi-
tion and flicking his wrist. On exam he had intermittent neck pain. On exam, there was mod-
reduced sensation to light touch and pinprick in erate intrinsic left hand muscle atrophy involving
the distal index finger and thumb. There is no ulnar-innervated muscles, and significant reduced
upper limb muscle weakness or atrophy, except sensations to light touch and pin prick in the fifth
for some noted in the right abductor pollicis bre- digit only.
vis (which was also moderately weak). Electrodiagnostic study (see Table 5.2) was
Electrodiagnostic studies were done (see done to work up the differential diagnoses of a
Table 5.1) primarily to evaluate for a neuropathy left ulnar neuropathy at the elbow or at the wrist,
of the right median nerve, either at/distal or prox- a lower brachial plexopathy or a radiculopathy
imal to the wrist, or a C6–C7 radiculopathy. involving C8–T1 nerve roots.
There was an absent right median sensory Findings included an absent left ulnar sensory
response. Markedly prolonged right median motor response recording the fifth digit, with a reduced
distal latency with reduced amplitude were dem- amplitude of the left ulnar sensory response
onstrated. The median minimal F-wave latency recording the dorsal hand. Ulnar motor responses
was prolonged. Ulnar motor and sensory studies recording at the abductor digiti minimi (ADM)
were normal, as was the radial sensory study. showed normal distal latencies with reduced
The needle EMG findings included moder- amplitude and markedly reduced conduction
ately reduced recruitment of motor unit action velocity between the above-elbow and below-
potentials (MUAPs) in the right abductor pollicis elbow stimulation sites (i.e. the elbow segment).
brevis muscle, with increased MUAP amplitude, A complete left ulnar motor conduction block
duration and the presence of polyphasic MUAP’s. (amplitude drop >50%) was demonstrated
No active/ongoing denervation features were between these two stimulation sites as well.
detected. The absence of abnormal findings in Additionally, ulnar motor responses recording at
more proximal median-innervated muscles such the first dorsal interosseous (FDI) muscle showed
as the flexor pollicis longus and flexor carpi radi- normal distal latency and reduced amplitude,
alis excludes a proximal median neuropathy like with reduced conduction velocity at the elbow
that at the forearm, and the normal studies of segment. However, no definite conduction blocks
other C8–T1 muscles do not favor a cervical were demonstrated in this or other segments stud-
motor radiculopathy at these levels. ied. The ulnar F-wave latency was significantly
In this case, the electrodiagnostic findings are prolonged. Median sensory and motor nerve con-
consistent with a severe and chronic right median duction studies were within normal limits.
neuropathy at or distal to the wrist, correspond- The findings on needle EMG included fibrilla-
ing to the clinical diagnosis of carpal tunnel syn- tion potentials in the FDI and ADM muscles, few
drome. This is supported by the prolongation of fasciculation potentials seen in the FDI, with
the right median motor distal latency with stimu- reduced recruitment of the motor unit action
lation at the wrist, as well as reduced right potentials (MUAPs) firing at a rapid rate in both
median motor amplitude, and prolongation of the these muscles, as well as the flexor digitorum
right median F-wave latency in the context of an profundus (to digits 4&5) and the flexor carpi
absent right median sensory response. ulnaris muscles. The MUAPs in these four mus-
cles were also increased in amplitude and dura-
tion, with some polyphasia. Other C8–T1
Ulnar Neuropathy (at the Elbow) innervated muscles as well as lower cervical
paraspinal muscles were normal on needle EMG.
istory of Presentation and Exam
H In this case, the electrodiagnostic findings are
Findings consistent with a subacute on chronic left ulnar
A 54-year-old man had symptoms of left-hand mononeuropathy localizable at the elbow. This
numbness mainly in the fifth digit for 6 months. lesion which is severe in degree electrically
He had also noticed loss of muscle bulk of the exhibits focal demyelinating (at the elbow seg-
left-hand muscles, with pain in the elbow and ment) as well as marked axon loss characteristics.
5
Table 5.1 Case electrodiagnostic data
Sensory nerve conduction studies
B-P Amp (μV) LatNPk (ms) CV (m/s) Dist (mm) Temp (°C)
Nerve Stimulus Recording L R L R L R L R Norm B-P Amp (μV) Norm LatNPk (ms) Norm CV (m/s) L R
Median Wrist Index Absent Absent Absent 130 >10 <3.6 >50 33.1
finger
Ulnar Wrist Digit 5 23.9 3.1 55 110 >10 <3.2 >50 33.3
Mononeuropathies
Radial Forearm Thumb 21.2 2.4 57 100 >14 <2.7 >50 33.1
Motor nerve conduction studies
B-P Amp (mV) LatOn (ms) CV (m/s) Dist (mm) Norm B-P Temp (°C)
Nerve Recording Stimulus L R L R L R L R Amp (mV) Norm LatON (ms) Norm CV (m/s) L R
Median Abductor pollicis brevis Wrist 1.8 12.5 50 >6 <4 >50 33.2
Elbow 1.5 18.2 40.0 230
Ulnar Abductor digiti minimi Wrist 12.2 1.8 50 >7 <3.1 >50 33.2
Below elbow 12.1 5.1 58.5 190
Above elbow 11.7 6.8 58.0 290
F-wave studies
F-waves
Lat (ms)
Nerve Stimulus Recording L R
Median Wrist Abductor pollicis brevis 35.3 (NL 22–31)
Ulnar Wrist Abductor digiti minimi 28.9 (NL 21–32)
Needle EMG summary
Side Muscle Ins Act Fib PW Fasc Other Number Recruit Dur Dur Amp Amp Poly Poly Descript
R Deltoid Norm 0 0 0 Norm Full Norm Norm Norm NC
R Flexor carpi radialis Norm 0 0 0 Norm Full Norm Norm Norm NC
R Abductor pollicis brevis Norm 0 0 0 −2 Rapid Many 1+ Many 1+ Many 1+ NC
R Flexor pollicis longus Norm 0 0 0 Norm Full Norm Norm Norm NC
R First dorsal interosseous Norm 0 0 0 Norm Full Norm Norm Norm NC
R Biceps brachii Norm 0 0 0 Norm Full Norm Norm Norm NC
R Triceps Norm 0 0 0 Norm Full Norm Norm Norm NC
99
100

B-P Amp (μV) LatNPk (ms) CV (m/s) Dist (mm) Norm B-P Amp Temp (°C)
Nerve Stimulus Recording L R L R L R L R (μV) Norm LatNPk (ms) Norm CV (m/s) L R
Median Wrist Index finger 18.7 3.4 60.1 130 >15 <3.6 >50 33.2
Ulnar Wrist Digit V Absent Absent Absent 110 >10 <3.1 >50 33.0
Ulnar Wrist Dorsum of hand 5.1 2.9 51.5 100 >10 <3.1 >50 33.1
B-P Amp (mV) LatOn (ms) CV (m/s) Dist (mm) Temp (°C)
Nerve Recording Stimulus L R L R L R L R Norm B-P Amp (mV) Norm LatON (ms) Norm CV (m/s) L R
Median Abductor pollicis Wrist 9.9 3.9 50 >6 <4 >50 33.2
brevis (APB) Elbow 8.2 10.9 51.2 320
Ulnar Abductor digiti Wrist 3.7 3.0 50 >7 <3.1 >50 33.1
minimi (ADM) Below elbow 3.0 7.5 53.3 240
Above elbow 1.4 17.1 24.1 340
Ulnar First dorsal Wrist 5.4 4.4 50 >7 <4.5 >50 33.1
interosseous Below elbow 4.4 8.5 58.5 240
Above elbow 3.7 18 25.0 340
F-wave studies
F-waves
Lat(ms)
Median/APB Wrist Abductor pollicis brevis 29.9 (NL 22–31)
Ulnar/ADM Wrist Abductor digiti minimi 45.5 (NL 21–32)
Needle EMG summary
Left First dorsal interosseous 1+ 1+ 1+ 1+ 2− Rapid Many 1+ Some 1+ Few 1+ NC
Left Abductor digiti minimi 1+ 1+ 1+ 0 2− Rapid Many 1+ Some 1+ Some 2+ NC
Left Flexor digitorum profundus to digits 5 0 0 0 0 2− Rapid Some 1+ Some 1+ Few 1+ NC
and 4
Left Flexor carpi ulnaris 0 0 0 0 2− Rapid Many 1+ Some 1+ Some 1+ NC
Left Pronator teres 0 0 0 0 Full Norm Norm Norm NC
Left Biceps brachii 0 0 0 0 Full Norm Norm Norm NC
Left Triceps 0 0 0 0 Full Norm Norm Norm NC
R. Lugo and A. Soriano
Left Low cervical paraspinals 0 0 0 0 Full Norm Norm Norm NC

This is supported by the findings of active/ongo- ral groove segment (in which there is also conduc-
ing and chronic denervation features in the ulnar- tion slowing/velocity reduction). Right radial motor
innervated hand and forearm muscles, and studies were done for comparison, and the responses
normal findings in other C8–T1 innervated mus- were normal. The left median and ulnar motor stud-
cles, with abnormal ulnar sensory (recording ies were also normal.
fifth digit & dorsal hand) and abnormal ulnar The left radial sensory response was markedly
motor responses recording the ADM and FDI reduced in amplitude with normal peak latency,
muscles. Localization of the lesion at the elbow is and the right radial sensory response showed nor-
based on the demonstration of conduction veloc- mal amplitude and peak latency. The left median
ity reduction/slowing and conduction block in and ulnar sensory studies were normal.
this segment. The needle EMG demonstrated fibrillation and
positive sharp wave potentials in the left brachiora-
dialis, extensor digitorum (communis) and extensor
Radial Neuropathy indicis (proprius) muscles. On needle EMG, these
three muscles showed markedly reduced recruit-
H ment with at least a few to some motor unit action
Findings potentials (MUAPs) displaying large amplitudes,
A 44-year-old woman presented with a 4 month increased duration and polyphasia.
history of a left wrist and finger drop, initially In this case, the electrodiagnostic findings are
noticed after awakening from a prolonged and consistent with a subacute to early chronic left
deep sleep, in the context of heavy alcohol use radial neuropathy, localizable at the spiral
before falling asleep on a couch “in an awkward groove. The lesion is at least moderate in degree
position”. She also noticed numbness in the dor- electrically and exhibits focal demyelinating (at
sum of her left hand, mostly around the first web the spiral groove segment) as well as marked
space. There was no significant pain, nor history axon loss characteristics. This is supported by
of known trauma. On exam she had a left wrist the presence of a conduction block (with slowing/
and finger drop with significant weakness graded velocity reduction) at the spiral groove, with pre-
as 2/5 on the Medical Research Council (MRC) served distal radial motor amplitudes, but signifi-
scale. There was modest weakness in hand grip, cant (>50%) amplitude drop when stimulating at
but finger flexion was normal, as well as strength the proximal spiral groove site, together with the
in the rest of the left upper limb. On sensory exam presence of fibrillation and positive sharp wave
there was reduced sensation to light touch and potentials, and reduced recruitment with some
pinprick in the dorsum of the left hand between large-sized MUAPs (with polyphasia) in all
the thumb and index fingers only. Deep tendon radial-innervated muscles examined below the
reflexes were normal throughout. spiral groove [i.e. brachioradialis, extensor indi-
Electrodiagnostic studies (see Table 5.3) were cis (proprius) and extensor digitorum
done to work up a differential diagnosis of a left (communis)].
radial neuropathy at the spiral grove versus at
other level, a left brachial plexopathy (especially
one involving the posterior cord), or a radiculop- Suprascapular Neuropathy
athy involving the C7 nerve root.
Left radial motor nerve conduction studies istory of Presentation and Exam
H
recording both the extensor indicis (proprius) and Findings
the extensor digitorum (communis) muscles showed A 52-year-old patient presented to the clinic with
a normal motor amplitude and latency when stimu- a 2-month history of right arm weakness with
lating at the elbow and distal spiral groove site, but external rotation and somewhat with elevating
the amplitude significantly reduced by >50% when the arm above the shoulder. She had posterior
stimulating at the proximal spiral groove site, which shoulder pain, but this was not a prominent symp-
indicates presence of a conduction block at the spi- tom. She denies trauma to the neck and shoulder
102
Sensory nerve conduction

B-P Amp (μV) LatNPk (ms) Dist (mm) Norm B-P Norm Temp (°C)
Nerve Stimulus Recording L R L R L R Amp LatNPk L R
Median Wrist Index 49.20 2.70 130 >20 μV <3.4 ms 33.2
Ulnar Wrist 5th dig 42.70 2.60 110 >12 μV <3.1 ms 33.2
Radial Forearm Thumb 10.50 47.50 2.20 2.10 100 100 >18 μV <2.7 ms 32.5 32.8
Motor nerve conduction
B-P Amp (mV) LatOn (ms) CV (m/s) Dist (mm) Norm B-P Norm Temp (°C)
Nerve Recording Stimulus L R L R L R L R Amp LatOn Norm CV L R
Median APB Wrist 10.50 2.60 n/a 50 >6 mV <3.9 ms >50 m/s 32.3
Elbow 10.00 6.50 65.4 255 32.2
Ulnar/ ADM Wrist 11.10 2.00 n/a 50 >7 mV <3.1 ms >50 m/s 32.9
ADM Below Elbow 10.80 5.30 59.1 195 32.9
Above Elbow 10.30 7.40 54.0 295 32.0
Radial/ EDC Elbow 6.70 7.10 1.30 1.00 n/a n/a >6 mV <3.1 ms >50 m/s 32.0 32.1
EDC Dist. Spir. Groove 6.10 7.00 2.60 2.60 81.5 68.8 110 110 32.7 32.0
Prox. Spir. Groove 1.30 7.70 31.3 200 32.7
Radial/ EIP Elbow 7.80 7.70 2.20 2.20 n/a n/a >6 mV <3.1 ms >50 m/s 32.6 32.7
EIP Dist. Spir. Groove 6.10 6.70 4.20 3.70 60.0 80.0 120 120 32.5 32.6
Prox. Spir. Groove 1.42 6.40 40.5 170 32.5
F-wave side-to-side comparison table
Nerve Stimulus Recording F-Waves
Lat (ms)
L R
Ulnar/ADM Wrist ADM 25.80
5
Needle EMG summary
Side Muscle Ins Act. Fib PW Fasc Other Number Recruit Dur Dur Amp Amp Poly Poly Descript Descript Descript
L 1st Dorsal Norm 0 0 0 2− Mod Norm Norm Norm NC
Interosseous
Flex.Pollicis Norm 0 0 0 2− Mod Norm Norm Norm NC
Longus
EIP Norm 2+ 1+ 0 3− Rapid Some 1+ Some 1+ Few 1+ NC
Mononeuropathies
Pronator Teres Norm 0 0 0 1− Mod-V Norm Norm Norm NC

EDC Norm 2+ 0+ 0 3− Rapid Many 1+ Some 1+ Many 1+ NC
Brachioradial Norm 1+ 2+ 0 3− Rapid Some 1+ Few 1+ Few 1+ NC
Anconeus Norm 0 0 0 2− Mod Norm Norm Norm NC
Biceps Brachii Norm 0 0 0 1− Mod Norm Norm Norm NC
Triceps— Norm 0 0 0 1− Mod-V Norm Norm Norm NC
Lateral head
Deltoid–Middle Norm 0 0 0 1− Mod Norm Norm Norm NC
head
APB Abductor pollicis brevis, ADM Abductor digiti minimi, EDC Extensor digitorum communis, EIP Extensor indicis proprius
103
area, and there is no definite numbness or pares- supply the glenoacromial and acromioclavicular
thesia. The patient states that she carries a heavy joints.
handbag frequently over her right shoulder.
Examination revealed weakness of external
shoulder rotation on the right, with normal Axillary Neuropathy
strength in the rest of the muscle groups. There
was no appreciable muscle atrophy, and there istory of Presentation and Exam
H
were intact deep tendon reflexes and a normal Findings
sensory exam. A 72-year-old man was evaluated 3 months after
Electrodiagnostic studies of the upper extrem- right shoulder dislocation in the context of a fall
ity (see Table 5.4) were performed to work up the which was treated conservatively in the emer-
differential diagnoses which included a cervical gency department. Now, he presents with persis-
radiculopathy, a brachial plexopathy or a proxi- tent numbness in the lateral aspect of his right
mal upper limb mononeuropathy. shoulder, with loss of muscle bulk around the
All motor responses, including axillary, mus- point of the shoulder and weakness with signifi-
culocutaneous, ulnar and median had normal cant difficulty raising the arm above the shoulder
amplitudes, distal latencies and conduction level. This had not improved following months of
velocities. Sensory nerve conduction studies, physical therapy.
including the median nerve recording at digits 1, On exam, there was atrophy of the right del-
2 and 3, the ulnar, radial, as well as the lateral and toid, moderate weakness with right shoulder
medial antebrachial cutaneous responses were abduction and (to a lesser degree) with arm/
also with normal peak latencies and amplitudes. shoulder flexion and extension as well. However,
These normal responses essentially ruled out a there was no scapular winging, and the rest of the
right brachial plexopathy. right arm muscles demonstrated normal strength;
Needle EMG showed the presence of fibrillation deep tendon reflexes were normal. There were no
potentials with reduced recruitment and rapid firing sensory deficits, except for reduced sensation to
pattern (of normal-sized motor unit potentials) in light touch and pinprick in small area of the right
the infraspinatus muscle only. The supraspinatus lateral shoulder.
and other muscles in the C5–C6 myotomes were Electrodiagnostic studies of the upper extremi-
normal, effectively excluding the presence of a C5– ties (see Table 5.5) were performed to work up the
C6 cervical motor radiculopathy. differential diagnoses including a right cervical
In this case, the electrodiagnostic findings are radiculopathy at C5–C6, a brachial plexopathy
consistent with a subacute axon loss lesion of the (especially one involving the upper trunk), or a
distal right suprascapular nerve, favoring local- proximal upper limb mononeuropathy.
ization at the spinoglenoid notch, moderate in The right axillary motor response recording
degree electrically, with evidence of active/ongo- the deltoid muscle was absent. All other motor
ing motor axon loss. This is supported by the responses (including the right median, right
needle EMG findings of active/ongoing denerva- ulnar, left axillary and bilateral musculocutane-
tion features and reduced recruitment of normal- ous) were with normal amplitudes, distal laten-
sized motor unit action potentials (too early for cies, and conduction velocities. Sensory nerve
remodeling and enlargement) in the infraspina- conduction studies, including the right median
tus muscle, sparing the supraspinatus muscle. nerve recording at digits 1, 2 and 3, the right
The finding of denervation features in the ulnar, radial, as well as the lateral and medial
infraspinatus muscle only supports the diagno- antebrachial cutaneous responses were also with
sis of suprascapular neuropathy localizable at normal peak latencies and amplitudes. These
the spinoglenoid notch, with little pain mainly other normal motor responses and sensory
due to sparing of the deep sensory branches that responses essentially ruled out a right brachial
plexopathy.
5
B-P Amp (μV) LatNPk (ms) CV (m/s) Dist (mm) Norm B-P Temp (°C)
Nerve Stimulus Recording L R L R L R L R Amp (μV) Norm LatNPk (ms) Norm CV (m/s) L R
Median Wrist Index (D2) 58.9 2.8 63 130 >10 <3.6 >50 33.4
Middle (D3) 52.5 2.8
Thumb (D1) 40.8 2.4
Mononeuropathies
Radial Forearm Thumb (D1) 50.4 2.2 57 100 >14 <2.7 >50 33.0
Medial antebrachial Medial elbow Medial 19.3 2.4 63 120 >8 <2.9 >55 33.0
cutaneous forearm
Lateral antebrachial Antecubital Lateral 19.4 2.2 60 120 >10 <2.9 33.0
cutaneous fossa forearm
Nerve Recording Stimulus L R L R L R L R Amp (mV) Norm LatON (ms) Norm CV (m/s) L R
Median Abductor Wrist 10.4 2.9 50 >6 <4 >50 33.2
pollicis brevis Elbow 10.2 7.4 57.8 260
Ulnar Abductor Wrist 8.9 2.1 50 >7 <3.1 >50 33.1
digiti minimi Below elbow 8.8 4.8 69.1 190
Above elbow 8.7 6.8 61.7 290
Musculocutaneous Biceps Axilla 5.3 3.4 >4 <3.5 33.5
Axillary Deltoid Erb’s point 10.7 3.1 >4 <4.9 33.5
F-wave studies
F-waves
Lat (ms)
(continued)
105
Table 5.4 (continued)
106
Needle EMG summary

Side Muscle Insertional activity Positive Sharp Fibrillation Fasciculation Recruitment Firing Amp Duration Polyphasia Descript
Wave pattern
R Infraspinatus 1+ None 2+ 0 2- Rapid Normal Normal Normal NC
R Supraspinatus Normal 0 0 0 Normal Normal Normal Normal Normal NC
R Rhomboid major Normal 0 0 0 Normal Normal Normal Normal Normal NC
R Deltoid Normal 0 0 0 Normal Normal Normal Normal Normal NC
R Pronator teres Normal 0 0 0 Normal Normal Normal Normal Normal NC
R Biceps brachii Normal 0 0 0 Normal Normal Normal Normal Normal NC
R Triceps Normal 0 0 0 Normal Normal Normal Normal Normal NC
R 1st dorsal Normal 0 0 0 Normal Normal Normal Normal Normal NC
interosseous
R Cervical Normal 0 0 0 Normal Normal Normal Normal Normal NC
paraspinals (mid)
5
B-P Amp LatNPk Norm
(μV) (ms) CV (m/s) Dist (mm) Norm B-P LatNPk Norm CV Temp (°C)
Nerve Stimulus Recording L R L R L R L R Amp (μV) (ms) (m/s) L R
Median Wrist Index (D2) 28.1 3.5 54 130 >10 <3.6 >50 33.2
Middle (D3) 17.7 3.5 130 33.2
Mononeuropathies
Thumb (D1) 17.7 3.4 130 33.2

Medial antebrachial cutaneous Medial elbow Medial 10.8 2.6 56 120 >8 <2.9 >55 33.2
forearm
Lateral antebrachial cutaneous Antecubital fossa Lateral 12.3 2.8 55 120 >10 <2.9 33.2
forearm
B-P Amp (mV) LatOn (ms) CV (m/s) Dist (mm) Norm B-P Norm Norm CV Temp (°C)
Nerve Recording Stimulus L R L R L R L R Amp (mV) LatON (ms) (m/s) L R
Median Abductor pollicis brevis Wrist 8.7 3.9 50 >6 <4 >50 33.5
Elbow 8.6 9.5 58.9 330
Ulnar Abductor digiti minimi Wrist 8.0 2.8 50 >7 <3.1 >50 33.5
Below 7.2 6.9 55.4 230
elbow
Above 7.1 8.9 54.1 330
Elbow
Musculocutaneous Biceps Axilla 8.5 7.9 2.9 3.0 >4 <3.1 33.5 33.2
Axillary Deltoid Erb’s 4.3 0 4.3 0 >4 <4.9 33.2 33.2
point
F-wave studies
F-waves
Lat (ms)
(continued)
107
108
Needle EMG Summary

R Infraspinatus 0 0 0 0 Norm Full Norm Norm Norm NC
R Supraspinatus 0 0 0 0 Norm Full Norm Norm Norm NC
R Deltoid, middle head 1+ 1+ 1+ 0 3− Rapid Many 1+ Many 1+ Many 1+ NC
R Extensor indicis 0 0 0 0 Norm Full Norm Norm Norm NC
proprius
R Pronator teres 0 0 0 0 Norm Full Norm Norm Norm NC
R First dorsal 0 0 0 0 Norm Full Norm Norm Norm NC
interosseous
R Biceps Brachii 0 0 0 0 Norm Full Norm Norm Norm NC
R Teres minor 1+ 3+ 1+ 0 3− Rapid Most 1+ Many 1+ Some 2+ NC
R Triceps 0 0 0 0 Norm Full Norm Norm Norm NC
R Mid cervical 0 0 0 0 Norm Full Norm Norm Norm NC
paraspinals
The needle EMG showed fibrillation poten- pronation with the elbow flexed. The patient was
tials and positive sharp waves in the middle head unable to make the “OK” sign with left hand, had
of the deltoid muscle and the teres minor muscle, some tenderness to touch in distal left forearm,
with motor unit action potentials demonstrating but no definite sensory deficits.
severely reduced recruitment, rapid firing pat- Electrodiagnostic studies (see Table 5.6) were
tern, increase in amplitude, duration and poly- done to work up the differential diagnoses which
phasia. All other muscles in the C5–C6 included an anterior interosseous neuropathy ver-
myotomes, as well as paraspinal muscles were sus a more proximal median neuropathy, a lower
normal, essentially ruling out a cervical motor brachial plexopathy or a radiculopathy involving
radiculopathy. predominantly C8–T1 nerve roots.
In this case, the electrodiagnostic findings are Nerve conduction studies demonstrated nor-
consistent with a subacute on early chronic axon mal left median and ulnar motor responses, as
loss lesion of the right axillary nerve, severe in well as normal left median, ulnar and radial sen-
degree electrically. This is supported by an absent sory responses. Corresponding left median and
right axillary motor response, and active/ongo- ulnar F-wave latencies were also within normal
ing denervation features with severe motor unit limits.
dropout demonstrated by needle EMG in the Needle EMG disclosed abundant fibrillation
right deltoid and teres minor muscles. potentials and positive sharp waves in the left
As in this case of a shoulder dislocation fol- flexor pollicis longus (FPL) and pronator quadra-
lowing a fall, isolated axillary neuropathies are tus (PQ) muscles. No motor unit action potential
usually secondary to trauma. The typical findings (MUAP) recruitment was found in these two
of weakness of shoulder abduction > arm/shoul- muscles (suggesting loss of nerve continuity to
der flexion and extension, as well as sensory loss these muscles). Needle EMG of the left flexor
over proximal lateral shoulder, and the EMG digitorum profundus to digits 2&3 (FDP-2&3)
findings of motor axon loss changes in the axil- disclosed fibrillation potentials as well, but also
lary nerve distribution (including in the deltoid markedly reduced recruitment with a few resid-
and teres minor muscles) are all strong support- ual MUAPs of normal size and morphology, fir-
ive evidence of an axillary neuropathy. ing moderately fast to rapidly. All other muscles
examined were normal, including the remaining
median-innervated muscles (the left flexor carpi
Anterior Interosseous Neuropathy radialis, pronator teres, and abductor pollicis bre-
vis), the left first dorsal interosseous, biceps bra-
H chii, deltoid and triceps.
Findings In this case, the electrodiagnostic findings are
A 44-year-old man presented with left forearm consistent with a subacute left anterior interosse-
pain over the past 5 weeks, noticed soon after sur- ous neuropathy, very severe in degree electri-
gery for left thumb carpometacarpal joint arthrod- cally, with evidence of active/ongoing motor
esis with bone graft. He had been in a left arm axon loss. This is supported by findings on needle
cast for about 4 weeks postoperatively (recently EMG, particularly abundant fibrillation and/or
removed), but was still having pain in the fore- positive sharp potentials without chronic dener-
arm, as well as weakness with thumb and index vation MUAP changes (actually nerve disconti-
finger flexion, often dropping keys and other nuity suggested by lack of MUAPs in the left FPL
items requiring a secure pincer grasp. and PQ), exclusively in the left anterior interos-
Examination of the left upper limb revealed seous nerve (AION) distribution (FPL, PQ, FDP-
severe weakness of flexion of the distal phalanx 2&3). The normal left median sensory and motor
of the thumb and the index, and mild weakness of nerve conduction studies are also compatible
110

Nerve Stimulus Recording L R L R L R L R Norm B-P Amp (μV) Norm LatNPk (ms) Norm CV (m/s) L R
Median Wrist Index finger 44.5 3.0 60.1 130 >20 <3.4 >50 33.2
Ulnar Wrist Digit V 24.6 2.6 58 110 >12 <3.1 >50 33.0
B-P Amp
(mV) LatOn (ms) CV (m/s) Dist (mm) Temp (°C)
Nerve Recording Stimulus L R L R L R L R Norm B-P Amp (mV) Norm LatON (ms) Norm CV (m/s) L R
Median Abductor Wrist 6.8 2.9 50 >6 <4 >50 33.2
pollicis brevis Elbow 6.5 7.3 54.5 240
Ulnar First dorsal Wrist 10.1 2.2 50 >7 <4.5 >50 33.1
interosseous Below elbow 8.1 6.2 52.0 210
Above elbow 7.9 8.3 51.2 310
F-wave studies
F-waves
Lat (ms)
Needle EMG Summary
Side Muscle Ins act Fib PW Fasc Other Number Recruit Dur Dur AMP Amp Poly Poly Descript
Left First dorsal interosseous 0 0 0 0 Full Norm Norm Norm NC
Left Flexor pollicis longus 1+ 2+ 1+ 0 None NC
Left Abductor pollicis brevis 0 0 0 0 Full Norm Norm Norm NC
Left Pronator quadratus 1+ 2+ 2+ 0 None NC
Left Pronator teres 0 0 0 0 Full Norm Norm Norm NC
Left Flexor carpi radialis 0 0 0 0 Full Norm Norm Norm NC
Left Flexor digitorum profundus 1+ 1+ 0 0 3− Mod-R Norm Norm Norm NC
(to D2&3)
Left Biceps 0 0 0 0 Full Norm Norm Norm NC
Left Triceps 0 0 0 0 Full Norm Norm Norm NC
Left Deltoid 0 0 0 0 Full Norm Norm Norm NC

with this diagnosis, ruling out a median neuropa- units detected) in the left AH, EDB and TP, with
thy at or distal to the wrist (as seen with carpal the other aforementioned muscles (plus the left
tunnel syndrome), or one more proximal to the semitendinosus) displaying electrically
branchpoint of the AION. moderate-to-severe chronic denervation changes,
including reduced recruitment with rapidly-firing
large MUAPs.
Sciatic Neuropathy Other left non-sciatic L5 and S1-innervated
muscles tested, including gluteus medius as well
H as the left low lumbar paraspinal muscles showed
Findings no definite evidence of motor axon loss. This
A 64-year-old woman presented with persistent helped exclude a left L5–S1 lumbar radiculopa-
left leg weakness and pain that was worst at the thy or a left lumbosacral plexopathy (noting nee-
end of the day. Pain would radiate from posterior dle EMG of the left rectus femoris was also
left hip to the dorsum of the foot, with stabbing normal).
and electrical-like sensations. Symptoms began In this case, the electrodiagnostic findings are
immediately after a left hip replacement surgery consistent with a subacute to chronic left sciatic
6 months ago. Her exam revealed moderate atro- mononeuropathy, axon loss in character and
phy of left leg muscles, particularly those below severe in degree electrically. This impression is
the knee, with marked weakness of knee flexors, supported by the absent motor responses of the
and also severe weakness with ankle dorsiflexion, left tibial and peroneal [fibular] nerves (deriva-
plantarflexion, inversion and eversion. The left tives of the sciatic nerve), an absent left tibial
ankle deep tendon reflex was absent, and sensory H-reflex, as well as absent left sural and superfi-
exam showed absent perception to light touch cial peroneal [fibular] sensory responses, with
and pin prick in the dorsal and lateral left foot and needle EMG findings of active/ongoing and
calf. To walk she required the help of a walker chronic denervation changes in left peroneal [fib-
and had a pronounced foot drop. ular] and tibial nerve supplied muscles below the
Electrodiagnostic studies (see Table 5.7) were knee as well as direct sciatic-innervated ham-
done to work up the differential diagnoses includ- string muscles, with sparing of the more proximal
ing a left sciatic mononeuropathy, peroneal (fibu- (non-sciatic supplied) L5 and S1 innervated mus-
lar) mononeuropathy, lumbosacral plexopathy or cles, including the gluteal and lumbosacral para-
a (mostly L5) radiculopathy. spinal muscles.
Findings were notable for an absent left tibial
(recording abductor hallucis) and peroneal [fibu-
lar] (recording both extensor digitorum brevis Peroneal (Fibular) Neuropathy
and tibialis anterior) motor nerve responses, as
well as absent left sural and superficial peroneal istory of Presentation and Exam
H
[fibular] sensory responses. The tibial H-reflex Findings
was absent on the left as well. Contralateral A 56-year-old woman presented with 5 weeks of
responses obtained were normal. burning pain and numbness in the right anterior
The needle EMG showed abundant fibrillation leg below the knee and on the dorsum of the foot.
and/or positive sharp wave potentials in the left Upon further questioning she mentioned fre-
abductor hallucis (AH), extensor digitorum quently tripping over her “floppy” right foot, but
brevis (EDB), medial gastrocnemius, tibialis
no falls. Just prior to symptom onset, she had
anterior, tibialis posterior (TP), and the short resumed frequent leg-crossing after losing 100
head of the biceps femoris muscle. On needle pounds over 9 months in the wake of bariatric
EMG, recruitment was absent (no residual motor surgery.
112

B-P Amp (μV) LatNPk (ms) Dist (mm) Temp (°C)
Nerve Stimulus Recording L R L R L R Norm B-P Amp Norm LatNPk L R
Sural Lower Leg Lat. Malleolus Absent 5.00 Absent 3.60 140 140 >3 μV <4.6 ms 33.0 30.6
Superficial peroneal (fibular) Lower leg Ankle Absent 3.90 Absent 3.40 100 100 >3 μV <4.6 ms 33.1 30.1
B-P Amp (mV) LatOn (ms) CV (m/s) Dist (mm) Norm B-P Norm Temp (°C)
Nerve Recording Stimulus L R L R L R L R Amp LatOn Norm CV L R
Peroneal Extensor Ankle Absent 5.80 Absent 4.00 n/a n/a >2.5 mV <6 ms >40 m/s 33.2 30.7
(fibular) digitorum Pop. foss—knee Absent 4.70 Absent 13.00 n/a 47.5 430 33.1 30.7
brevis
Tibial Abductor Ankle Absent 7.60 Absent 4.30 n/a n/a >4 mV <6 ms >40 m/s 33.3 30.5
hallucis Pop. fossa-knee Absent 4.50 Absent 13.30 n/a 44.4 400 33.3 30.5
Peroneal Tibialis Below fibular Absent 5.10 Absent 2.70 n/a n/a >3 mV <4.5 ms >40 m/s 33.3 30.5
(fibular) anterior head
Femoral Rectus Inguinal Lig 5.50 6.20 4.90 5.30 n/a n/a >3 mV <6.5 ms 33.2 30.1
femoris
H-reflex summary table
M-Wave H-Wave
Nerve Stimulus Recording Side Lat (ms) Amp (mV) Lat (ms) Amp (mV)
Tibial Popliteal Fossa Soleus L Absent Absent
Tibial Popliteal Fossa Soleus R 5.7 ms 8.4 mV 32.1 ms 1.3 mV
Needle EMG summary
Muscle Ins Fib PW Fasc Other Number Recruit Dur Dur Amp Amp Poly Poly
Side Act. Descript
L Abductor Hallucis Norm 1+ 1+ 0 None NC
Extn.Digitorum Brevis Norm 1+ 3+ 0 None NC
Tibialis Posterior Norm 0 2+ 0 None NC
Gluteus Medius Norm 0 0 0 Norm Full Norm Norm Norm NC
Tibialis Anterior Norm 1+ 2+ 0 SMU Mod All 2+ Norm All 2+ NC
Gastrocnemius, medial head Norm 0 2+ 0 CRD 3− Mod Most 1+ Norm Norm NC
Rectus Femoris Norm 0 0 0 Norm Full Norm Norm Norm NC
Biceps Femoris, short head Norm 0 1+ 0 2− Mod-R Many 1+ Few 1+ Norm NC
Semitendinosus Norm 0 0 0 2− Mod-R Some 1+ Some 1+ Norm NC
Lumbar paraspinals (Low) Norm 0 0 0 1− Mod Norm Norm Norm NC

CRD complex repetitive discharges
On exam there was moderate weakness of right ing) at the fibular head recording both EDB and TA,
ankle dorsiflexion and right foot eversion. There severe in degree electrically. These findings are fur-
was reduced sensation to light touch and pinprick ther supported by absence of the right superficial
in the dorsum of the right foot, including the first peroneal (fibular) sensory response, in the context of
web space. The remainder of the neurological normal sural (sensory) and tibial motor responses. In
exam was normal, specifically: intact power with addition, supportive needle EMG findings include
ankle inversion, knee and hip flexion and exten- active/ongoing denervation changes in peroneal
sion. The deep tendon reflexes were normal. (fibular)-innervated muscles supplied by both deep
Electrodiagnostic studies (see Table 5.8) were and superficial branches, with sparing of the tibial-
done to work-up the differential diagnoses innervated, and more proximal non-peroneal (non-
including a right peroneal (fibular) mononeurop- fibular)-innervated L5/S1-innervated muscles. It is
athy, a right sciatic mononeuropathy or a right too soon in the timeline to be considered a chronic
lumbar (particularly L5) radiculopathy. lesion since the MUAPs are still normal in size and
Motor nerve conduction studies of the right configuration (not yet remodeled and larger) despite
peroneal (fibular) nerve demonstrated normal the reduced recruitment pattern seen.
CMAP amplitude when recording both the exten-
sor digitorum brevis (EDB) and the tibialis ante-
rior (TA) muscles, stimulating at the ankle (for ibial Neuropathy (at the Tarsal
T
the EDB) and below fibular head sites (for both Tunnel)
EDB and TA). For the EDB and TA, the CMAP
amplitude was markedly reduced (drop >50%, A 30 year old dancer presented with 3 months of
implicating conduction block) when stimulating progressive pain and burning paresthesia on the
above the fibular head/at the popliteal fossa. The sole of her left foot, noticing the onset of these at
right superficial peroneal (fibular) sensory the end of a very busy performance season. She
response was absent. The right tibial motor does not recall any definite antecedent accident
responses showed normal amplitude, distal or injury to the left foot, and there is no visible
latency and conduction velocity. The right sural swelling or skin color changes in the affected
response was also normal. area. The symptoms appear to be aggravated with
Needle EMG demonstrated abundant fibrilla- prolonged time spent on her feet, especially when
tion (and often positive sharp wave) potentials in dancing. Although when pain becomes severe it
the right TA, EDB and peroneous longus muscles may make her walk “funny”, she denies any sig-
with decreased recruitment of motor unit poten- nificant limb weakness.
tials (which were normal in size and configura- Examination findings are most notable for
tion), firing at a moderately fast to rapid rate. numbness intermixed with hyperalgesic/dyses-
Findings were normal in the right tibial- thetic distortion of pinprick perception through-
innervated muscles including the medial gastroc- out the plantar aspect of her left foot. Pain
nemius, tibialis posterior and abductor hallucis, symptoms of similar quality and distribution
as well as sciatic-innervated (peroneal/fibular appear to be triggered with lightly tapping over
divison) muscles like the short head of the biceps an area slightly posterior and inferior to the right
femoris. More proximal right L5/S1-innervated medial malleolus. She also demonstrates some
muscles, such as the semitendinosus and gluteus weakness with abduction of toes of the left foot.
medius displayed normal findings. Deep tendon reflexes, including the left ankle
In this case, the electrodiagnostic findings are jerk are all intact. Gait is notable for slight antal-
consistent with a subacute right common peroneal gic features on the left.
(fibular) mononeuropathy, localizable at the fibular Electrodiagnostic studies (see Table 5.9) were
head, axon loss in character but also with prominent done to workup the differential diagnoses which
focal demyelination manifested by complete conduc- included a left tibial mononeuropathy at the tar-
tion block (and segmental conduction velocity slow- sal tunnel (i.e. tarsal tunnel syndrome), versus a
114

B-P Amp (μV) LatNPk (ms) CV (m/s) Dist (mm) Norm B-P Norm Temp (°C)
Nerve Stimulus Recording L R L R L R L R Amp LatNPk Norm CV L R
Sural Lower Leg Lat. Malleolus 13.52 4.04 n/a 140 >4 μV <4.6 ms >40 m/s 32.1
Superficial Lower Leg Ankle 5.60 Absent 3.30 Absent n/a n/a 100 100 >4 μV <4.6 ms 31.7 31.5
peroneal (fibular)
Nerve Recording Stimulus L R L R L R L R Amp Norm LatOn Norm CV L R
Peroneal Extensor Ankle 3.91 3.41 4.65 5.75 n/a n/a >2.5 mV <6 ms >40 m/s 32.7 31.1
(fibular) digitorum Pop Foss—Knee 3.38 0.39 14.00 17.45 46.0 37.6 430 440 32.8 31.0
brevis Below Fibular 2.97 13.90 45.4 370 31.0
Head
Tibial Abductor Ankle 5.38 3.95 n/a >4 mV <6 ms >40 m/s 30.4
hallucis Pop Fossa—Knee 4.10 12.55 51.2 440 30.6
Peroneal Tibialis Below Fib Head 3.87 3.10 2.30 2.25 n/a n/a >3 mV <4.5 ms >40 m/s 32.3 30.8
(fibular) anterior Pop Fossa—Knee 3.65 0.80 4.40 5.05 47.6 35.7 100 100 32.0 30.9
Nerve Stimulus Recording F-Waves
Lat (ms)
L R
Tibial Ankle Abductor hallucis 44.20
Nerve Stimulus Recording Side M-Wave H-Wave
Lat (ms) Amp (mV) Lat (ms) Amp (mV)
Tibial Popliteal Fossa Soleus Right 4.0 ms 10.6 mV 30.3 ms 2.7 mV
5
Needle EMG summary
Side Muscle Ins Act. Fib PW Fasc Other Number Recruit Dur Dur Amp Amp Poly Poly. Descript
R Abductor Hallucis Norm 0 0 0 1− Mod-V Norm Norm Norm NC
Extensor Digitorum 1+ 2+ 0 0 3− Mod-R Norm Norm Norm NC
Brevis
Tibialis Posterior Norm 0 0 0 Norm Full Norm Norm Norm NC
Tibialis Anterior 1+ 2+ 1+ 0 3− Mod-R Norm Norm Norm NC
Mononeuropathies
Peroneus Longus 1+ 1+ 1+ 0 3− Mod-R Norm Norm Norm NC

Gastrocnemius, medial Norm 0 0 0 Norm Full Norm Norm Norm NC
head
Biceps Femoris, short Norm 0 0 0 Norm Full Norm Norm Norm NC
head
Semitendinosus Norm 0 0 0 Norm Full Norm Norm Norm NC
115
116

Nerve Stimulus Recording L R L R L R L R Norm B-P Amp Norm LatNPk Norm CV L R
Superficial Lower Leg Ankle 16.36 3.42 n/a 100 >6 μV <4.4 ms 31.1
peroneal (fibular)
Med. Plantar Med. Sole Ankle 11.20 24.02 7.0 3.08 n/a n/a 110 110 >5.3 <6.8 31.5 30.3
Nerve Recording Stimulus L R L R L R L R Norm B-P Amp Norm LatOn Norm CV L R
Peroneal EDB Ankle 5.90 3.90 n/a >3 mV <5.5 ms >41 m/s 31.0
(fibular) Pop Foss—Knee 5.59 11.45 50.3 380 30.9
Tibial AH Ankle 7.50 7.70 n/a >8 mV <5.8 ms >41 m/s 30.8
Pop Fossa—Knee 7.25 15.70 48.1 385 30.6
Tibial ADQP Ankle 6.50 13.45 7.80 3.15 n/a n/a >4 mV <6 ms >41 m/s 30.1 30.5
Nerve Stimulus Recording F-waves
Lat (ms)
L R
Tibial Ankle AH 56.10
M-Wave H-Wave
Tibial Pop Fossa Soleus Left 3.9 ms 15.4 mV 27.5 ms 4.7 mV
Needle EMG summary
L Abductor Hallucis 1+ 1+ 0 0 2− Rapid Many 1+ Some 1+ Few 1+ NC
Extensor digitorum brevis Norm 0 0 0 Norm Full Norm Norm Norm NC
Abductor Digiti Quinti Pedis 1+ 1+ 0 0 2− Rapid Some 1+ Some 1+ Norm NC
Flex. Digitrum Longus Norm 0 0 0 Norm Full Norm Norm Norm NC
Tibialis Anterior Norm 0 0 0 Norm Full Norm Norm Norm NC
Gastrocnemius, medial head Norm 0 0 0 Norm Full Norm Norm Norm NC
EDB extensor digitorum brevis, AH abductor hallucis, ADQP Abductor digiti quinti pedis
left tibial mononeuropathy at another level/site, a gery, the patient noticed weakness on right knee
left lumbosacral (particularly S1) radiculopathy, extension with the inability to lock the knee when
or early manifestation of a generalized large fiber standing to prevent buckling of the leg. Despite
peripheral polyneuropathy. physical therapy, the weakness did not improve
Nerve conduction studies disclosed significant over time, now accompanied by numbness and
prolongation of the peak/distal latency of the left “pins and needles” paresthesia in the anterior
medial plantar mixed nerve response, as well as thigh and medial leg on the right.
distal latency of the left tibial motor responses Examination revealed mildly reduced muscle
recording the abductor hallucis (AH) and the bulk in the right quadriceps, with strength
abductor digiti quinti pedis (ADQP) muscles. reduced particularly with right knee extension
Other sensory and motor nerve conduction (MRC grade 2/5), noting normal strength in all
responses (including the left tibial H-reflex other muscle groups, but reduced sensation to
response, and contralateral tibial/plantar light touch, temperature and pinprick in the right
responses) were within normal limits. Needle anterior thigh, medial knee and medial calf area.
EMG of left lower limb disclosed mild-to- There was an absent right knee jerk, but all other
moderate chronic motor axon loss changes in the deep tendon reflexes were normal.
AH and ADQP muscles, with normal findings in Electrodiagnostic studies (see Table 5.10)
other muscles (including extensor digitorum bre- were done to work up the differential diagnoses
vis). Both AH and ADQP also demonstrated some which included a right femoral neuropathy, and
fibrillation potentials. less likely a lumbar plexopathy or lumbar radicu-
In this case, the electrodiagnostic findings are lopathy (especially L2–L4 nerve root lesions).
consistent with a left tibial mononeuropathy at or Nerve conduction studies demonstrated an
distal to the tarsal tunnel, compatible with a clinical absent right femoral motor response recording
diagnosis of left tarsal tunnel syndrome. This lesion rectus femoris, and an absent right saphenous
would be considered at least moderate in degree sensory response. Additional routine nerve con-
electrically, especially considering the degree of duction studies of the lower extremity including
distal/peak latency prolongation of the medial plan- peroneal (fibular) motor, tibial motor, as well as
tar mixed nerve response and the distal latency pro- sural and superficial peroneal (fibular) sensory
longation of the tibial motor responses recording responses were normal.
the AH (medial plantar nerve supplied) and ADQP The needle EMG showed abundant fibrillation
(lateral plantar nerve supplied), plus the denerva- and positive sharp wave potentials in the right
tion changes exclusively appreciated in these two quadriceps (rectus femoris and vastus lateralis
muscles. muscles), with only a few to some rapidly-firing
large and polyphasic MUAPs in each of these
two muscles. The iliacus, adductor longus, lum-
Femoral Neuropathy bar paraspinal muscles, as well as the muscles
below the knee were all normal on needle EMG.
H In this case, the electrodiagnostic findings are
Findings consistent with a subacute on early chronic right
A 50-year-old woman complained of persistent femoral mononeuropathy, predominantly axon
right leg weakness and difficulty walking loss in character and severe in degree electri-
3 months after a total hip replacement surgery via cally, localizable at or distal to the inguinal liga-
an anterior approach. Immediately following sur- ment (i.e. distal to the branch supplying the
118

Nerve Stimulus Recording L R L R L R L R Amp (μV) Norm LatNPk (ms) Norm CV (m/s) L R
Sural Lower leg Lateral malleolus 14 3.6 48 140 >6 <4.4 >41 30.8
Superficial Lower leg Dorsolateral ankle 9.4 2.5 46 100 >6 <4.4 >41 31.0
peroneal
(fibular)
Saphenous Medial calf Medial/anterior ankle 10.3 Absent 2.6 Absent 42 Absent 140 >4 <4.6 >41 31.1
B-P Amp Dist
(mV) LatOn (ms) CV (m/s) (mm) Norm B-P Temp (°C)
Nerve Recording Stimulus L R L R L R L R Amp (mV) Norm LatOn (ms) Norm CV (m/s) L R
Tibial AH Ankle 6.7 2.9 >4 <6 >40 31.8
Pop Foss-knee 6.4 12.3 40.8 385
Peroneal EDB Ankle 3.0 4.0 >2.5 <6 >40 31.8
(fibular) Popliteal fossa 2.8 11.6 50.0 380
Femoral Rectus femoris Middle of inguinal area 3.5 Absent 4.3 Absent Absent >3 <6.5 >40 30.2
F-wave studies
F-waves
Lat(ms)
Tibial Ankle AH 49.7
Needle EMG summary
R Iliacus 0 0 0 0 Norm Norm Norm Norm NC
Vastus lateralis 1+ 2+ 2+ 0 3− Rapid Few 1+ Few 1+ Few 1+ NC
Rectus femoris 1+ 3+ 2+ 0 3− Rapid Some 1+ Few 1+ Some 1+ NC
Gastrocnemius, medial head 0 0 0 0 Norm Norm Norm Norm NC
Tibialis anterior 0 0 0 0 Norm Norm Norm Norm NC
Extensor digitorum brevis 0 0 0 0 Norm Norm Norm Norm NC
Adductor longus 0 0 0 0 Norm Norm Norm Norm NC
Lumbar paraspinals (Mid) 0 0 0 0 Norm Norm Norm Norm NC
EDB extensor digitorum brevis, AH abductor hallucis
iliacus muscle). This is supported by the findings 10. Hermiz SJ. Evidence-based medicine: current evi-
dence in the diagnosis and management of carpal tun-
of an absent right femoral motor response nel syndrome. Plast Reconstr Surg. 2017;140:1
(recording rectus femoris) and an absent right 11. Ballestero-Pérez R. Effectiveness of nerve gliding
saphenous response, and evidence of marked exercises on carpal tunnel syndrome: a systematic
active/ongoing denervation with early chronic review. J Manipulative Physiol Therap. 2017;40(1).
12. Mediouni Z. Is carpal tunnel syndrome related to
motor axon loss changes in the rectus femoris computer exposure at work?. JOEM. 2014;56(2).
and vastus lateralis muscles only. 13. Smeraglia F. Endoscopic cubital tunnel release: a
systematic review. Br Med Bull. 2015;116:155–63.
https://doi.org/10.1093/bmb/ldv049.
14. Jonathan Robert Staples MD. Cubital tunnel syn-
References drome: current concepts. J Am Acad Orthop Surg.
2017;25:e215–24.
1. Sternbach G. The carpal tunnel syndrome. J Emerg 15. Ren Y-M. Open versus endoscopic in situ decompres-
Med. 1999;17(3):519–23. sion in cubital tunnel syndrome: a systematic review
2. Chen P-C. A Bayesian network meta-analysis: and meta-analysis. Int J Surg. 2016;35:104e110.
comparing the clinical effectiveness of local corti- 16. Anderson JC. Common fibular nerve compres-
costeroid injections using different treatment strate- sion anatomy, symptoms, clinical evaluation, and
gies for carpal tunnel syndrome. BMC Musculoskel surgical decompression. Clin Podiatr Med Surg.
Disord. 2015;16:363. https://doi.org/10.1186/ 2016;33:283–91.
s12891-015-0815-8. 17. Bregman PJ. Current diagnosis and treatment of
3. Newington L. Carpal tunnel syndrome and work. Best superficial fibular nerve injuries and entrapment. Clin
Pract Res Clin Rheumatol. 2015;29:440e453. Podiatr Med Surg. 2016;33:243–54.
4. Vasiliadis HS. Treatment of carpal tunnel syndrome. 18. Doneddu PE. Tarsal tunnel syndrome: still more
A systematic review and meta-analysis. PLoS One. opinions than evidence. Status of the art. Neurol
10(12):e0143683. https://doi.org/10.1371/journal. Sci. 2017;38:1735–9. https://doi.org/10.1007/
pone.0143683. s10072-017-3039-x.
5. Ellis R. Is there a relationship between impaired 19. Tladi MJ. Schwannoma and neurofibroma of the
median nerve excursion and carpal tunnel syndrome? posterior tibial nerve presenting as tarsal tunnel syn-
A systematic review. J Hand Ther. 2017;30:3e12. drome: review of the literature with two case reports.
6. Yi-Ming Ren MD. Efficacy, safety, and cost of sur- Foot. 2017;32:22–6.
gical versus nonsurgical treatment for carpal tunnel 20. Casazza GC. Systematic review of facial nerve out-
syndrome. Medicine. 2016;95:40. comes after middle fossa decompression and trans-
7. Yunoki M. Importance of recognizing carpal tunnel mastoid decompression for bells’s palsy with complete
syndrome for neurosurgeons: a review. Neurol Med facial paralysis. Otol Neurol. 2018;39:1311–8.
Chir (Tokyo). 2017;57:172–83. 21. Nicholas S. Andresen. Facial nerve decompres-
8. Lim YH. Median nerve mobilization techniques in sion. Curr Opin Otolaryngol Head Neck Surg.
the treatment of carpal tunnel syndrome: a systematic 2018;26:280–5.
review. J Hand Ther. 2017;30:397e406.
9. Bekhet AH. Efficacy of low-level laser therapy in
carpal tunnel syndrome management: a system-
atic review and meta-analysis. Lasers Med Sci.
2017;32:1439–48.
Polyneuropathies
6
Megha Chetan Dhamne and John A. Morren
Introduction Classification of Polyneuropathy
Polyneuropathy (often also referred to as peripheral Polyneuropathies may be classified based on:
neuropathy) is a broad term that encompasses con-
ditions characterized by a generalized disorder of 1. Pathogenesis
peripheral nerves. By fairly conservative estimates, 2. Temporal evolution
it affects 1–3% of the population, with incidence 3. Type of nerve fibers involved/modalities
increasing with age to 3–5% above the age of 50 affected
years [1]. Most commonly, patients present with 4. Pattern of neuropathy
sensory symptoms such as numbness or paresthesia
starting in the toes/feet (i.e. following a length-
dependent pattern). Common causes of polyneu- Pathogenesis
ropathy include diabetes mellitus, chronic
alcoholism, nutritional deficiencies such as vitamin Based on the pathogenesis, polyneuropathies can
B12 and immune-mediated conditions. Leprosy or be divided into:
Hansen’s disease is still considered among the lead-
ing causes of polyneuropathy worldwide, albeit rare 1. Axon loss polyneuropathies
in most developed countries. Each of these have dis- 2. Demyelinating polyneuropathies
tinct clinical and electrophysiological features. 3. Nodopathies/Paranodopathies
Hence a systematic and practical approach is needed
for cost-effective diagnosis and early recognition of Polyneuropathies are traditionally classified
treatable forms of polyneuropathy. into axon loss (axonal) or demyelinating accord-
ing to whether the pathologic process primarily
affects the axon itself or the Schwann cell
M. C. Dhamne
Department of Neurology, Dr. L H Hiranandani
myelin sheath. Electrophysiological studies are
Hospital, Mumbai, Maharashtra, India helpful in determining whether the neuropathy
e-mail: megha.dhamne@hiranandanihospital.org is axonal or demyelinating. Peripheral nerves
J. A. Morren are surrounded by an incomplete blood-nerve
Neuromuscular Center, Neurological Institute, barrier. Additionally, the length of the nerve is
Cleveland Clinic, and Cleveland Clinic Lerner an important factor in the vulnerability of axons
to injury. Microtubules are used as molecular
e-mail: morrenj@ccf.org tracks to guide delivery of cargoes (such as

https://doi.org/10.1007/978-3-030-74997-2_6
122 M. C. Dhamne and J. A. Morren
newly synthesized proteins, lipids, RNA, and Autoantibodies have been implicated in the
organelles) to different parts of the axon. Axonal pathogenesis of acquired demyelinating neuropa-
transport is bidirectional and is essential for the thy such as anti-GQ1B antibodies in Miller
nutrition of axons. An intact microtubule net- Fisher syndrome. Defective genes encoding for
work is required for the clearance of damaged structural myelin proteins are implicated in the
organelles by cellular degradation. Disturbance pathogenesis of inherited demyelinating neurop-
in the axonal transport or alterations in the cyto- athies [e.g. PMP22 deletion causing Hereditary
skeleton by various insults such as toxins, meta- Neuropathy with liability to Pressure Palsies
bolic alterations, vitamin deficiencies, and (HNPP)].
inflammation causes peripheral axonal neuropa- Nodopathies are disorders of the nodal/parano-
thies. Improving these mechanisms may facili- dal region including channelopathies affecting
tate regeneration of axons and are explored as those sites. Some toxic, hereditary and immune
potential therapeutic targets in axonal neuropa- mediated conditions cause a physiological con-
thies. Inherited axonal neuropathies include duction failure at the nodes of Ranvier resulting in
those that are a result of defective genes that a transient slowing or conduction block. Patients
influence axonal transport (example: MFN2, often show a quick clinical recovery. A notable
DYNC1H1, HSPB1) [2]. example of this is a recently described entity,
Integrity of the myelin sheath is essential for early reversible conduction failure (ERCF) in
effective nerve function. Disorders of the “axonal” Guillain–Barré syndrome (GBS) [4].
Schwann cells/myelin sheath cause demyelinat-
ing neuropathies which may be either hereditary
or acquired. The pathologic hallmark of acquired Temporal Evolution
inflammatory demyelinating polyradiculoneu-
ropathies is segmental demyelination that may Peripheral neuropathies may be classified
begin at the nodes of Ranvier, usually extending depending on their time course as acute (days
to the internodal area and accompanied by lym- ~4 weeks), sub-acute (~4–8 weeks) and
phocytic infiltration [3]. Acute inflammatory chronic (> ~8 weeks) (see Table 6.1). Acute
demyelinating polyradiculoneuropathy (AIDP) polyneuropathies include GBS and its vari-
and chronic inflammatory demyelinating polyra- ants [for e.g. Acute Inflammatory
diculoneuropathy (CIDP) are the commonest Demyelinating Polyradiculoneuropathy
acquired inflammatory demyelinating polyradic- (AIDP), Acute Motor Axonal Neuropathy
uloneuropathies. Determining whether the under- (AMAN), Acute Motor and Sensory Axonal
lying polyneuropathy is demyelinating is crucial Neuropathy (AMSAN), Miller Fisher syn-
as this is a potentially treatable neuropathy. drome (MFS)], as well as less common enti-
Table 6.1 Classification of polyneuropathies by temporal course

Acute Subacute Chronic
GBS and variants (AMAN, AMSAN, MFS, PCB, AMAN/ Subacute GBS Chronic axon loss
AMSAN with reversible conduction failure, AIDP) polyneuropathy
Acute porphyria Mononeuritis CIDP and variants
multiplex (MNM)
Acute thallium toxicity Paraproteinemic
neuropathy
Acute diphtheria Hereditary neuropathies
(CMT, HNPP)
Abbreviations: GBS Guillain–Barré syndrome, AMAN acute motor axonal neuropathy, AMSAN acute motor and sensory
axonal neuropathy, MFS Miller Fisher syndrome, PCB pharyngeal-cervical-brachial variant, AIDP acute inflammatory
demyelinating polyradiculoneuropathy, CIDP chronic inflammatory demyelinating polyradiculoneuropathy, CMT
Charcot-Marie Tooth disease, HNPP hereditary neuropathy liable to pressure palsies
6 Polyneuropathies 123
ties like acute porphyria. Subacute mally painful. Allodynia is an increased painful
neuropathies include subacute GBS, and mono- response to a normally innocuous stimulus.
neuritis multiplex due to vasculitis. Chronic Common autonomic symptoms are orthostatic
neuropathies are either due to axon loss or light-headedness, heat or cold intolerance, bloat-
demyelination. Chronic Inflammatory Demy ing, constipation, diarrhea, urinary retention, or
elinating Polyradiculoneuropathy (CIDP) and change in the frequency of urination and sexual
porphyria are among those which may have a dysfunction. Diabetic neuropathy, acute por-
relapsing and remitting course [5]. phyric neuropathy, GBS, amyloid neuropathy
and other small fiber neuropathies may have
autonomic involvement.
ype of Modality Affected/Type
T Nerve fibers are generally categorized by fiber
of Nerve Fiber Affected size into large-diameter, myelinated fibers and
small-diameter unmyelinated fibers. Motor fibers
1. Modalities affected—motor/sensory/autonomic are large-diameter myelinated fibers while auto-
2. Type of nerve fiber affected—large fiber/ nomic fibers are small, unmyelinated c-fibers.
small fiber Sensory fibers are either large diameter fibers that
carry sensation of vibration and joint position (pro-
Polyneuropathy is clinically suspected based prioception) or small thinly myelinated A-delta
on the characteristic pattern of sensory and/or fibers that carry sensation of somatic pain and tem-
motor involvement. Most polyneuropathies perature. It is important to recognize clinically and
involve both sensory and motor fibers. Initially a electrophysiologically the type of nerve fiber
patient may have only sensory symptoms. Sensory involved as the causes of each are varied. A large
symptoms may be classified as positive (increased fiber neuropathy may cause muscle weakness, tin-
or hyperfunction of the sensory pathways) or neg- gling or numbness, with sensory ataxia due to loss
ative (due to decreased or lack of function of sen- of proprioception. Small fiber neuropathy causes
sory pathways) [6]. Positive sensory symptoms loss of pain and temperature sensation, and/or auto-
include tingling or prickling sensation. Patients nomic dysfunction. It manifests as a “painful” neu-
often describe it as a sensation of “pins and nee- ropathy. Patients complain of “burning pain”,
dles”, “ants crawling sensation”, “bunched up dysesthesia, hyperalgesia and allodynia.
socks under the toes” or an “electric shock-like” Small fiber neuropathy most commonly pres-
sensation. Negative sensory symptoms include ents as a length dependent neuropathy, with a loss
numbness or loss of sensation to touch or tem- of pain and temperature sensation in a glove-
perature. Sensory loss in the feet may go unrecog- stocking distribution. Sometimes patients may
nized in very slowly progressive neuropathies present with focal, asymmetric symptoms as
such as hereditary neuropathy, diabetic polyneu- mononeuritis multiplex or with a proximal sen-
ropathy or in the elderly. Loss of proprioception sory impairment as in sensory ganglionopathy.
may cause gait imbalance leading to increased Causes of small fiber neuropathy include diabe-
falls, especially at night or in dim light. tes mellitus/prediabetes ± metabolic syndrome,
Pain when present, may be extremely severe hypothyroidism, vitamin B12 deficiency, vitamin
and the most disabling symptom. Pain may be the B6 excess, excessive alcohol, celiac disease, col-
heralding symptom in ischemic and some inflam- lagen vascular diseases, HIV, sarcoidosis, che-
matory neuropathies or small fiber neuropathy. motherapy, amyloidosis and hereditary causes.
Dysesthesias, hyperalgesia, and allodynia are Despite extensive work up, 30–50% patients with
terms used to describe abnormal sensations. small fiber neuropathy are labelled as “idio-
Dysesthesia is an unpleasant abnormal sensation, pathic”. Diagnosis of small fiber neuropathy
whether spontaneous or evoked. Hyperalgesia requires a detailed history, physical examination,
(increased perception of painful sensation) is an a skin biopsy to evaluate for the intraepidermal
increased sensitivity to a stimulus that is nor- nerve fiber density and/or Quantitative Sudomotor
Axon Reflex Test (QSART). Small fiber neuropa- fibers which are generally thinner are most vul-
thy is not detected on EMG. EMG in this setting nerable to the underlying insult. Some acquired
is usually done to look for or rule out a large fiber demyelinating neuropathies are classically distal,
neuropathy, that may be coexisting, based on (e.g. distal acquired demyelinating neuropathy),
clinical features [7]. as are many hereditary polyneuropathies.
Typically, a large fiber neuropathy is a mixed
sensorimotor neuropathy. Some large fiber neu- istal Symmetric Polyneuropathy
D
ropathies may present as predominantly motor or Distal symmetric polyneuropathy (DSP) is one of
pure motor neuropathies. Patient presents with the commonest basis of referral for electrodiagnos-
pure weakness, and no or minimal sensory symp- tic testing. DSP may be a large fiber and/or a small
toms. Upper motor neuron (UMN) type weak- fiber neuropathy. Diabetes mellitus and/or impaired
ness suggests disease of the spinal cord, brainstem glucose tolerance accounts for 32–53% of cases.
or higher in the corticospinal tract or motor cor- Other causes of DSP include hereditary, metabolic,
tex. Lower motor neuron (LMN) weakness local- nutritional (e.g. B12 deficiency), alcohol/toxic/
izes the lesion to anterior horn cell (AHC) (e.g. drug-related, infectious (HIV, leprosy), inflamma-
spinomuscular atrophy, progressive muscular tory, autoimmune, paraproteinemia-related and
atrophy), nerve roots, plexus, peripheral nerves, paraneoplastic. Despite a thorough evaluation, the
or neuromuscular junction. A thorough clinical cause may remain idiopathic in approximately
evaluation and EMG is crucial for the localiza- 30% of cases.
tion of the cause of muscle weakness along the Diabetes mellitus is the commonest cause of
neuroaxis. chronic axon loss length dependent sensorimotor
polyneuropathy in the United States, affecting up
to 50% patients with type 1 and type 2 diabetes
Pattern of Polyneuropathy mellitus. However, only 10–15% of patients with
neuropathy due to diabetes mellitus may be symp-
The pattern of neuropathy is determined based on tomatic. The occurrence of neuropathy in diabetes
the distribution of motor and sensory involvement. mellitus correlates with the duration of diabetes,
The most commonly encountered neuropathy is glycemic control, and presence of microvascular
distal, symmetric axonal sensory/sensorimotor ischemia with nephropathy and retinopathy [8].
neuropathy. However, it is important to recognize Electrophysiologic studies commonly show fea-
atypical patterns such as non-length dependence, tures of axonal degeneration and at least subtle
asymmetry, upper limb predominance, predomi- segmental demyelination. The reason for slow
nantly motor neuropathy as these may be poten- conduction velocity was believed to be due to loss
tially treatable neuropathies. of fast-conducting large fibers or an alteration at
The pattern of neuropathy is assessed as: the nodes of Ranvier. Conduction block or tempo-
ral dispersion are not found, however the degree of
1. Length dependent/Non-length dependent slowing or increase in distal latency may almost
2. Symmetric/Asymmetric reach demyelinating criteria. These findings have
to be interpreted with caution, as it needs to be dif-
It is useful to identify the pattern of polyneu- ferentiated from chronic inflammatory demyelin-
ropathy first as length dependent or non-length ating neuropathy (CIDP) to avoid unnecessary
dependent. The next step is to assess if the weak- immune therapy [9].
ness and/or the sensory deficits are symmetrical
or asymmetrical in distribution. Inherited (Hereditary)
Polyneuropathies
ength Dependent Polyneuropathy
L Inherited polyneuropathies are diverse group of
Axon loss polyneuropathies are often length disorders, both clinically and genetically and
dependent. This is because the distal-most nerve may present with systemic features or central
nervous system involvement. They are often and vibration. Muscle weakness when present, is
overlooked as the cause of chronic length depen- confined to the distal muscles (toes, ankles, fingers
dent, motor predominant or sensorimotor poly- and wrists), thus differentiating it from the more
neuropathy. A positive family history is useful common clinical presentation of proximal and dis-
but may be difficult to obtain as patients may tal weakness in chronic inflammatory demyelinat-
have a normal lifespan with or without minimal ing neuropathy (CIDP). Two-thirds of patients with
disability. Some hereditary polyneuropathies DADS neuropathy harbour a monoclonal gammop-
may have an acute/subacute onset. They may athy, which is almost exclusively IgM monoclonal
have fairly specific characteristics, for e.g.: (1) protein (called DADS-M). More than 90% are men
myelin protein zero (MPZ) mutation phenotype, with onset in the sixth decade or later. In most series,
is sometimes accompanied by an Adie pupil or one half to two-thirds patients with DADS-M neu-
bulbar involvement; (2) PMP22 deletion, leading ropathy patients may express anti-myelin associ-
to hereditary neuropathy with liability to pressure ated glycoprotein antibodies (anti-MAG antibodies).
palsies (HNPP); (3) septin 9 (SEPT9) mutation, MAG is a glycoprotein localized to the periaxonal
presenting with attacks of brachial plexopathy; membranes of myelin forming Schwann cells.
(4) androgen receptor (AR) mutation, causing Patients with DADS-M neuropathy with anti-MAG
spinal bulbar muscular atrophy (Kennedy’s dis- antibodies either do not respond or respond poorly
ease) and (5) mitofusin 2 (MFN2) mutation, pre- to immunomodulating therapies [12].
senting with sudden-onset optic neuropathy [10].
Inherited polyneuropathies may be classified Non-length Dependent
broadly to include: Polyneuropathies
After determining that the pattern of polyneurop-
1. Hereditary motor sensory neuropathy athy is non-length dependent, it may be further
(HMSN), or Charcot-Marie-Tooth disease classified as symmetric vs asymmetric based on
2. Hereditary sensory autonomic neuropathy the distribution of weakness and/or sensory find-
(HSAN) or hereditary sensory neuropathy ings (see Table 6.2).
(HSN)
3. Distal hereditary motor neuronopathy Neuropathies with Symmetric,
(dHMN), affecting the lower motor neurons. Predominantly Proximal Weakness
It overlaps phenotypically and genetically The importance of identifying predominantly
with HMSN proximal, symmetric weakness cannot be over-
4. Hereditary brachial plexus neuropathy emphasized as this identifies an important subset
(HBPN), of patients with potentially treatable acquired
5. Hereditary neuropathy with liability to pres- demyelinating neuropathies. This clinical pattern
sure palsies (HNPP), presenting as recurrent is the hallmark of acute and chronic acquired
mononeuropathies, often at compressible sites inflammatory demyelinating polyradiculoneurop-
athies (AIDP and CIDP), and their variants. GBS
is the acute acquired polyradiculoneuropathy
istal Acquired Demyelinating
D which incorporates demyelinating (AIDP) and
Symmetric (DADS) Neuropathy axonal (AMAN and AMSAN) variants. Clinically,
Distal acquired demyelinating symmetric (DADS) patients present with ascending paralysis with
neuropathy is another rare distal symmetric demy- areflexia and minimal sensory involvement
elinating neuropathy that should be differentiated (except for AMSAN). Acute porphyric neuropa-
from the axonal neuropathy and hereditary neurop- thy mimics GBS. However, it occurs during por-
athy. Katz et al., 2000 [11] described patients with phyric attacks and usually after exposure of
DADS neuropathy presenting with chronic length- provocating factors, commonly medications (e.g.
dependent, distal symmetric sensory symptoms dapsone, isoniazid, metronidazole, tricyclic anti-
with sensory ataxia due to loss of proprioception depressants, anti-epleptics).
Table 6.2 Non-length dependent polyneuropathies

Predominant upper extremity
Proximal symmetric weakness Asymmetric weakness
weakness
• Acute: • Acute porphyric neuropathy • Proximal asymmetric weakness:
• GBS and variants • Lead intoxication neuropathy • Polyradiculoplexopathy:
• Acute Porphyric Neuropathy# • CIDP variants: • DLSRPN
• Toxic neuropathy • MMN with CB • Infectious (TB, HIV, Lyme)
• Tick paralysis • MADSAM • Malignant infiltration
• Familial brachial plexopathy
• Chronic:
• CIDP and variants • Distal asymmetric weakness:
• Paraproteinemic neuropathy • Motor neuropathy
• Multiple mononeuropathies:
• Entrapment
• Inherited (HNPP)
• MNM: Infectious (leprosy, HIV)
• Vasculitis
• Paraneoplastic
• CIDP variants:
• MADSAM,
• MMN with CB
Abbreviations: GBS Guillain Barré syndrome, CIDP chronic inflammatory demyelinating polyradiculoneuropathy,
MMN with CB multifocal motor neuropathy with conduction block, MADSAM multifocal acquired demyelinating sen-
sory and motor neuropathy, HNPP hereditary neuropathy with liability to pressure palsies, DLSRPN diabetic lumbosa-
cral radiculoplexus neuropathy, MNM mononeuritis multiplex
#
Acute porphyric neuropathy may be asymmetric but classically resembles GBS
Guillain–Barré syndrome (GBS) Table 6.3 Clinical and electrophysiological variants of

GBS is the most common and the most severe Guillain–Barré syndrome (GBS)
acute paralytic polyneuropathy, with about 1. Acute inflammatory demyelinating
100,000 people worldwide developing the dis- polyradiculoneuropathy (AIDP)
order every year. The incidence increases with 2. Acute motor axonal neuropathy (AMAN)
3. Acute motor sensory axonal neuropathy (AMSAN)
age and is slightly more frequent in males than
4. Miller Fisher syndrome (MFS)
females. Approximately 70% patients have a
5. Bickerstaff brainstem encephalitis (BBE)
history of upper respiratory or gastrointestinal 6. Pharyngo-cervico-brachial variant (PCB)
infection, trauma, surgery, or vaccination 7. Paraparetic variant
within 4 weeks prior to the onset of weakness. 8. Facial diplegia variant
Under the umbrella term of GBS are several 9. Pure sensory GBS
recognisable variants with distinct clinical and 10. Pure autonomic failure variant
pathological features (Table 6.3). Patients pres- 11. Reversible conduction failure (nodopathy) (RCF)
ent with ascending paralysis, symmetric weak-
ness and areflexia. Cranial nerve involvement,
autonomic dysfunction and/or respiratory fail- Chronic inflammatory demyelinating
ure may occur. Cerebrospinal fluid (CSF) polyradiculoneuropathy (CIDP)
examination typically shows albuminocytologi- CIDP is the commonest chronic immune-mediated
cal dissociation. Weakness is monophasic and polyradiculoneuropathy. The pathologic features
deficits rapidly progress to reach a nadir usually of CIDP described by Dyck were “onion bulb”
by 2 weeks with no progression beyond 4 formation, perivascular inflammatory infiltrates
weeks. The severe form of GBS with respira- and segmental demyelination in teased fibers [14].
tory failure is seen in 20–30% [13]. Recovery Apart from the temporal course (>8 weeks), CIDP
may take months to years. differs from GBS in the following ways: (1)
GBS is a monophasic illness while CIDP is
relapsing-remitting or slowly progressive; (2) almost always lambda. Other plasma cell dyscra-
patients may or may not have a prior history of sias; namely, multiple myeloma, Waldenstrom’s
infection or a trigger; (3) Cranial nerve palsies, macroglobulinemia, POEMS, primary amyloidosis
autonomic dysfunction or respiratory failure rarely are commonly associated with an axon loss distal
occurs in CIDP; (4) CIDP is steroid responsive. symmetric sensorimotor polyneuropathy (see
Sometimes it may present acutely like GBS. CSF Table 6.6). The underlying plasma cell dyscrasia is
in CIDP shows albuminocytological dissociation, treated with chemotherapy and/or radiation therapy
with cell count <10/mm3. CSF pleocytosis sug- (surgical resection may be done for solitary plas-
gests a co-infection such as HIV. Most patients macytomas). Resistant cases are treated with autol-
with CIDP respond to immunomodulatory ther- ogous stem cell transplantation [15].
apy. CIDP variants are outlined in Table 6.4.
Although very rare, CIDP has been shown to be
Table 6.4 Chronic inflammatory demyelinating polyra-
associated with several malignancies: lung (typi- diculoneuropathy (CIDP) variants
cally small cell carcinoma), ovary, and uterus, 1. Multifocal acquired demyelinating sensory and
colon, pancreas, and Hodgkin’s lymphoma. motor neuropathy (MADSAM) or Lewis-Sumner
syndrome
Paraproteinemic polyneuropathy 2. Multifocal motor neuropathy with conduction block
Paraproteinemic polyneuropathies are a heteroge- (MMN with CB or MMNCB)
3. Distal acquired demyelinating symmetric
nous group of polyneuropathies associated with an neuropathy (DADS)
abnormally elevated monoclonal (M) protein (see 4. Chronic immune sensory polyradiculopathy (CISP)
Table 6.5). Typically, the neuropathy associated is a 5. Chronic ataxic neuropathy with ophthalmoparesis,
length dependent axon loss sensory/sensorimotor M protein, cold agglutinins and disialosyl
polyneuropathy. However, demyelinating polyneu- ganglioside antibodies (CANOMAD)
ropathy can also occur. Distal acquired demyelinat-
ing sensory (DADS) neuropathy is seen with IgM Table 6.5 Polyneuropathy associated with MGUS
monoclonal gammopathy of undetermined signifi-
Type of neuropathy Monoclonal protein
cance (MGUS), while a CIDP-like presentation is Distal acquired demyelinating IgM,
seen in patients with IgA/IgG MGUS, or plasma symmetric neuropathy (DADS ~50% have anti-MAG
cell dyscrasias including osteosclerotic myeloma neuropathy), antibody,
and POEMS (Polyneuropathy, Organomegaly, Rarely asymmetric neuropathy ~15% have anti-GD1b
and GQ1b antibodies
Endocrinopathy, Monoclonal protein, Skin
Distal symmetric sensorimotor IgG or IgA
changes) syndrome- the associated M protein is axonal neuropathy
typically IgG or IgA, but the light chain type is CIDP—MGUS IgG or IgA
Table 6.6 Plasma cell dyscrasias and polyneuropathy

Type of neuropathy Plasma cell dyscrasia Monoclonal protein
Distal symmetric sensory > motor Waldenstrom’s Monoclonal spike >3 g/dl
neuropathy macroglobulinemia IgM
Anti-MAG +
Distal sensory > motor neuropathy Multiple myeloma Kappa light chains
Demyelinating, motor predominant Osteosclerotic myeloma Often IgG or IgA, or elevated lambda light
neuropathy, CIDP-like chains
Demyelinating sensorimotor POEMS Often IgG or IgA, or elevated lambda light
neuropathy, CIDP-like chains (often elevated VEGF levels also)
Distal symmetric sensorimotor Primary light chain Lambda light chains predominate
neuropathy amyloidosis
CIDP Chronic inflammatory demyelinating polyradiculoneuropathy; POEMS Polyneuropathy, Organomegaly,
Endocrinopathy, Monoclonal protein, Skin changes; MAG myelin-associated glycoprotein; VEGF Vascular endothelial
growth factor
Upper Limb Predominant Weakness Table 6.7 Etiology of mononeuritis multiplex

When a patient presents with acute/subacute • Vasculitis—primary, secondary
upper limb predominant weakness, distally with • Infections—HIV, hepatitis B and C, leprosy, Lyme
a wrist drop, lead neuropathy should be sus- disease
pected. A more slowly progressive course with • Diabetes mellitus
• Cancer associated conditions—Paraneoplastic, direct
asymmetric hand/arm weakness with sensory tumor invasion with intraneural spread (lymphoma,
symptoms suggests a MADSAM neuropathy, B cell leukemia, carcinoid tumor), chronic graft vs
while pure motor weakness without sensory fea- host disease (GVHD)
tures point towards MMN with CB. These are • Infiltrative—neurosarcoidosis, amyloidosis
• Cryoglobulinemia
rare variants of CIDP that typically respond well
to immune therapy. MMN with CB needs to be
differentiated from motor neuron disease. sensorimotor neuropathy. A comprehensive array
Asymmetric weakness in the hands is also seen in of tests should be done to investigate for the
myopathies like inclusion body myositis (IBM) causes of mononeuritis multiplex. If identified
and anoctamin 5 myopathy. Rarely, neuromuscu- early and the underlying disease is treated appro-
lar junction transmission disorders like myasthe- priately then full recovery is possible, although it
nia gravis may present with hand weakness. may take months to years.
Radiculopathies are an important differential
Asymmetric Weakness that is commonly encountered in routine electro-
Distal asymmetric weakness should alert the neu- diagnostic studies. Polyradiculoplexopathies
rologist to consider radiculopathies (particularly such as diabetic lumbosacral or cervical plexop-
affecting C8-T1 or L5-S1 roots), multiple mono- athy present with proximal lower or upper
neuropathies (including lead toxicity-related radial extremity asymmetric weakness respectively. An
neuropathy that may present with wrist drop), or important differential is familial brachial plexop-
rarely motor neuron disease, or acute porphyric athy (hereditary neuralgic amyotrophy, as seen
neuropathy. Mononeuropathies may be demyelin- with mutations in the SEPT9 gene) that may
ating as in MMN with CB, compressive or entrap- present with unilateral or bilateral upper limb
ment neuropathies, and hereditary as in HNPP, or weakness.
axonal as in infectious, infiltrative, or mononeuri-
tis multiplex (often vasculitic). Patients with
HNPP may or may not have a family history. Determining the Underlying
These patients typically present with multiple Etiology: Asking the Right Questions
entrapment neuropathies, including those of ulnar, Related to the Past Medical/Surgical,
median and common peroneal (fibular) nerves. Medication, Family and Social History
An asymmetric weakness with axon loss
characteristics on electrodiagnostic studies is Past medical history of diabetes mellitus, or met-
seen in mononeuritis multiplex. This is a poten- abolic syndrome are risk factors for distal sym-
tially treatable condition and hence should not metric axonal polyneuropathy. Cancer may be
be missed. Causes are summarized in Table 6.7. associated with polyneuropathy due to the che-
The clinical presentation of mononeuritis motherapeutic medications, direct invasion into
multiplex is distinctive. It typically presents as a the nerves or plexus, late effects of radiation, or
painful subacute, stepwise, asymmetric, large as a paraneoplastic syndrome. A positive family
fiber sensorimotor polyneuropathy. Weakness history suggests a possible inherited cause for the
and sensory loss is in the distribution of multiple chronic slowly progressive polyneuropathy,
peripheral nerves of the lower and upper extrem- especially if the patient has foot deformities like
ity but when advanced it may be less focal and hammer toes and high arches. Screening the
more symmetrical/confluent. It may then be dif- other family members with EDX, and/or genetic
ficult to differentiate it from the common distal testing is sometimes carried out for establishing
the inheritance pattern and counselling the patient relates closely with muscle weakness. CMAP
and his/her family. Alcohol is another common amplitude or area may also be reduced from a
cause of distal symmetric neuropathy and a his- conduction block in acquired demyelinating neu-
tory of the duration, and amount of alcohol intake ropathies due to segmental demyelination located
needs to be specifically obtained. Exposure to between the site of stimulation and the recorded
toxins (accidental, or occupational exposure) and muscle (failure of the impulse to travel across the
medications (e.g. chemotherapy, antibiotics like point of conduction block), a presynaptic neuro-
isoniazid and metronidazole) needs to be looked muscular junction disorder such as Lambert Eaton
into thoroughly. Vitamin B12 deficiency is a myasthenia gravis (LEMS) and myopathies.
common cause of distal symmetric axonal neu- Weakness in demyelinating neuropathies is due to
ropathy in patients who are vegetarians or veg- interruption or block of conduction along the
ans, and those after bariatric surgery. motor nerves, which is reflected as slowed con-
duction or conduction block along the nerves.
While all fibers contribute to the CMAP ampli-
Electrodiagnostic Studies tude and area, only the fastest conducting fibers
in Polyneuropathy contribute to the conduction velocity and the
latency measured by routine NCS. Hence mild
Nerve conduction studies (NCS) and needle elec- slowing of conduction velocity (CV) and distal
tromyographic studies (EMG) aid in the classifi- latency occur with loss of the largest or the fastest
cation and differentiation of peripheral conducting axons in an axon loss polyneuropathy.
neuropathies. The distinction between axon loss However, marked slowing will not occur because
and demyelinating neuropathies has diagnostic even the slowest nerve fibers conduct at ~35 m/s.
and prognostic implications. The routine electro- With a random drop out of fibers in axon loss
diagnostic evaluation of patients with suspected polyneuropathies there is: (1) reduced CMAP and
polyneuropathy includes sensory NCS, motor sensory nerve action potential (SNAP) amplitude
NCS, F-wave studies, H-wave studies and needle (and area), (2) mild slowing of conduction veloc-
electrode examination (NEE). A wide variability ity; and (3) mild prolongation of distal latency. In
exists amongst neurophysiologists regarding the severe axon loss neuropathy with preservation of
use of electrodiagnostic examination techniques, few fastest fibers, CMAP amplitude decreases
reference values, interpretation of individual markedly with preservation of conduction veloc-
tests, and criteria for diagnosis and classification ity and distal latency. With loss of almost all axons
of peripheral neuropathy. However, the basic including the fastest fibers, the conduction veloc-
approaches are similar in most laboratories. ity may drop down to as low as ~35 m/s with
reduced CMAPs. However, reduction in the CV
or prolongation of distal latency will not be in the
Electrodiagnosis of Axonal demyelinating range [CV <70% lower limit of
Polyneuropathies normal (LLN) and distal latency >130% upper
limit of normal (ULN)].
Axonal or “axon loss” polyneuropathy is the com- Different patterns on NCS and EMG are seen
monest type of polyneuropathy encountered in in axonal polyneuropathy, depending on (1)
clinical practice and in the electrodiagnostic lab. Whether axon loss is acute/subacute or chronic;
These are primarily associated with Wallerian or (2) Whether it involves motor and/or sensory
axonal degeneration, sometimes referred to as fibers and (3) Distribution of polyneuropathy:
“dying back” neuropathy. When studied electro- length dependent or non-length dependent and
physiologically, this is reflected as reduced motor symmetric or asymmetric. Acute axonal polyneu-
and sensory amplitude and area. A reduced com- ropathies include inflammatory neuropathies
pound muscle action potential (CMAP) amplitude (rare variants of GBS—AMAN: acute motor
is seen in an axon loss polyneuropathy and it cor- axonal motor neuropathy, AMSAN: acute motor
sensory axonal neuropathy) and metabolic neu- examined (e.g. sural and superficial peroneal (fib-
ropathies: acute porphyria and critical illness ular) sensory nerves) are proximal to the sites
neuropathy. Electrodiagnosis of acute axonal affected very early in distal polyneuropathy. The
inflammatory neuropathies (AMAN and utility of more distal sensory nerves; plantar sen-
AMSAN) will be discussed under electrodiagno- sory/mixed and dorsal sural nerves have been
sis GBS and its variants. Subacute axonal neu- studied in improving the diagnostic yield of early
ropathies include the asymmetric mononeuritis or subclinical polyneuropathy. However, their
multiplex, seen for example in vasculitides. routine use in DSP is limited due to the absence of
Therefore, asymmetry on electrodiagnostic stud- plantar nerve responses in healthy individuals
ies should alert the electromyographer to con- over the age of 40–50 years, technical difficulties
sider vasculitic neuropathy as this is potentially in the recording, and damage in entrapment neu-
treatable. The other differential for an asymmet- ropathies of the foot, particularly tarsal tunnel
ric pattern in an axon loss neuropathy is an under- syndrome [20]. Dorsal sural nerve, the distal con-
lying or a superimposed radiculopathy. In a tinuation of the sural nerve in the foot that sup-
review of patients referred for EDX testing at an plies the lateral border of the foot, is easily
academic neurology department, NCS/EMG accessible to nerve conduction techniques because
resulted in alternative diagnoses in 43% of sus- of its superficial location and is less prone to dam-
pected cases, most often lumbar radiculopathy age by local trauma or entrapment compared to
(18%) [16]. the medial plantar and interdigital nerves.
We will discuss here the electrodiagnosis of However, it may be absent in healthy individuals,
more commonly encountered chronic axonal have a lower SNAP amplitude (dorsal sural nerve
length dependent distal symmetric polyneuropa- SNAP amplitude was found to be 50–73% lower
thy (DSP). In DSP, greater clinical and electro- than that of the sural nerve, using antidromic
physiological changes are seen distally in the recording) and has anatomic variability of its
nerves of the lower extremity before they affect branches, limiting its use in routine evaluation of
the upper extremity nerves. Since the neuropathy distal symmetric polyneuropathies [21, 22]. Sural
is chronic, enough time has passed for Wallerian SNAPs may be absent with increasing age in up to
degeneration to occur. Hence, both SNAP and 24% of healthy individuals >70 years of age, and
CMAP amplitudes are reduced. Sensory axons 40% in >80 years age. More recent studies showed
are generally involved earlier and more severely that sural SNAPs can be obtained, albeit with
affected than motor axons. reduced amplitude in 98–100% of healthy elderly
Sensory NCS, particularly in the lower extrem- individuals. Tavee et al. showed that absence of
ity are more sensitive than motor NCS in the sural SNAP response before the age of 75 years
detection of peripheral neuropathy [17]. Patients of age should be regarded as abnormal. She
may have minimal or no sensory symptoms but proposed the lower limit of normal (LLN) for
have decreased sensory responses or absent sural amplitudes as 3 μV for individuals aged
SNAPs. The earliest sign of an axon loss polyneu- 60–69 years and 1 μV for those aged 70–74
ropathy is absent or decreased sensory nerve years [23]. Lo et al. showed that even though
action potential (SNAP) amplitude of distal superficial peroneal (fibular) nerve is at equal
nerves in the lower extremity. The commonly distance to the sural nerve, it is more involved
studied sensory responses in the lower extremity in peripheral neuropathy (88.5% abnormalities
are those of the sural and superficial peroneal (fib- for superficial peroneal (fibular) nerve, com-
ular) sensory nerves. The demonstration of low pared to 75% for sural nerve in patients with
amplitude or absent sural SNAPs and normal peripheral neuropathy) [24].
radial SNAP improves the diagnostic accuracy of The only abnormality on NCS in early or mild
axonal p olyneuropathy, although recent evidence polyneuropathy may be reduced or absent sen-
supports the use of sural SNAP amplitude alone sory responses in the lower extremity. Motor
[18, 19]. The sensory nerves that are routinely NCS may be completely normal. NEE may show
evidence of active/ongoing denervation (abnor- When reinnervation occurs (within months after
mal spontaneous activity—fibrillation, positive axon loss), the motor unit action potentials
sharp wave potentials) and decreased recruitment (MUAPs) become longer in duration, higher in
limited to the intrinsic foot muscles only. amplitude and show polyphasia. Long duration,
As the polyneuropathy progresses further, high amplitude polyphasic MUAPs are not seen
axon loss involves the motor nerve fibers with in acute neuropathies. When present, they always
reduction in the CMAP amplitude seen on NCS imply a chronic neuropathy that has been present
along with a reduced/absent SNAP response of for at least a few weeks to months or longer.
sensory nerves of the lower extremity, including EMG follows a similar pattern of length depen-
sural and superficial peroneal (fibular) nerves. dent changes as seen on NCS, with muscles of
SNAPs and CMAPs of lower extremity nerves the foot (intrinsic foot muscles) and distal leg (for
are affected while those in the upper extremity e.g. tibialis anterior, tibialis posterior and gas-
are normal or less affected. trocnemius muscles) being involved earlier in a
In a severe axon loss neuropathy, sensory length dependent axonal polyneuropathy. Only
responses are absent in the lower extremity, when the polyneuropathy is relatively severe, the
motor responses are reduced to absent and there muscles above the knee (quadriceps, and ham-
is now involvement of distal nerves in the upper strings) are affected and the small muscles of the
extremity with reduced to absent sensory and hand will also show evidence of denervation–rein-
motor responses in the median and ulnar nerves nervation. Any asymmetric findings should alert
before it affects the radial nerves. As discussed the neurologist to search for a superimposed
earlier, with moderate to severe axon loss neu- radiculopathy/plexopathy or mononeuritis multi-
ropathy, there may be secondary demyelination- plex in the appropriate clinical setting. In the lower
type changes, including mild slowing of extremity, L5 and S1 radiculopathies are the com-
conduction velocity and prolongation of distal monest radiculopathies encountered. These should
latency due to loss of large diameter, fastest-con- be taken into consideration while evaluating for
ducting nerve fibers. polyneuropathy. Thus, a proximal L5-innervated
Active/ongoing motor axon loss changes occur muscle (e.g. gluteus medius) and a proximal
generally after ~3 weeks of axonal injury or insult S1-innervated muscle (e.g. short head of biceps
and reinnervation takes months. Hence the changes femoris) is routinely sampled while performing
seen in NCS and EMG may guide us to understand EMG for polyneuropathy. Denervation–reinnerva-
the chronicity of the underlying polyneuropathy. tion changes in these proximal muscles with rela-
EMG in acute—subacute polyneuropathy may tively unaffected/less affected nearby muscles
show only denervation potentials (positive sharp (e.g. the quadriceps) outside those L5-S1 myo-
waves and fibrillations), while more long-standing tomes would suggest an underlying chronic L5
neuropathies will show changes of reinnervation/ and/or S1 radiculopathy.
motor unit remodelling. Often, with routine EMG
studies, we observe a combination of denerva- ole of Electrodiagnostic Studies
R
tion–reinnervation changes which suggests that in Diabetes Mellitus
the neuropathy is subacute on chronic. In very Diabetes (including prediabetes) is the common-
slowly progressive long-standing neuropathies, est cause of chronic distal symmetric sensory or
reinnervation occurs in pace with denervation. sensorimotor polyneuropathy (DSP).
EMG may show reinnervation, with little or no Electrodiagnostic studies may not be required for
active/ongoing denervation. Decreased recruit- evaluation of every distal symmetric polyneurop-
ment may be the only finding in very acute neu- athy. Review of family history and screening
ropathies even before any changes are seen on tests for diabetes, vitamin B12 deficiency and
NCS. Reinnervation occurs when there is primary monoclonal gammopathy should be assessed in
regrowth of the axons or collateral sprouting. This almost every case of polyneuropathy. According
is a very slow process and takes months to years. to the latest AANEM policy statement [25], EDX
testing in a patient with diabetic polyneuropathy electrophysiological features that favor CIDP
is likely to be of low yield when: over DM are:
1. Symptoms and physical findings are mild 1. Features of substantial demyelination (partial
2. Clinically, distal symmetric sensory/senso- or complete motor conduction block, or
rimotor polyneuropathy is evident abnormal temporal dispersion) in at least two
3. There is a known cause (e.g. diabetes nerves.
mellitus) 2. Distal CMAP duration prolongation in at least
4. There is little suspicion of a coexisting etiol- one nerve and at least one other demyelinat-
ogy for neuropathy ing parameter in ≥1 nerve.
The potential goals of pursuing electrodiag- Another classic form of diabetic neuropathy is
nosis in diabetes mellitus is to define the extent, diabetic lumbosacral radiculoplexoneuropathy
severity and prognosis of the neuropathy and (DLRPN) or diabetic amyotrophy. This is an
identify changes that would define an alternate/ asymmetric neurogenic process and it associated
additional diagnosis. Diabetes affects both large with pain and weight loss. Femoral motor and
and small nerve fibers. As discussed earlier, the sensory nerve responses are absent or reduced in
first abnormalities in NCS in DSP are seen in the amplitude. Femoral sensory i.e. saphenous nerve
distal sensory nerves with absent or reduced study is technically difficult, especially in obese
plantar (mixed nerve), sural and/or superficial individuals. However, when absent unilaterally, it
sensory SNAP amplitudes. As larger nerve fibers may suggest a lumbar plexopathy or femoral neu-
are lost, conduction velocity decreases and distal ropathy rather than an L2-L4 radiculopathy.
latency, as well as F-wave latency increases (in EMG shows denervation–reinnervation changes
the axon loss range). As the disease progresses, in the muscles innervated by the femoral nerve
motor nerves are affected similarly. EMG shows (quadriceps, iliopsoas) and obturator nerve
denervation in distal muscles of the foot and leg. (adductor longus).
Polyneuropathy remains in the mild stage in
most cases. However, it may progress to involve Nutritional Deficiency
more proximal muscles in a glove-stocking pat- and Polyneuropathy
tern. In addition to abnormal electrodiagnostic Polyneuropathy is seen in 25% patients with vita-
studies in the lower extremities, upper extremity min B12 deficiency. Electrodiagnostic studies
nerves commonly show abnormalities such as have shown various subtypes based on NCS: 76%
entrapment neuropathies: carpal tunnel syn- axon loss peripheral neuropathy, while 24% with
drome and ulnar neuropathy in particular. NCS demyelinating features. Rare cases of demyelinat-
correlate with the clinical severity of the disease. ing neuropathy with conduction block and also an
The challenge in the electrodiagnosis of diabetic acute sensorimotor axon loss polyneuropathy have
neuropathy is when there are atypical presenta- been reported. Vitamin B12 levels may be normal
tions or there is a clinical suspicion of CIDP in a [27]. Hence the need to test for the metabolites,
patient with severe diabetic polyneuropathy. methylmalonic acid (MMA) ± homocysteine in
NCS may show absent distal sensory and motor patients with borderline levels of B12, to confirm
responses in the lower and upper extremity or the diagnosis of B12 deficiency.
severe slowing or even conduction block which B6 (pyridoxine) deficiency causes distal sym-
may raise the possibility of a superimposed pri- metric axon loss polyneuropathy. On the other
mary demyelinating disorder. In diabetes melli- hand, B6 toxicity affects the dorsal root ganglion
tus, NCS change slowly over years, so a more (sensory neuronopathy) leading to sensory ataxic
rapid decline should raise concerns about the polyneuropathy. Nerve conduction studies show
adequacy of glycemic control, or another super- widespread absent sensory responses with pre-
imposed neuropathy, especially CIDP [26]. The served or mildly affected motor responses.
Toxic Polyneuropathies symmetric polyneuropathy due to metabolic or

Electrophysiologic studies in polyneuropathies toxic insult. Clinical features (e.g. high plantar
due to neurotoxic exposure are rather non-specific. arches, hammer toes, childhood onset) with a
Very few may have characteristic electrophysio- motor predominant distal polyneuropathy and fam-
logic features. However, it is important to recog- ily history if present, may be clues to an inherited
nize these as improvement may be seen once the polyneuropathy. DADs is a distal demyelinating
exposure to a particular toxin is reduced or elimi- polyneuropathy which will be discussed in the sec-
nated. The most common pattern of toxic neuropa- tion on electrodiagnosis of demyelinating neuropa-
thy seen on NCS is distal symmetric sensory/ thies. An important consideration while performing
sensorimotor neuropathy, as seen with exposure to electrodiagnostic studies of polyneuropathy is
vincristine, chronic arsenic poisoning and ethyl motor neuron disease and radiculopathies, particu-
alcohol. Sensory predominant distal polyneuropa- larly when there is any asymmetry found on clini-
thy or a sensory neuronopathy is classically seen cal or electrodiagnostic tesing. Abnormal sensory
in patients with thallium toxicity, or with drugs/ studies rule out or lowers the possibility of amyo-
agents such as pyridoxine, platinum-based chemo- trophic lateral scleroris (ALS). In the absence of
therapy, styrene or thalidomide. Lead toxicity, sensory abnormalities, it is difficult to distinguish a
dapsone and L-tryptophan may cause mononeuri- pure motor axonal polyneuropathy (albeit a rare
tis multiplex. Lead toxicity may present with an condition) from an anterior horn cell disease or a
isolated wrist drop or a finger drop. Exposure to polyradiculopathy. Topographical distribution of
dapsone may resemble mononeuritis multiplex, changes seen in NEE provide some clues: active/
however this is a motor predominant polyneuropa- ongoing and chronic denervation changes in distal
thy. Organophosphorus poisoning may have two muscles symmetrically would suggest an axonal
different neuropathic manifestations: rapidly pro- polyneuropathy while in ALS, these changes are
gressive early distal motor axonal polyneuropathy seen asymmetrically in both proximal and distal
(within 2–3 weeks) or a delayed distal sensorimo- muscles, often accompanied by fasciculation
tor axonal polyneuropathy. Nitrofurantoin poly- potentials and motor unit instability features.
neuropathy may present with rapid onset Denervation–reinnervation changes due to radicu-
respiratory failure with painful paresthesias and lopathy are restricted to the muscles innervated by
progressive limb weakness mimicking GBS with the affected nerve root (supplied by different
an acute motor/sensorimotor axonal polyneuropa- peripheral nerves).
thy (AMAN or AMSAN). Predominantly motor
neuropathies with conduction slowing may be
seen with amiodarone, disulfiram, acute arsenic lectrodiagnosis of Demyelinating
E
exposure and n-Hexane exposure. The acute pre- Polyneuropathies
sentation of these polyneuropathies may resemble
GBS and hence a careful history of exposure to It is very important to recognize features of demy-
various toxins and medications will guide the elination during electrophysiological studies as
treating neurologist make appropriate treatment demyelinating polyneuropathies are potentially
decisions in patients not responding to plasma- treatable polyneuropathies. Mononeuropathies,
pheresis or intravenous immunoglobulin for sus- such as entrapment mononeuropathies often show
pected GBS. features of focal demyelination (at site of entrap-
ment) on NCS. When a patient has evidence of pri-
ifferential Diagnosis of Distal
D mary demyelination on NCS, the differential
Symmetric Polyneuropathy diagnosis is narrowed as shown in Table 6.8. CIDP
Many inherited polyneuropathies and DADs neu- is the most common chronic demyelinating neu-
ropathy also present with distal symmetric neuro- ropathy. Inherited demyelinating polyneuropathies
pathic features. Axonal inherited polyneuropathy are rare, typically have onset in childhood and may
may be difficult to distinguish from a severe distal have central nervous system involvement.
Table 6.8 Demyelinating polyneuropathies Secondary axon loss may be seen in severe demy-
Symmetric elinating polyneuropathy with reduction in
Hereditary CMAP amplitudes.
• Hereditary sensorimotor neuropathy type I
(Charcot–Marie-Tooth 1)
Electrodiagnosis of Acute
• Hereditary sensorimotor neuropathy type III
(Dejerine-Sottas) Inflammatory Demyelinating
• Hereditary sensorimotor neuropathy type IV Polyradiculoneuropathy (AIDP)
(Refsum disease) Electrophysiologic studies play an important role in
• Krabbe disease the diagnosis of GBS, classification of the subtypes
• Metachromatic leukodystrophy and in establishing prognosis. Albuminocytological
• Adrenoleukodystrophy dissociation in cerebrospinal studies or abnormal
• Cockayne syndrome
NCS may not be demonstrable in the first few days
• Niemann-Pick disease
of the illness as demyelination is segmental or
• Cerebrotendinous xanthomatosus
Acquired
patchy. This makes the diagnosis challenging. Early
• diagnosis is important, however, because early
polyradiculoneuropathy (AIDP) treatment has been shown to improve outcomes.
• In the first few days, nerve conduction studies
polyradiculoneuropathy (CIDP) are normal despite clinical weakness. Prolonged
• CIDP associated with paraproteinemia (e.g.- IGA/ or absent late responses: particularly, F-wave
IgG MGUS, Osteosclerotic myeloma, POEMS),
malignancy (lymphoma) latency and tibial H-reflex are the earliest findings
• CIDP variants: particularly distal acquired on nerve conduction studies. Wilbourn et al. stud-
demyelinating symmetric neuropathy (DADS) ied the electrodiagnostic findings in the first week
Asymmetric of illness in GBS. He reported an absent tibial
Mononeuropathies H-reflex in 97% of patients with early GBS. Other
• Entrapment or compression neuropathy abnormal early electrodiagnostic findings were:
• Hereditary neuropathy with liabilty to pressure
abnormal F-wave (84%), combination of abnor-
palsies (HNPP)
CIDP variants mal sensory responses in the upper extremity with
• Multifocal motor neuropathy with conduction a normal sural response (67%) and evidence of
block (MMN with CB) demyelination (slow conduction velocity, pro-
• Multifocal acquired demyelinating sensory and longed distal latency, conduction block and tem-
motor neuropathy (MADSAM) poral dispersion). Although very sensitive, an
absent tibial H-reflex, by itself is non-specific.
However, along with an abnormal F-wave, abnor-
Myelin is essential for saltatory conduction mal upper extremity SNAP and normal sural
along the nerves. Loss of myelin results in slow- SNAP, these findings are characteristic of early
ing of conduction. Electrophysiologically, demy- demyelinating GBS or AIDP. Hence, Wilbourn
elination is determined by the following: suggested that the tibial H-reflex is the most sensi-
tive test for early GBS and should be a part of the
• Slowing of conduction (prolongation of peak/ standard NCS protocol for GBS. If multiple
distal latencies, slowing of nerve conduction nerves are tested, a definitive diagnosis of AIDP is
velocity, prolongation/absence of F-wave possible in half of the patients but not until the
latency) fifth day after the onset of symptoms [28].
• Conduction block Normal peripheral nerve conduction study
• Temporal dispersion of Compound Muscle with an abnormal F-wave (absent or slow) as an
Action Potential (CMAP) configuration isolated finding was seen in 10–30% of patients
in early GBS [29]. Absent or reduced F-waves
The electrodiagnostic hallmark of acquired with preserved distal CMAPs is used to assess
demyelination is conduction block and temporal demyelination in the proximal segments. At least
dispersion of M-wave/CMAP configurations. four mechanisms account for the selectively
abnormal F-wave in early GBS: (1) demyelinat- patient’s body habitus, including effects of obe-
ing conduction block in the proximal segment (if sity or large neck girth.
normal distal CMAPs), (2) proximal axonal Acute inflammatory demyelinating polyradic-
degeneration (3) physiological conduction failure uloneuropathy (AIDP) is electrophysiologically
causing a proximal conduction block (4) impaired characterized by conduction slowing, conduction
excitability of the motor neuron. block and temporal dispersion. Due to loss of
A-wave (axon reflex) is seen in demyelinating myelin, nerve conduction velocity is either mark-
polyneuropathies, often in the first several days edly slow or blocked. Slowing of nerve conduc-
of illness in GBS. Hence, A-waves may also be tion is associated with marked slowing of
utilized as a marker of early demyelination. conduction velocity (<70% LLN) and/or prolon-
gation of distal motor latency (>130% ULN) and
“Sural sparing” pattern in GBS prolongation of late responses—F-wave latency
“Sural sparing” pattern is described in acute and (>130% ULN). SNAPs are often low or absent
chronic demyelinating neuropathies. Definition of (outside of sural sparing). CMAPs are reduced in
“abnormal sural pattern” is variable and different amplitude depending on the site of demyelination
patterns have been studied in GBS. It was first that produces a conduction block and/or temporal
described by Bromberg and Albers in 1993 as dispersion. Conduction block is defined as
“abnormal median normal sural” and shown to be obtaining a response at a proximal site of stimu-
100% specific for AIDP and CIDP versus motor lation with a drop in CMAP amplitude or area
neuron disease and diabetes mellitus polyneurop- >50% when CMAP duration increase is <30%.
athy [30]. Al-Shekhlee et al., in their 2005 paper Temporal dispersion is defined as >30% increased
described it as “normal or relatively preserved duration of proximal CMAP compared to the dis-
sural sensory nerve action potential (SNAP) com- tal CMAP.
pared with at least two abnormal SNAPs in the 80% of patients with AIDP have evidence of
upper limb” [31]. Sural sparing (abnormal ulnar/ nerve conduction slowing or conduction block at
normal sural response) has been found to be most some point of their illness although up to 20%
specific in distinguishing GBS from its mimics in will have normal conduction studies. The sites of
a multicenter study [32]. Sural sparing was seen demyelinating conduction slowing, and block are
in both demyelinating and axonal forms of GBS patchy or segmental, the most affected regions
[33]. Rajabally et al., in 2016 reconfirmed the use- are: terminal segment distal to the wrist, common
fulness of “absent median present sural” and sites of entrapment neuropathy and proximal
“absent median normal sural” patterns with sensi- nerve segments including spinal roots. The rela-
tivities of 27.8% and 19.4% respectively, with tive deficiency of the blood-nerve barrier at the
specificity of 100% for AIDP vs axonal GBS, proximal nerve roots may make these more vul-
regardless of the criteria used to define GBS. Ulnar nerable for the immune attack in GBS. Distal
and radial patterns were not helpful [34]. demyelination affects both sensory and motor
When distal NCS are normal in a patient with nerves, with absent sensory responses and evi-
suspected GBS, electrophysiological studies at dence of reduced CMAP amplitude at distal and
the Erb’s point (site at the brachial plexus, located proximal stimulation sites. This may mimic acute
2–3 cm above the clavicle) have been shown to motor/+ sensory axonal neuropathy (AMAN/
be very helpful in the diagnosis of early GBS, as AMSAN). Proximal demyelination at nerve roots
it reflects the condition of proximal nerves. The is reflected as normal distal sensory and motor
NCS at Erb’s point may show prolonged latency, responses with abnormal F-wave studies. Erb’s
low motor amplitude or conduction block (>50% point or axillary stimulation is helpful in these
amplitude drop). This may be the only abnormal cases to look for evidence of additional demyelin-
parameter in early GBS and is relatively easy to ation at proximal sites. Electrodiagnostic studies
study [35]. Technical difficulties are encountered involving proximal extremity nerves, facial nerve
in achieving a supramaximal stimulus due to the studies and blink responses provide additional
deeper location of the brachial plexus and evidence of demyelination. These studies may be
particularly useful in the first 2 weeks of the ill- trophysiological correlate is limited to wide-
ness when the nerve conduction study of the distal spread severely reduced CMAP amplitudes.
nerves may fail to demonstrate demyelination. When sensory responses are also affected, it is
Sequential electrodiagnostic abnormalities in defined as acute motor and sensory axonal neu-
patients with AIDP were described by Albers ropathy (AMSAN). More recently, some patients
et al., in 1985. Abnormal median sensory with AMAN and AMSAN have been shown to
response with relative sparing of sural response demonstrate a transient conduction block/slow-
(“sural sparing”) was the earliest finding observed ing due to nodal dysfunction mimicking demye-
during the first 2 weeks. The earliest abnormality lination. Recovery is quick and without evidence
seen on needle EMG was decreased recruitment of remyelination (abnormal temporal disper-
of motor unit action potentials (MUAPs), with no sion). This has been referred to as reversible con-
change in configuration or evidence of abnormal duction failure (RCF) [38]. It is thought to be
spontaneous activity. Early presence of relatively due to antiganglioside antibodies attacking the
large amplitude MUAPs may be seen due to nodes of Ranvier by a complement mediated
selective loss of small MUAPs. However, this immune attack. This causes a transient dysfunc-
should not be considered as evidence of chronic- tion of the Na+/K+ channels at the nodes and
ity. Abnormal spontaneous activity occurs may not progress to axonal degeneration. The
between the second and the fourth week of ill- conduction failure improves quickly within a
ness, in both proximal and distal muscles. few weeks without the otherwise typical evi-
Myokymic discharges can be seen transiently dence of remyelination, so that there is no tem-
during the first 3 weeks but not subsequently in poral dispersion demonstrated. RCF can be
most cases. Among early abnormalities seen oth- recognized only by serial nerve conduction stud-
erwise is increased percentage of polyphasic ies. It is not included in the current electrodiag-
MUAPs at about the fourth week in both proxi- nostic criteria of GBS. A new criterion for GBS
mal and distal muscles [36]. Prominent fibrilla- has been proposed by Rajabally et al. 2015 to
tion potentials in the paraspinal muscles in the incorporate reversible conduction failure by a
first 2–3 weeks should raise the possibility of single NCS study [39].
acute porphyric polyneuropathy that often mim-
ics AMAN/AMSAN. lectrodiagnostic Findings in Other
E
Clinical recovery in GBS precedes that of Variants of GBS
NCS abnormalities. Restoration of demyelinat- Pharyngeal–cervical-brachial variant of GBS is
ing conduction block begins with remyelination a subtype of axonal GBS. Miller Fisher syn-
resulting in temporal dispersion. drome shows axonal neuropathy with decreased
CMAPs and SNAPs. Demyelinating forms are
Electrodiagnosis of AMAN/AMSAN less reported. RCF has been reported in patients
Three patterns of nerve conduction abnormalities with MFS and MFS/GBS overlap syndrome
are described in axonal GBS [37]: [40]. Facial motor and blink responses may be
abnormal. Loss of the H-reflex may be the ear-
1. Simple axonal degeneration (at least ~50%) liest finding due to involvement of Ia afferent
2. Transient conduction block/slowing in the fibers. EMG may show fibrillation potentials in
motor nerve terminals (early reversible con- the facial muscles in addition to the limb
duction failure) (~20%) muscles.
3. Absent F-waves as an isolated conduction Pure sensory GBS shows abnormal SNAPs
abnormality (~12%) with absent or reduced amplitude and normal or
slightly slow sensory conduction velocity. Motor
Acute motor axonal neuropathy (AMAN) conduction studies are normal. Low SNAPs are
occurs due to axonal degeneration and the elec- generally found to be reversible.
lectrodiagnostic Studies in Chronic

E Conduction slowing is suggestive of
Inflammatory Demyelinating demyelinating polyneuropathy
Polyradiculoneuropathy (CIDP) A nerve is composed of slow and fast conducting
As we discussed earlier, demyelinating polyneu- fibers. Distal latency and conduction velocity are
ropathy is diagnosed by evidence of conduction essentially the same measurement, they differ
slowing, and in the case of acquired etiologies: only by a multiplication factor (i.e. distance) and
conduction block with or without temporal dis- represent the conduction of the fastest conducting
persion. It is important to determine whether fibers in a nerve. Demyelination affecting these
chronic (>8 weeks) polyneuropathy has features fastest conducting fibers results in slowing of con-
consistent with chronic inflammatory demyelin- duction. Slowing of conduction velocity may also
ating polyradiculoneuropathy (CIDP) as this is a be evident in patients with axonal polyneuropathy
potentially treatable polyneuropathy. The diag- if the fastest conducting fibers are affected.
nosis can be very challenging in a patient with However, the conduction velocity in such situa-
underlying long-standing severe axonal diabetic tions typically does not fall into the demyelinating
polyneuropathy with markedly reduced/absent range (70% of lower limit of normal). This is
lower extremity sensory and motor responses. because normal myelinated fibers do not conduct
slower than this. Hence greater slowing of con-
CMAP amplitude in primary demyelinating duction on NCS suggests a primary demyelinat-
polyneuropathy ing process. Only in extremely rare cases of
CMAP amplitudes are typically normal or slightly regenerating nerve fibers, after a complete axonal
reduced in primary demyelinating polyneuropa- injury (e.g. nerve transection) can conduction
thies. However, if markedly reduced, it is difficult velocities be reduced in the demyelinating range.
to determine whether this is due to axon loss or When conduction velocity is reduced, other fea-
demyelination. Decrease in CMAP amplitude is tures of NCS are taken into consideration, such as
due to either (1) axon loss (2) conduction block (3) CMAP amplitude, conduction block, and tempo-
abnormal temporal dispersion. A linear correlation ral dispersion to help the electromyographer
was found between the decrease in amplitude and determine with certainty whether the underlying
slowing of conduction in the majority of motor as process is primary demyelinating or axonal.
well as sensory nerves, which was steeper in the An absence or prolongation of mean latency
axonal than in demyelinating polyneuropathies of the F-wave is among the most common fea-
[41, 42]. However, a more frequent and a larger tures in acute and chronic demyelinating neurop-
decrease in mean amplitude was found in sensory athies. It reflects conduction slowing due to
and motor nerves in demyelinating neuropathies, demyelination of the proximal nerve segment.
contrary to the belief that axon loss results in
greater decrease in CMAP and SNAP amplitudes. Temporal dispersion
Thus, a pathophysiological basis of electrodiagno- Temporal dispersion (Fig. 6.1) results from an
sis may be missed by just looking at the CMAP and abnormally increased range of conduction veloci-
SNAP amplitudes. Tankisi et al., in 2007 demon- ties amongst individual nerve fibers, leading to
strated that a more pronounced decrease in SNAP decreased amplitude with increased duration of
or CMAP amplitude in demyelinating neuropa- CMAP or SNAP, especially with longer recording
thies was due to abnormal temporal dispersion or distances [43]. Caliandro et al., in 2007 found that
distal conduction block. Part of the explanation for CMAP amplitude and temporal dispersion are
more pronounced decrease in CMAPs in demye- more sensitive electrophysiologic parameters
linating neuropathies is also because motor fibers than conduction velocity to determine moderate
are generally more affected in demyelinating neu- demyelination. Conduction velocity is reduced
ropathies while sensory fibers are generally more only when myelin damage is severe as it decreases
affected in axonal neuropathies [42]. when both large and small axons are affected [44].
Fig. 6.1 Temporal dispersion in the right median motor nerve conduction study in a patient with CIDP. It is more pro-
nounced at the proximal recording site (elbow), compared to the distal site at the wrist
Conduction block >20% in proximal CMAP area/amplitude defines

Conduction block (see Fig. 6.2) and temporal dis- conduction block and increase in CMAP duration
persion are electrophysiologic hallmarks of >15% signifies abnormal temporal dispersion. The
acquired demyelination. A sufficient number of effects of temporal dispersion are increased for
motor axons have to be blocked to result in a more proximal stimulation sites such as Erb’s
CMAP drop which can be caused by either con- point or axilla. Hence, the cut off values for motor
duction block or abnormal temporal dispersion. conduction block are doubled to >40% drop in
Conduction block along the motor or sensory proximal CMAP area or amplitude in the absence
nerve results in interruption of transmission of of temporal dispersion (<30% increase in CMAP
impulse across the nerve, resulting in a reduced duration). However, a drop in proximal CMAP
CMAP/SNAP amplitude and area. Verifying that a amplitude/area of <20% over a very short segment
drop in CMAP amplitude/area between the distal should not be disregarded as it may imply an
and proximal stimulation site is due to a true con- underlying conduction block. Currently the crite-
duction block can be very challenging. The CMAP ria of more than 50% drop in CMAP area or
is the summation of nearly synchronous muscle amplitude between proximal and distal stimula-
fiber action potentials recorded from a muscle. tion sites best defines conduction block, regardless
Demyelination amplifies the effect of normal of the amount of temporal dispersion [45]. CMAP
phase cancellation causing a further drop in ampli- area rather than CMAP amplitude is the preferred
tude/area of the proximal CMAP. Therefore, diag- measure for evaluating conduction block.
nostic criteria are necessary to distinguish between Errors in recording a conduction block may be
reduction in CMAP amplitude due to conduction due to the following physiological or technical
block versus abnormal temporal dispersion. factors:
From studies in normal subjects, CMAP ampli-
tude and area normally does not decrease by more 1. Temperature
than 20% and CMAP duration increase by more 2. Stimulus site
than 15%, when recorded from the typical distal 3. Supramaximal stimulus
and proximal stimulation sites (e.g. wrist to elbow, 4. Movement of stimulating electrode
ankle to knee). These studies imply that a drop of 5. Martin–Gruber anastomosis
Fig. 6.2 Left ulnar motor study shows a decrease in the below elbow. There is no temporal dispersion, hence this
CMAP amplitude >50% at the proximal recording site at drop in CMAP amplitude is due to a true conduction block
above elbow, compared to the distal recording site at across the elbow
The limb that is being studied for NCS should median nerve at the wrist and at proximal stimu-
be appropriately warmed if necessary, to achieve lation sites where multiple nerves are in the
a limb temperature ideally >37 °C (hands should vicinity of each other. A Martin–Gruber anasto-
be >33 °C and feet >30 °C as per European mosis between the proximal median or anterior
Federation of Neurological Societies/Peripheral interroseous nerve and ulnar nerve may occa-
Nerve Society (EFNS/PNS) Joint Task Force, sionally produce a “pseudo” conduction block
2010 [46]. Conduction block may be missed in between the proximal and distal ulnar stimulat-
cold temperatures. Lower stimulus intensity at ing sites. This has to be differentiated from a true
deeper sites such as Erb’s point, and popliteal conduction block by appropriate “crossover”
fossa may erroneously result in reduced CMAPs. studies.
If supramaximal stimulus is not applied to obtain
a maximum CMAP response by increasing the Sensory studies
stimulus intensity by further 20% after a maxi- Sensory nerve action potentials (SNAPs) have
mum CMAP response is obtained, then a reduced low amplitude and short duration. Hence, the
CMAP response may be seen. This may be spu- effect of normal temporal dispersion is ampli-
riously interpreted as a true conduction block. fied in sensory nerves. Demyelination causes
Care must be taken to avoid recording a volume further increase in temporal dispersion and con-
conducted CMAP from the neighbouring motor duction slowing/block. This makes the use of
nerves. This is important while recording the sensory NCS less reliable in assessing demye-
lination in peripheral neuropathies. However, Distal demyelination

significant slowing on sensory NCS is sugges- CMAP duration is defined as the distance from
tive of demyelination. Absence of responses in the onset of the first negative deflection to return
the commonly studied distal sensory nerves in to baseline of the last negative deflection. The
both lower and upper extremities is seen in terminal positive deflection is not included [49].
severe axonal as well as demyelinating neuropa- Methods that do not include the later negative
thy and makes the distinction between them components of the CMAP may fail to accurately
unreliable based only on sensory studies. The measure the duration of the desynchronised
Ad Hoc Subcommittee of the American CMAP. Prolongation of distal CMAP duration
Academy of Neurology AIDS Task Force (1991) or dispersion of distal CMAP duration was pro-
Criteria recognized sensory nerve conduction posed as a new electrodiagnostic criteria for
velocity <80% LLN as being supportive of diagnosis of CIDP by the EFNS/PNS task force
CIDP [47]. Acute and chronic acquired inflam- in 2010 to recognize distal demyelination. A
matory demyelinating neuropathies have seg- cutoff value of 9 ms duration of distal CMAP
mental demyelination with “sural sparing” that duration was proposed in the earlier criteria and
is classically described in demyelinating poly- later the limits were specified for different
neuropathies. Thus, the sensory nerve conduc- nerves as: median ≥6.6 ms, ulnar ≥6.7 ms,
tion studies play an important role in peroneal (fibular) ≥7.6 ms, and tibial ≥8.8 ms
combination with motor nerve conduction stud- [48]. While distal motor latency reflects conduc-
ies in the diagnosis of CIDP (Table 6.9). tion along the fastest conducting motor fibers,
distal CMAP duration depends on the temporal
Supportive criteria dispersion between the slow and fast motor
fibers distally within the nerve. It is a useful
(A) Elevated CSF protein with cell counts <10/ marker of distal demyelination.
mm3 Needle EMG in CIDP shows changes of sec-
(B) Magnetic resonance imaging showing gado- ondary axonal degeneration in the proximal and
linium enhancement and/or hypertrophy of the distal muscles of the upper and lower extremity.
cauda equina, lumbosacral or cervical nerve Evidence of chronic motor axon loss is seen in
roots, or the brachial or lumbosacral plexuses these muscles in the form of long duration, large
(C) Nerve biopsy showing unequivocal evidence amplitude, polyphasic motor unit action poten-
of demyelination and/or remyelination in >5 tials (MUAPs), often with active/ongoing dener-
fibers by electron microscopy or in >6 of 50 vation features comprising fibrillation and/or
teased fibers positive sharp wave potentials.
(D) Clinical improvement following immuno-
modulatory treatment CIDP Variants
Multifocal motor neuropathy with conduction
block (MMNCB or MMN)
Diagnostic categories Conduction block at non-entrapment sites along
• Definite CIDP: Clinical criteria 1 A or B and the motor nerve is the electrophysiological hall-
2 and EDX criteria 1; or Probable (electro- mark of MMN. Sensory nerves are typically not
physiology) CIDP + at least 1 Supportive cri- affected. Motor conduction block is not specific
terion or Possible (electrophysiology) CIDP + for MMN. It may be seen in other acquired
at least 2 Supportive criteria demyelinating and entrapment neuropathies.
• Probable CIDP: Clinical criteria 1 A or B and Other features of acquired demyelination such as
2 with EDX criteria 2; or Possible (electro- temporal dispersion with prolongation of distal
physiology) CIDP + at least 1 Supportive latency, slowing of conduction velocity and pro-
criterion longation of F-wave latency may be present.
• Possible CIDP: Clinical criteria 1 A or B and CMAP area/amplitude drop (between proximal
2 with EDX criteria 3 and distal stimulation sites) of >50% defines
Table 6.9 Clinical and electrodiagnostic criteria for CIDP [48]

Clinical
1. Inclusion criteria
(a) Typical CIDP
Chronically progressive, stepwise, or recurrent symmetric proximal and distal weakness and sensory
dysfunction of all extremities, developing over at least 2 months; cranial nerves may be affected; and
Absent or reduced tendon reflexes in all extremities
(b) Atypical CIDP (still considered CIDP but with different features)
One of the following, but otherwise as in (a) (tendon reflexes may be normal in unaffected limbs):
Predominantly distal (distal acquired demyelinating symmetric, DADS) or
Asymmetric [multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), Lewis–Sumner
syndrome] or
Focal (e.g., involvement of the brachial or lumbosacral plexus or of one or more peripheral nerves in one
upper or lower limb)
Pure motor or
Pure sensory (including chronic immune sensory polyradiculopathy affecting the central process of the
primary sensory neuron)
(2) Exclusion criteria
Borrelia burgdorferi infection (Lyme disease), diphtheria, drug or toxin exposure probably to have caused the
neuropathy
Hereditary demyelinating neuropathy
Prominent sphincter disturbance
Diagnosis of multifocal motor neuropathy
IgM monoclonal gammopathy with high titre antibodies to myelin-associated glycoprotein
Other causes for a demyelinating neuropathy including POEMS syndrome, osteosclerotic myeloma, diabetic
and non-diabetic lumbosacral radiculoplexus neuropathy. PNS lymphoma and amyloidosis may occasionally
have demyelinating features
Electrodiagnostic
1. Definite
At least one of the following:
(a) Motor distal latency prolongation ≥50% above ULN in two nerves (excluding median neuropathy at the wrist
from carpal tunnel syndrome), or
(b) Reduction of motor conduction velocity ≥30% below LLN in two nerves, or
(c) Prolongation of F-wave latency ≥30% above ULN in two nerves (≥50% if amplitude of distal negative peak
CMAP <80% of LLN values), or
(d) Absence of F-waves in two nerves if these nerves have distal negative peak CMAP amplitudes ≥20% of LLN
+ ≥1 other demyelinating parameter in ≥1 other nerve, or
(e) Partial motor conduction block: ≥50% amplitude reduction of the proximal negative peak CMAP relative to
distal, if distal negative peak CMAP ≥ 20% of LLN, in two nerves, or in one nerve + ≥1 other demyelinating
parameter in ≥1 other nerve, or
(f) Abnormal temporal dispersion (>30% duration increase between the proximal and distal negative peak
CMAP) in ≥2 nerves, or
(g) Distal CMAP duration (interval between onset of the first negative peak and return to baseline of the last
negative peak) increase in ≥1 nerve (median ≥6.6 ms, ulnar ≥6.7 ms, peroneal (fibular) ≥7.6 ms, tibial
≥8.8 ms) + ≥1 other demyelinating parameter in ≥1 other nerve.
2. Probable
• ≥30% amplitude reduction of the proximal negative peak CMAP relative to distal, excluding the posterior tibial
nerve, if distal negative peak CMAP ≥20% of LLN, in two nerves, or in one nerve + ≥1 other demyelinating
parameter in ≥1 other nerve.
3. Possible
• As in “definite” but in only one nerve.
Motor conduction block is not considered in the ulnar nerve across the elbow and at least 50% amplitude reduction
between Erb’s point and the wrist is required for probable conduction block. Temperatures should be maintained to at
least 33°C at the palm and 30°C at the external malleolus
CMAP compound muscle action potential, ULN upper limit of normal values, LLN lower limit of normal values
conduction block, regardless of the presence of point due to the large amount of subcutaneous
temporal dispersion. CMAP area is the preferred tissue. In the lower extremity, caution is war-
measure for evaluating conduction block than ranted with stimulation of the tibial nerve at the
CMAP amplitude. An abrupt drop in CMAP popliteal fossa as the stimulus may not be supra-
over a short segment also signifies a conduction maximal to the deeper location of the nerve at
block. When the distal CMAP amplitude is this site, erroneously resulting in a reduction in
<1 mV, it is not useful to define the presence of CMAP amplitude. It is emphasized that the elec-
focal conduction block in MMN. There are two tromyographer should thoroughly search for the
reasons for this— presence of a motor conduction block as MMN is
a potentially treatable neuropathy that mimics
1. The effects of normal temporal dispersion are Amyotrophic Lateral Sclerosis (ALS).
magnified with very few fibres, giving a false Conduction block is not an absolute requirement
positive proximal CMAP drop, thus errone- for diagnosis if other features of demyelination
ously giving the impression of a conduction are present. In a series of MMN patients, only
block. 31% had conduction block; 44% had temporal
2. IVIG has not been shown to be useful in nerves dispersion, and 94% had other electrodiagnostic
with distal CMAP <1 mV, probably because features of demyelination with superimposed
such low CMAPs represent marked secondary axonal degeneration [50]. Response to treatment
axon loss (late stage of MMN) [45]. with IV immunoglobulin was no different with/
without the conduction block. Presence of anti-
Conduction block is most frequently present ganglioside antibodies in high titers is rather spe-
in the upper extremity nerves at non-entrapment cific for MMN but not an absolute requirement
sites. It may be missed on routine NCS if present for diagnosis of MMN (anti-GM1 antibodies are
in proximal nerve segments (plexus, or root) or absent in approximately 50% of MMN patients).
when associated with significant secondary axo- By definition, MMN affects more than one
nal loss. When conduction block is more proxi- motor nerve, clinically and electrophysiologi-
mal, both the routine proximal and distal CMAP cally. However rarely conduction block may be
will be normal. When a conduction block is distal seen in only one motor nerve, with a very good
to the distal stimulation site along the motor response to immunotherapy. An axonal pheno-
nerve, both the proximal and distal CMAPs are type of MMN, multifocal axonal motor neuropa-
reduced in amplitude and this mimics an axon thy (MAMN) has been described in a small series
loss motor neuropathy or motor neuron disease. of patients, lacking overt conduction block, ±
Identification of conduction block along very elevated titers of anti-GM1 antibodies, but
proximal or distal segments can be challenging responded very well to IV immunoglobulin ther-
for the electromyographer. Prolongation of apy just like MMN [51]. MMN should be differ-
F-wave latency favors demyelination in the entiated from other asymmetric neuropathies: e.g.
absence of an easily identifiable conduction mononeuritis multiplex and MADSAM.
block or other indicators of demyelination. In
exceptional cases, only proximal conduction Multifocal acquired demyelinating sensory
block may be present, hence more proximal seg- and motor neuropathy (MADSAM)
ments of motor nerve should be studied (e.g. NCS show features consistent with acquired
stimulating axilla, Erb’s point or cervical root). demyelination as in CIDP. However motor and
Needle electrode examination shows abnormal sensory nerves are affected in an asymmetric
spontaneous activity with presence of fibrillation manner. These include prolongation of F-wave
potentials ± fasciculation potentials, in addition latency (>130% ULN), distal latency (>150%
to decreased motor unit recruitment manifested ULN), slowing of nerve conduction velocity
by large motor unit potentials. (<70% LLN), conduction block, temporal disper-
Technical difficulties may arise due to inabil- sion and increase in distal CMAP duration.
ity to use a supramaximal stimulus at the Erb’s Mononeuritis multiplex and MMN remain
important differentials. Mononeuritis multiplex ing neuropathies. Lack of temporal dispersion

is an axon loss polyneuropathy affecting multiple and conduction block with very slow (and uni-
nerves in an asymmetric fashion. These features form) conduction velocities in the range of
also overlap with HNPP which should be consid- 20–30 m/s on motor NCS is suggestive of an
ered especially in patients who are young, have a inherited demyelinating neuropathy.
history of recurrent multifocal entrapment neu- CMT-IA (caused by PMP22 duplication) is
ropathies and a positive family history. characterized by widespread, uniform conduc-
tion slowing, which remains stable over
Distal acquired demyelinating symmetric decades; conduction block is unusual, and when
neuropathy (DADS) found, probably reflects superimposed nerve
NCS shows widespread slowing in distal sensory entrapment. A lack of profound conduction
and motor nerves of the lower extremity. Distal slowing does not exclude a diagnosis of
latencies are markedly prolonged, resulting in CMT-IA. Neurologic disability correlates more
short terminal latency index (TLI) which is the closely with reduced motor amplitudes (a
electrodiagnostic hallmark of DADs neuropathy. marker of axonal loss) than with slowed con-
Short TLI can reliably distinguish DADs with duction velocities. Substantial phenotypic and
anti MAG antibodies (anti-MAG neuropathy) electrophysiologic variability can occur within
from CIDP and other chronic demyelinating neu- families. There is marked slowing of conduc-
ropathies [52]. Conduction block is uncommon. tion velocity, usually below 75% of the lower
limit of normal. Since slowing is uniform in all
ther Demyelinating Polyneuropathies
O nerves, conduction block or temporal disper-
Monoclonal gammopathy with undetermined sion is not seen. Slowing can also be demon-
significance (MGUS) strated in the upper extremities, which may or
If a patient with CIDP-like pattern on EDX does may not be weak clinically. Velocities as low as
not respond to standard line of care, the treating 20–25 m/s are seen in patients with
provider should reconsider the diagnosis, work CMT1A. CMT-1B may have even slower veloc-
up for an alternative cause such as paraprotein- ities, 15 m/s or less [10].
emic neuropathy. Two patterns of demyelinating CMT-X, an X-linked neuropathy is caused by a
neuropathies are seen in patients with mutation of the gene for connexin 32 protein and
MGUS. DADs neuropathy is an IgM MGUS is characterized by nonuniform conduction slow-
associated neuropathy with (or without) anti- ing. In CMT-X, conduction slowing may be “inter-
MAG antibodies with distal weakness, prominent mediate” between CMT-1 and CMT-2 (axonal
hand tremor and predominant distal demyelin- form) with velocities of 30–40 ms. Furthermore,
ation manifested by prolonged distal latencies. in CMT-X there can be multifocal, segmental
CIDP-MGUS (non-IgM) essentially has the same demyelination with temporal dispersion and con-
clinical and electrophysiological characteristics duction block that simulates CIDP [10]. Females
as CIDP, with patients typically having an IgA or with the mutation may have modest conduction
IgG M protein. Patients with MGUS have a risk slowing, and many such patients have been mis-
of lymphoproliferative malignancies and hence classified as having CMT- 2. CMT-X always
should be followed up regularly. Neuropathy should be considered in any inherited neuropathy
associated with lymphoma and osteosclerotic without male-to-male transmission.
myeloma, as opposed to multiple myeloma may Conduction velocity parameters in the fore-
also present like CIDP. It responds to treatment arm have been used to classify inherited neuropa-
of the underlying malignancy. thies to help select the genetic test for inherited
neuropathies. Sensory studies are typically
Inherited Polyneuropathies abnormal with low or absent amplitudes. Some
Neurophysiologic studies are very helpful in dis- secondary axonal loss is expected causing reduc-
tinguishing inherited from acquired demyelinat- tion in the CMAPs and concurrent disability.
Fig. 6.3 Inherited neuropathy vs CIDP ([10]—used with by the different pathological findings in the two. Nerve
permission). Nerve conduction study in inherited neurop- biopsy in HMSN1A reveals uniform myelin lamella col-
athy (hereditary motor and sensory neuropathy-HMSN1A) lagen thickening (onion bulbs) with PMP22 duplications;
shows uniform demyelinating slowing, without conduc- while in CIDP there are onion bulbs seen; the latter pro-
tion block or temporal dispersion as seen below in chronic duces variable conduction velocities between fibers with
acquired demyelinating polyradiculoneuropathy (CIDP). observed dispersion and potential conduction blocks
These electrophysiological differences can be explained
Nerve conduction studies are helpful in early conduction velocity [meters/second]) less than
diagnosis and prognostication in patients with 0.26 is seen in IgM MAG-associated DADS neu-
CMT. ropathy whereas hereditary demyelinating neu-
Demyelinating neuropathy in the absence of ropathies generally have a TLI >0.26.
dispersion and conduction block favors an inher- Small fiber and autonomic testing is helpful in
ited etiology (particularly CMT-1) over acquired patients with suspected hereditary sensory and
etiology (e.g. CIDP), as illustrated in Fig. 6.3. autonomic neuropathies.
Unlike CIDP, DADS neuropathy associated Hereditary neuropathy with liability to pressure
with IgM MGUS is difficult to differentiate from palsies (HNPP) is characterized by segmental slow-
inherited neuropathy due to their common clinical ing at common points of compression and by pro-
features, prominent distal weakness, and NCS longed distal motor latencies. However, conduction
showing uniform slowing with absence of tempo- block is found only in a minority of patients.
ral dispersion and conduction block. Also, the
prevalence of IgM monoclonal protein in the gen-
eral population increases with age >50 years and Electrodiagnostic Examination
anti-MAG antibody, hence, it may be difficult to Techniques and Protocol
determine if an abnormal M protein is the cause of for Evaluation of Polyneuropathy
the neuropathy. NCS may allow this distinction as
DADS neuropathy shows preferential distal slow- The goal of electrodiagnostic studies is to con-
ing in conduction velocities, while conduction firm the presence of a polyneuropathy, assess the
velocities are equally slow proximally and distally type of nerve fibers involved (sensory and/or
in inherited neuropathies. Specifically, the TLI motor), pattern, chronicity (and possibly tempo),
= (1/distal motor latency [milliseconds] × (distal pathophysiology (primarily axonal vs demyelin-
conduction distance [millimeters]/distal motor ating) and its severity.
Sensory NCS or secondary axon loss. This may be done by

Antidromic versus orthodromic stimulation may be semi-quantitative (qualitative) or quantitative
employed when performing sensory NCS. methods. Semi-quantitative technique includes
Antidromic stimulation (stimulating proximally, assessment of insertional activity, spontaneous
recording distally) generally produces a higher activity and recruitment pattern of motor units,
SNAP amplitude (due to the proximity to the record- while quantitative techniques use motor unit
ing electrodes), and tends to be less painful. However, potential analysis, including turn–amplitude
orthodromic stimulation (stimulating distally, analysis.
recording proximally) generally produces a lower Factors influencing NCS in the electrophysi-
SNAP amplitude, and tends to be more painful. ology lab need to be considered prior to assessing
H-reflex response is obtained when a low sub- the results of the NCS. These are:
maximal stimulus of long duration is applied to a
motor nerve. This selectively activates the Ia 1. Age:
fibers. The tibial H-reflex response is an electri- Sensory nerve conduction velocities (NCVs)
cal correlate of the S1-mediated deep tendon are slower at birth and reach adult values by
reflex (ankle jerk). age 5 years. This is explained by incomplete
myelination of sensory nerves that is com-
Motor NCS pleted by 5 years of age. Sensory NCVs start
Motor NCS are critical in the assessment of poly- declining at the age of 20 years, by 1 m/s until
neuropathy. Similar to the sensory NCS, the dif- 55 years. Thereafter the decline occurs by
ferences in the techniques of motor NCS are due 3 m/s per decade.
to the use of surface vs needle stimulation elec- 2. Height:
trodes and use of different recording electrodes. Increased height is associated with lower
The different recording electrodes affect the SNAP amplitudes, prolonged distal latency
compound muscle action potential (CMAP) and slower conduction velocity. This is partly
amplitude. Surface recording using the “belly- attributed to cooler distal limb temperatures
tendon” method is preferred, where the active and smaller axonal diameter of distal nerves
electrode is over the muscle belly and the refer- in taller individuals.
ence electrode is over the tendon insertion point. 3. Temperature:
F-wave response, a late motor response that is Cooler temperatures slow down nerve con-
recorded after a CMAP with supramaximal stim- duction and prolong the distal latencies,
ulus to a motor nerve assesses the proximal nerve while increasing the sensory and motor
segments. It travels through the entire length of amplitudes. This is due to longer duration of
the nerve including the distal segment. opening of sodium channels. To reduce the
Axon (A) reflex is an intermediate or late confounding effect of temperature, the limb
response seen during routine F-wave recording. should be appropriately warmed (hands
This is a small motor response identical in latency should be at least >32 °C and feet >30 °C), if
and configuration that occurs between the M- and necessary.
the F-responses. It occurs from ephaptic spread
from one nerve fiber to another and proximal re-
excitation at a point of inflammation and demye- Nerve Conduction Study
lination. Axon reflexes are typically seen in Before starting nerve conduction study, the
reinnervated nerves, especially when a submaxi- limb to be tested is appropriately warmed to
mal stimulus is given. ensure the desired limb temperature as afore-
mentioned. Sensory NCS, motor NCS, F-wave
Needle electrode examination (NEE) or response and H-reflex responses are typically
electromyography (EMG) performed on one side in the lower and upper
NEE/EMG evaluates the extent of denervation extremity. This is followed by needle electrode
and reinnervation in the muscles due to primary examination. Most commonly, surface elec-
trodes are used to record the sensory and motor Radial sensory response is spared in local entrap-
nerve action potentials. ment neuropathies such as median neuropathy at
Nerve conduction studies are generally per- the wrist (carpal tunnel syndrome) or ulnar neurop-
formed on the most symptomatic extremity. Sensory athy at the elbow. Hence a reduced radial sensory
nerve conduction studies in the lower extremity: response, with a reduced median and ulnar sensory
sural and superficial sensory nerve studies are usu- response is likely due to an underlying axonal poly-
ally performed first. Then peroneal (fibular) motor neuropathy, with reduced or absent sural and super-
response is recorded at the extensor digitorum bre- ficial peroneal (fibular) sensory responses. Reduced
vis muscle (EDB). If reduced, then proximal studies sensory responses in the upper extremity with an
are recorded at the tibialis anterior muscle. The tib- intact sural sensory response in the lower extremity
ial motor response is recorded distally at the abduc- suggests a demyelinating polyneuropathy. Further
tor hallucis brevis (AH) muscle along with the studies are then directed to find more evidence of a
F-wave response. In most polyneuropathies and demyelinating polyneuropathy. If motor amplitudes
radiculopathies (C8-T1 in the upper limb, and are low, test for Lambert Eaton myasthenic syn-
L5-S1 in the lower limb), F-responses are prolonged drome by exercising muscles for 10 s, followed by a
or even absent. F-response prolongation or absence single stimulation post exercise.
may be the earliest and the only finding in GBS, as If a demyelinating neuropathy (GBS, CIDP) is
demyelination occurs at the nerve roots. Finally, the suspected, additional studies are performed
tibial/soleus H-reflex is performed. Prolonged involving the motor nerves of the upper extremity
H-reflex latency is seen in S1 radiculopathy, lumbo- in addition to the routine motor and sensory
sacral plexopathy, sciatic or tibial neuropathy or nerves in the lower and upper extremity, along
axonal polyneuropathy. It is useful in the assess- with the F-wave response and H-reflex response
ment of very early polyneuropathy. as discussed above. The ulnar motor nerve is
For any abnormal nerve conductions, an identi- stimulated at five points instead of routine three-
cal study is performed on the opposite side. point stimulation; namely at wrist, below elbow,
Thereafter, routine distal motor and sensory studies above elbow, axilla and Erb’s point. A more
are performed in the upper extremity. In a clinically extensive search is carried out to find evidence of
suspected length dependent axonal polyneuropathy demyelinating neuropathy, especially so in cases
(pain or tingling or weakness in bilateral toes or feet where all routine distal sensory and motor
or gait abnormality), upper extremity nerve conduc- responses in the lower and upper extremity are
tion studies may not necessarily be performed if all absent. In such cases it may be worthwhile to
the lower extremity nerve conduction studies are study additional proximal nerves of the upper
normal (absent sural sensory nerve action potential extremity (musculocutaneous, axillary nerves) or
above the age of 75 years or absent H-reflex above facial motor nerves and blink responses.
60 years is considered normal for age), and demye-
linating or a pure sensory neuropathy or sensory Electromyography
ganglionopathy is not suspected. The routine nerve The EMG approach is similar to the NCS approach
conduction studies performed in the upper extrem- in the assessment of axonal polyneuropathy.
ity for clinically suspected distal symmetric length Needle EMG is performed first in the muscles of
dependent axonal polyneuropathy are median the lower extremity. Muscles on the contralateral
motor nerve conduction study recorded at the side are sampled to check for symmetry or
abductor pollicis brevis muscle and ulnar motor confirming a focal abnormality on the tested side.
nerve conduction study recorded at the abductor An axonal polyneuropathy is typically length-
digiti minimi muscle, along with the ulnar motor dependent and symmetric. So, the most distal
F-wave response. Sensory nerves studied are muscles, extensor digitorum brevis (EDB), abduc-
median sensory at digit II, ulnar sensory at digit V tor hallucis (AH) and abductor digiti quinti pedis
and superficial radial sensory nerve at the snuff box. (ADQP) muscles will be affected early. Needling
these muscles is often very painful and abnormal- spinal muscles). Contralateral muscles in each limb
ities seen in EMG in these intrinsic foot muscles is compared to assess for symmetry.
may be otherwise seen in normal subjects as well In mild polyneuropathies, or early polyneu-
even without an underlying polyneuropathy due ropathies with normal NCS, the only abnormali-
to repetitive trauma from walking or running and ties may be found in the intrinsic foot
use of foot wear. Hence if intrinsic foot muscles muscles. Loss of only a few axons may result in
are needled in the evaluation of polyneuropathy, fibrillation potentials that are easily seen in EMG,
the chronic motor axon loss changes seen should but this may cause little or no appreciable change
be interpreted with caution. Muscles routinely on routine distal motor and sensory nerve con-
studied are tibialis anterior, tibialis posterior, duction studies. Hence in the evaluation of poly-
medial gastrocnemius, vastus lateralis, and glu- neuropathy, needle EMG is very sensitive in
teus medius. If any abnormality is seen in one identifying early changes.
muscle, a root search is also performed by sam- EMG also helps in identifying other possible
pling more proximal muscles as these will not be differentials, superimposed radiculopathy, ante-
affected in a typical length-dependent, axonal rior horn cell disease or a myopathy.
polyneuropathy unless the polyneuropathy is
severe. Hence, a proximal L5 muscle, e.g. gluteus ecommended Nerve Conduction
R
medius and a proximal S1 muscle, e.g. biceps Study Protocol for Polyneuropathy
femoris short head or gluteus maximus may be Routine sensory studies
studied routinely if tibialis anterior (distal L5
muscle) and medial gastrocnemius muscle (distal 1. Sural, recording at the ankle
S1 muscle) shows evidence of active/ongoing or 2. Superficial peroneal (fibular), recording at the
chronic motor axon loss. Check for symmetry by ankle
sampling corresponding muscles on the contralat- 3. Median sensory, recording at digit II
eral side. 4. Ulnar sensory, recording at digit V
After sampling the lower extremity, if abnor- 5. Radial sensory, recording at the snuff box
malities are found in the proximal muscles of the
thigh (vastus lateralis, semitendinosus), distal mus- Routine motor studies
cles of the upper extremity (first dorsal interro-
seous, abductor digiti minimi, abductor pollicis 1. Peroneal (fibular), recording extensor digito-
brevis, extensor indicis proprius and flexor pollicis rum brevis and stimulating at the ankle, below
longus) are sampled to look for evidence of active fibular neck, and lateral popliteal fossa. If low
or chronic motor axon loss. If a demyelinating or absent responses, peroneal (fibular) motor
polyneuropathy is the question, then proximal study is performed recording the tibialis ante-
muscles in the upper extremity (e.g. deltoid, biceps, rior and stimulating at below fibular head and
triceps) and proximal muscles in the lower extrem- lateral popliteal fossa
ity (e.g. gluteus medius and maximus) are sampled 2. Tibial, recording abductor hallucis brevis and
including the cervical and lumbosacral paraspinal stimulating ankle and popliteal fossa
muscles. A major role of needle EMG in a demye- 3. Median, recording abductor pollicis brevis
linating neuropathy is to assess the amount of sec- and stimulating wrist and antecubital fossa
ondary axon loss and the severity of the axon loss. 4. Ulnar, recording abductor digiti minimi and
If proximal muscles are predominantly involved stimulating wrist, below elbow and above elbow
along with the distal muscles, this supports a non-
length dependent pattern of neuropathy. As dis- Late responses
cussed earlier, proximal predominance is seen in
demyelinating neuropathies (AIDP, CIDP) and 1. F-wave responses: tibial and ulnar
porphyria (particularly with involvement of para- 2. Tibial/Soleus H-reflexes
Additional studies: chronic inflammatory demyelinating polyradic-

If clinical suspicion of asymmetry (e.g. mono- uloneuropathy (CIDP). There is evidence of
neuritis multiplex), then the contralateral side is some motor involvement, limited to the distal
also studied. muscles as well in both the upper and lower
If a demyelinating polyneuropathy is sus- extremities. Typically, in CIDP, proximal mus-
pected, then the ulnar nerve is stimulated at five cles are weak too. Based on the history and
points: wrist, below and above elbow, axilla and physical examination, this is a chronic non-
Erb’s point. Additional proximal motor or facial length dependent sensorimotor polyneuropathy,
motor nerve with blink responses may be studied predominantly affecting the distal limbs. This
for more evidence of demyelination. may be a distal variant of CIDP.
Nerve conduction studies are performed on the
Case 1 left upper and lower extremities (see Table 6.10).
A 58-year-old Caucasian female was seen in the The sural sensory nerve amplitude is normal while
neuromuscular clinic for numbness in her hands that of the median and ulnar sensory nerve is
and feet that started 5 years ago. She first noticed reduced. The radial sensory amplitude is also
tingling in the tips of toes and fingers on either mildly reduced. This pattern is that of a reduced
side. After a year, she had tingling and numb- median and ulnar sensory amplitude with an intact
ness “everywhere”, including her head, face, sural sensory response (sural sparing). Motor stud-
trunk, arms and legs. Such symptoms have been ies in the lower extremity reveal >50% drop in
pretty constant over the past 4 years or so. She proximal compared to distal CMAP in the pero-
endorses generalized fatigue, and gait difficulty neal (fibular) nerve recorded at the extensor digito-
with some tendency to fall over the past 1.5 rum brevis, and the tibial nerve recorded at the
years (mostly has near-falls), no gait assistive abductor hallucis muscle, consistent with a con-
device use reported. She is equivocal about the duction block along the nerves. There is slowing
presence of significant muscle atrophy, but sus- of peroneal (fibular) motor conduction velocity
pects some in the shin area, and possibly the (<70% of LLN). Tibial F-wave latency is margin-
forearms bilaterally. She has a past medical his- ally delayed (20% above ULN), with absence of
tory of gastroesophageal reflux disease, irritable the tibial H-reflex response. Looking at the upper
bowel disease, depression with anxiety, and extremity motor studies, we find a conduction
hypothyroidism. Neurological examination was block (>50% proximal/distal CMAP drop) along
remarkable for focal atrophy in the intrinsic foot the left median nerve, with mild prolongation of its
muscles and distal leg muscles. Strength was distal latency, and concomitant slowing of motor
reduced moderately in the intrinsic muscles of conduction velocity. Median F-wave latency is
hand and toe dorsiflexors and plantar flexors, prolonged in the demyelinating range (>30%
however, in all other muscle groups it was intact. above ULN). The mildly increased median distal
Deep tendon reflexes were reduced to absent motor latency is potentially attributable to carpal
throughout. Pinprick sensation was decreased tunnel syndrome. The reduction in ulnar CMAP
around the ankles bilaterally, while other modal- seen with Erb’s point stimulation compared to that
ities of sensations were intact. at the axilla may suggest a true conduction block
SUMMARY: along the left ulnar motor nerve, with conduction
The patient’s clinical history is consistent slowing also manifested by a prolonged ulnar
with a polyneuropathy that started 5 years ago, F-wave latency (>30% above ULN). Hence, the
which is now stable. She has sensory symptoms NCS findings are consistent with a diagnosis of
which started distally in the fingers and toes at CIDP with features of demyelination in at least >2
around the same time. This suggests that we are motor nerves as discussed above. The sural spar-
dealing with a some what non-length dependent ing pattern is also consistent with CIDP.
polyneuropathy. A chronic non-length depen- The EMG (see Table 6.10) shows at least some
dent polyneuropathy, with reduced or absent chronic motor axon loss changes with large
deep tendon reflexes should make one think of amplitude, long duration, polyphasic MUAPs in
6

B-P Amp (μV) LatNPk (ms) CV (m/s) Dist (mm) Norm B-P Temp (ºC)
Polyneuropathies
Nerve Stimulus Recording L R L R L R L R Amp Norm LatNPk Norm CV L R

Median Wrist Index 9.56 3.88 n/a 130 >15 μV <3.6 ms >50 m/s 32.7
Ulnar Wrist 5th Dig 4.80 3.02 n/a 110 >10 μV <3.1 ms >50 m/s 33.5
Radial Thumb Forearm 13.46 2.00 n/a 100 >14 μV <2.7 ms >50 m/s 33.8
B-P Amp (mV) LatOn (ms) CV (m/s) Dist (mm) Temp (ºC)
Peroneal Extensor digitorum brevis (EDB) Ankle 3.21 3.95 n/a >2.5 mV <6 ms >40 m/s 30.7
(fibular) Pop Fossa–Knee 0.63 18.40 28.4 410 30.5
Below Fibular Head 0.60 15.15 29.5 330 30.1
Tibial Abductor hallucis (AH) Ankle 12.96 3.30 n/a >4 mV <6 ms >40 m/s 30.5
Pop Fossa–Knee 5.87 15.65 34.8 430 30.4
Peroneal Tibialis anterior (TA) Below Fib Head 4.85 1.95 n/a >3 mV <4.5 ms >40 m/s 31.2
(fibular) Pop Fossa–Knee 3.76 6.05 24.4 100 31.0
Median Abductor pollicis brevis (APB) Wrist 8.25 4.10 n/a 50 >6 mV <4 ms >50 m/s 33.9
Elbow 3.54 9.95 44.4 260 33.8
Ulnar/wrist NR NR n/a 33.6
Ulnar/elbow NR NR n/a 33.6
Ulnar Abductor digiti minimi (ADM) Wrist 6.59 2.65 n/a 50 >7 mV <3.1 ms >50 m/s 33.9
Below elbow 4.03 7.30 49.5 230 33.9
Above elbow 2.89 9.50 48.2 330 33.9
Axilla 2.21 13.55 48.6 530 33.9
Erb’s 0.30 17.50 49.2 730 33.6
Post exercise 6.73 2.55 n/a 33.8
(continued)
149
150
F-Waves
Lat (ms)
Tibial/AH Ankle AH 68.50
Median/APB Wrist APB 49.30
H-Reflex summary table
M-Wave H-Wave
Tibial Pop Fossa Soleus Left 5.9 ms 8.5 mV NR
Electromyography
Needle EMG summary
Side Muscle IInsertional Fibrillation Positive Fasciculation Other Number Recruitment Duration Duration Amplitude Amplitude Polyphasia Polyphasia Description
Activity Potentials Sharp Potentials
Wave
Potentials
L 1st Dorsal Norm 0 0 0 Norm Full Norm Norm Norm NC
Interosseous
APB Norm 0 0 0 1− Mod-V Norm Norm Norm NC
Flex.Pollicis Norm 0 0 0 1− Mod Norm Norm Few 1+ NC

Longus
Extensor Norm 0 0 0 Norm Full Norm Norm Norm NC
Indicis
Pronator Teres Norm 0 0 0 1− Mod Norm Norm Norm NC
Biceps Brachii Norm 0 0 0 2− Mod Norm Norm Norm NC
Triceps-Lateral Norm 0 0 0 1− Mod Norm Norm Norm NC
Head
Deltoid, Norm 0 0 0 1− Mod Norm Norm Norm NC
Middle Head
Cervical Norm 0 0 0 2− Mod Norm Norm Norm NC
paraspinal
(Low)
M. C. Dhamne and J. A. Morren
6
Abductor Norm 0 0 0 3− Mod-V Many 1+ Some 1+ Norm NC
Hallucis
EDB Norm 1+ 0 0 SMU Rapid All 1+ All 1+ All 1+ NC
Flexor Norm 0 0 0 2− Mod-V Some 1+ Some 1+ Some 1+ NC
Digitorum
Longus
Tibialis Norm 0 0 0 2− Mod-V Some 1+ Some 1+ Some 1+ NC
Polyneuropathies
Anterior
Gastrocnemius, 1+ 0 0 0 2− Mod Some 1+ Few 1+ Few 1+ NC
Medial Head
Rectus Norm 0 0 0 1− Mod Few 1+ Norm Few 1+ NC
Femoris
Tensor Fascia Norm 0 0 0 1− Mod Few 1+ Few 1+ Norm NC
Lata
Lumbar Norm 0 0 0 2− Mod Norm Norm Norm NC
paraspinal
(Low)
151
the lower extremity muscles. There are almost no The pattern of sensory involvement is also distal/
active/ongoing motor axon loss changes seen in length-dependent. Some clinical features such as
the form of fibrillation or positive sharp wave atrophy of the distal leg muscles, high arched feet
potentials. Accordingly, the secondary axon loss and scoliosis with onset likely in the childhood
is relatively mild and this is a predominantly and a positive family history suggest an underly-
demyelinating polyneuropathy. ing hereditary polyneuropathy. The mother being
IMPRESSION: affected points towards a possible autosomal dom-
There is electrodiagnostic evidence of an inant inherited polyneuropathy. It is not uncom-
acquired, chronic, non-length dependent senso- mon in longstanding inherited polyneuropathy for
rimotor predominantly demyelinating polyneu- patients to present late at this age (in 40s–50s)
ropathy, compatible with a diagnosis of chronic with worsening of their symptoms. Clinically dis-
inflammatory demyelinating polyneuropathy tal areflexia/reduced reflexes globally also suggest
(CIDP). an underlying demyelinating polyneuropathy.
Looking at the nerve conduction studies (see
Case 2 Table 6.11), the sensory responses are all absent in
A 50-year-old left-handed Caucasian female with the lower and the upper extremities. This strongly
a history of scoliosis (s/p corrective surgery at supports a diagnosis of polyneuropathy. Motor
childhood), presented to the neuromuscular clinic nerve conduction studies reveal an absent peroneal
for evaluation of progressive generalized weak- (fibular) motor response recorded at the extensor
ness. She reported having numbness and tingling digitorum brevis muscle and a severely reduced
in her feet over the past ~3 years with imbalance motor amplitude when recorded from the tibialis
and a couple of falls in the past year. She also anterior muscle, together with a markedly reduced
noted muscle twitching in her arms at night and motor conduction velocity (15.9 m/s) and pro-
cramps in her hands and feet. Her mother had sim- longed distal latency (7.15 ms) which is in the
ilar symptoms with weakness in the legs, and she demyelinating range. The tibial motor response is
died at the age of 76 years. No other family mem- also absent. In the upper extremity, the median
ber had similar problems. motor response recorded at the abductor pollicis
Examination was notable for thinning of distal brevis muscle reveals a markedly prolonged distal
legs and ankles and high arched feet on both sides. latency (13.55 ms), and reduced motor conduction
She was also noted to have bilateral foot drop velocity (17.1 m/s) with a severely reduced motor
which she recollected as being there since her amplitude. The ulnar motor response is absent.
childhood, requiring bracing. Muscle strength Such markedly reduced motor conduction veloci-
was moderately reduced proximally in the upper ties and prolonged distal motor latencies, without
and lower extremity. Distally, her finger abductors evidence of acquired demyelination (conduction
and ankle dorsiflexors were much weaker. Her block and temporal dispersion) are suggestive of
ankle reflex was absent, knee jerks were trace and an inherited demyelinating polyneuropathy.
the deep tendon reflexes in the upper extremity Needle electrode examination is most notable for
were very reduced. Sensations were markedly widespread marked chronic (without significant
reduced to all modalities in a glove-stocking pat- active/ongoing) motor axon loss changes in the
tern. No palpable nerves were appreciated. lower and upper limbs, with features generally
SUMMARY: conforming to a length-dependent patern as well
This is a 50-year-old lady with chronic/long- (see Table 6.11).
standing foot drop and at least moderate general- IMPRESSION:
ized weakness with sensory involvement and There is electrodiagnostic evidence of a
reduced to absent deep tendon reflexes. Clinically, chronic, length-dependent, sensorimotor, pre-
this suggests a polyneuropathy with both motor dominantly demyelinating poly neuropathy, with
and sensory involvement, predominating distally, marked secondary chronic axon loss features.
causing bilateral foot drop and hand weakness. Findings compatible with a hereditary etiology
6
Nerve conduction study
B-P Amp (μV) LatNPk (ms) CV (m/s) Dist (mm) Norm B-P Norm Temp (°C)
Nerve Stimulus Recording L R L R L R L R Amp LatNPk Norm CV L R
Sural Lower leg Lat. Malleolus NR NR n/a 140 >4 μV <4.6 ms >40 m/s 32.1
Polyneuropathies
Superficial peroneal lower leg Ankle NR NR n/a 100 >4 μV <4.6 ms 32.0
(fibular)
Median Wrist Index NR NR n/a 130 >15 μV <3.6 ms >50 m/s 33.6
Ulnar Wrist 5th Dig NR NR n/a 110 >10 μV <3.1 ms >50 m/s 33.7
Radial Thumb Forearm NR NR n/a 100 >14 μV <2.7 ms >50m/s 33.7
Peroneal Extensor Ankle NR NR n/a >2.5 mV <6 ms >40 m/s 31.7
(fibular) digitorum Pop Foss–Knee NR NR n/a 31.6
brevis
(EDB)
Tibial Abductor Ankle NR NR n/a >4 mV <6 ms >40 m/s 31.6
hallucis
(AH)
Peroneal Tibialis Below Fib Head 0.95 7.15 n/a >3 mV <4.5 ms >40 m/s 31.4
(fibular) anterior Pop Fossa–Knee 0.75 13.45 15.9 100 31.5
(TA)
Median Abductor Wrist 0.39 13.55 n/a 50 >6 mV <4 ms >50 m/s 34.0
pollicis Elbow 0.35 27.60 17.1 240 34.0
brevis Post exercise 0.37 15.40 n/a 33.9
(APB)
Ulnar Abductor Wrist NR NR n/a 50 >7 mV <3.1 ms >50 m/s 34.0
digiti
minimi
(ADM)
H-Reflex summary table
Nerve Stimulus Recording Side M-Wave H-Wave
Lat (ms) Amp (mV) Lat (ms) Amp (mV)
Pop Fossa Soleus Left NR NR
153
(continued)
154
Electromyography
Needle EMG summary
L 1st Dorsal Interosseous Norm 0 1+ 0 2− Mod-R Many 1+ Many 1+ Some 1+ NC
APB Norm 0 0 0 3− Rapid Many 1+ Many 1+ Some 2+ NC
Flexor Pollicis Longus Norm 0 0 0 3− Rapid Many 1+ Many 1+ Some 1+ NC
Pronator Teres Norm 0 0 0 2− Mod-R Some 1+ Few 1+ Some 1+ NC
Extensor Digitorum Norm 0 0 0 2− Mod-V Some 1+ Some 1+ Some 1+ NC
Biceps Brachii Norm 0 0 0 2− Rapid Some 1+ Some 1+ Norm NC
Triceps-Lateral Head Norm 0 0 0 1− Mod Few 1+ Few 1+ Some 1+ NC
Deltoid, Middle Head Norm 0 0 0 1− Mod-R Some 1+ Some 1+ Norm NC
Abductor Hallucis 1− 0 0 0 None Atrophy
EDB 1− 0 0 0 None Atrophy
Flexor Digitorum Longus 1− 0 0 0 SMU Rapid All 1+ All 1+ Norm NC
Tibialis Anterior 1− 0 0 0 3− Rapid Most 2+ 1+ Norm NC
Gastrocnemius, 1− 0 0+ 0 SMU Rapid All 2+ All 1+ Norm NC
Medial Head
Rectus Femoris 1− 0 0 0 3− Rapid All 2+ All 1+ Many 2+ NC
Tensor Fascia Lata Norm 0 0 0 3− Rapid Most 1+ Most 1+ Norm NC
M. C. Dhamne and J. A. Morren
include uniform conduction velocity slowing, as 16. Cho SC, Siao-Tick-Chong P, So YT. Clinical utility of
electrodiagnostic consultation in suspected polyneu-
well as conspicuous absence of conduction ropathy. Muscle Nerve. 2004;30(5):659–62.
blocks and/or temporal dispersion of M-wave 17. Gilliatt RW, Sears TA. Sensory nerve action potentials
configurations. in patients with peripheral nerve lesions. J Neurol
Neurosurg Psychiatry. 1958;21(2):109–18.
18. Barnett C, Perkins BA, Ngo M, Todorov S, Leung R,
Bril V. Sural-to-radial amplitude ratio in the diagno-
References sis of diabetic sensorimotor polyneuropathy. Muscle
Nerve. 2012;45(1):126–7.
1. Hanewinckel R, Drenthen J, van Oijen M, Hofman A, 19. Rutkove SB, Kothari MJ, Raynor EM, Levy ML,
van Doorn PA, Ikram MA. Prevalence of polyneurop- Fadic R, Nardin RA. Sural/radial amplitude ratio in
athy in the general middle-aged and elderly popula- the diagnosis of mild axonal polyneuropathy. Muscle
tion. Neurology. 2016;87(18):1892–8. Nerve. 1997;20(10):1236–41.
2. Prior R, Van Helleputte L, Benoy V, Van Den Bosch 20. Reeves ML, Seigler DE, Ayyar DR, Skyler JS. Medial
L. Defective axonal transport: a common pathological plantar sensory response. Sensitive indicator of
mechanism in inherited and acquired peripheral neu- peripheral nerve dysfunction in patients with diabetes
ropathies. Neurobiol Dis. 2017;105:300–20. mellitus. Am J Med. 1984;76(5):842–6.
3. Asbury AK, Arnason BG, Adams RD. The inflamma- 21. Killian JM, Foreman PJ. Clinical utility of dor-
tory lesion in idiopathic polyneuritis. Its role in patho- sal sural nerve conduction studies. Muscle Nerve.
genesis. Medicine (Baltimore). 1969;48(3):173–215. 2001;24(6):817–20.
4. Uncini A, Kuwabara S. Nodopathies of the periph- 22. Kural MA, Karlsson P, Pugdahl K, Isak B, Fuglsang-
eral nerve: an emerging concept. J Neurol Neurosurg Frederiksen A, Tankisi H. Diagnostic utility of distal
Psychiatry. 2015;86(11):1186–95. nerve conduction studies and sural near-nerve needle
5. Barohn RJ, Amato AA. Pattern-recognition approach recording in polyneuropathy. Clin Neurophysiol.
to neuropathy and neuronopathy. Neurol Clin. 2017;128(9):1590–5.
2013;31(2):343–61. 23. Tavee JO, Polston D, Zhou L, Shields RW, Butler RS,
6. Apfel SC, Asbury AK, Bril V, Burns TM, Campbell Levin KH. Sural sensory nerve action potential, epi-
JN, Chalk CH, et al. Positive neuropathic sensory dermal nerve fiber density, and quantitative sudomo-
symptoms as endpoints in diabetic neuropathy trials. J tor axon reflex in the healthy elderly. Muscle Nerve.
Neurol Sci. 2001;189(1–2):3–5. 2014;49(4):564–9.
7. Terkelsen AJ, Karlsson P, Lauria G, Freeman R, 24. Lo YL, Xu LQ, Leoh TH, Dan YF, Tan YE, Nurjannah
Finnerup NB, Jensen TS. The diagnostic challenge of S, et al. Superficial peroneal sensory and sural nerve
small fibre neuropathy: clinical presentations, evalua- conduction studies in peripheral neuropathy. J Clin
tions, and causes. Lancet Neurol. 2017;16(11):934–44. Neurosci. 2006;13(5):547–9.
8. Pasnoor M, Dimachkie MM, Kluding P, Barohn 25. AANEM policy statement on electrodiagnosis for
RJ. Diabetic neuropathy. Part 1: Overview and sym- distal symmetric polyneuropathy. Muscle Nerve.
metric phenotypes. Neurol Clin. 2013;31(2):425–45. 2018;57(2):337–9.
9. Callaghan BC, Cheng HT, Stables CL, Smith AL, 26. Dyck PJ, Albers JW, Andersen H, Arezzo JC,
Feldman EL. Diabetic neuropathy: clinical mani- Biessels GJ, Bril V, et al. Diabetic polyneuropathies:
festations and current treatments. Lancet Neurol. update on research definition, diagnostic criteria
2012;11(6):521–34. and estimation of severity. Diabetes Metab Res Rev.
10. Klein CJ, Duan X, Shy ME. Inherited neuropa- 2011;27(7):620–8.
thies: clinical overview and update. Muscle Nerve. 27. Huang CR, Chang WN, Tsai NW, Lu CH. Serial
2013;48(4):604–22. nerve conduction studies in vitamin B12
11. Katz JS, Saperstein DS, Gronseth G, Amato AA, deficiency-associated polyneuropathy. Neurol Sci.
Barohn RJ. Distal acquired demyelinating symmetric 2011;32(1):183–6.
neuropathy. Neurology. 2000;54(3):615–20. 28. Gordon PH, Wilbourn AJ. Early electrodiagnostic
12. Kissel JT, Mendell JR. Neuropathies associated with findings in Guillain-Barre syndrome. Arch Neurol.
monoclonal gammopathies. Neuromuscul Disord. 2001;58(6):913–7.
1996;6(1):3–18. 29. Kuwabara S, Ogawara K, Mizobuchi K, Koga M,
13. Willison HJ, Jacobs BC, van Doorn PA. Guillain- Mori M, Hattori T, et al. Isolated absence of F-waves
Barre syndrome. Lancet. 2016;388(10045):717–27. and proximal axonal dysfunction in Guillain-Barre
14. Peltier AC, Donofrio PD. Chronic inflammatory syndrome with antiganglioside antibodies. J Neurol
demyelinating polyradiculoneuropathy: from bench Neurosurg Psychiatry. 2000;68(2):191–5.
to bedside. Semin Neurol. 2012;32(3):187–95. 30. Bromberg MB, Albers JW. Patterns of sensory nerve
15. Rison RA, Beydoun SR. Paraproteinemic neuropathy: conduction abnormalities in demyelinating and
a practical review. BMC Neurol. 2016;16:13. axonal peripheral nerve disorders. Muscle Nerve.
1993;16(3):262–6.
31. Al-Shekhlee A, Hachwi RN, Preston DC, Katirji M. Consensus criteria for the diagnosis of multifocal
B. New criteria for early electrodiagnosis of acute motor neuropathy. Muscle Nerve. 2003;27(1):117–21.
inflammatory demyelinating polyneuropathy. Muscle 44. Caliandro P, Stalberg E, La Torre G, Padua
Nerve. 2005;32(1):66–72. L. Sensitivity of conventional motor nerve con-
32. Derksen A, Ritter C, Athar P, Kieseier BC, Mancias P, duction examination in detecting patchy demy-
Hartung HP, et al. Sural sparing pattern discriminates elination: a simulated model. Clin Neurophysiol.
Guillain-Barre syndrome from its mimics. Muscle 2007;118(7):1577–85.
Nerve. 2014;50(5):780–4. 45. Bromberg MB, Franssen H. Practical rules for elec-
33. Umapathi T, Li Z, Verma K, Yuki N. Sural-sparing trodiagnosis in suspected multifocal motor neuropa-
is seen in axonal as well as demyelinating forms thy. J Clin Neuromuscul Dis. 2015;16(3):141–52.
of Guillain-Barre syndrome. Clin Neurophysiol. 46. Joint Task Force of the E, the PNS. European
2015;126(12):2376–80. Federation of Neurological Societies/Peripheral
34. Hiew FL, Rajabally YA. Sural sparing in Guillain- Nerve Society guideline on management of multifo-
Barre syndrome subtypes: a reappraisal with his- cal motor neuropathy. Report of a joint task force of
torical and recent definitions. Clin Neurophysiol. the European Federation of Neurological Societies
2016;127(2):1683–8. and the Peripheral Nerve Society – first revision. J
35. Ye Y, Zhu D, Liu L, Wang K, Huang K, Hou Peripher Nerv Syst. 2010;15(4):295–301.
C. Electrophysiological measurement at Erb’s point 47. Research criteria for diagnosis of chronic inflamma-
during the early stage of Guillain-Barre syndrome. J tory demyelinating polyneuropathy (CIDP). Report
Clin Neurosci. 2014;21(5):786–9. from an Ad Hoc Subcommittee of the American
36. Albers JW, Donofrio PD, McGonagle TK. Sequential Academy of Neurology AIDS Task Force. Neurology.
electrodiagnostic abnormalities in acute inflamma- 1991;41(5):617–618.
tory demyelinating polyradiculoneuropathy. Muscle 48. Van den Bergh PY, Hadden RD, Bouche P, Cornblath
Nerve. 1985;8(6):528–39. DR, Hahn A, Illa I, et al. European Federation of
37. Hiraga A, Kuwabara S, Ogawara K, Misawa S, Neurological Societies/Peripheral Nerve Society
Kanesaka T, Koga M, et al. Patterns and serial changes guideline on management of chronic inflamma-
in electrodiagnostic abnormalities of axonal Guillain- tory demyelinating polyradiculoneuropathy: report
Barre syndrome. Neurology. 2005;64(5):856–60. of a joint task force of the European Federation
38. Uncini A, Kuwabara S. Electrodiagnostic crite- of Neurological Societies and the Peripheral
ria for Guillain-Barre syndrome: a critical revi- Nerve Society – first revision. Eur J Neurol.
sion and the need for an update. Clin Neurophysiol. 2010;17(3):356–63.
2012;123(8):1487–95. 49. Thaisetthawatkul P, Logigian EL, Herrmann
39. Rajabally YA, Durand MC, Mitchell J, Orlikowski D, DN. Dispersion of the distal compound muscle action
Nicolas G. Electrophysiological diagnosis of Guillain- potential as a diagnostic criterion for chronic inflam-
Barre syndrome subtype: could a single study suffice? matory demyelinating polyneuropathy. Neurology.
J Neurol Neurosurg Psychiatry. 2015;86(1):115–9. 2002;59(10):1526–32.
40. Umapathi T, Tan EY, Kokubun N, Verma K, Yuki 50. Katz JS, Wolfe GI, Bryan WW, Jackson CE,
N. Non-demyelinating, reversible conduction failure Amato AA, Barohn RJ. Electrophysiologic find-
in Fisher syndrome and related disorders. J Neurol ings in multifocal motor neuropathy. Neurology.
Neurosurg Psychiatry. 2012;83(9):941–8. 1997;48(3):700–7.
41. Caliandro P, Stalberg E, Padua L. In response to 51. Katz JS, Barohn RJ, Kojan S, Wolfe GI, Nations SP,
“Correlations of nerve conduction measures in axo- Saperstein DS, et al. Axonal multifocal motor neu-
nal and demyelinating polyneuropathies”. Clin ropathy without conduction block or other features of
Neurophysiol. 2008;119(7):1694–5. demyelination. Neurology. 2002;58(4):615–20.
42. Tankisi H, Pugdahl K, Johnsen B, Fuglsang- 52. Trojaborg W, Hays AP, van den Berg L, Younger DS,
Frederiksen A. Correlations of nerve conduction mea- Latov N. Motor conduction parameters in neuropa-
sures in axonal and demyelinating polyneuropathies. thies associated with anti-MAG antibodies and other
Clin Neurophysiol. 2007;118(11):2383–92. types of demyelinating and axonal neuropathies.
43. Olney RK, Lewis RA, Putnam TD, Campellone Muscle Nerve. 1995;18(7):730–5.
JV Jr. American Association of Electrodiagnostic
Brachial Plexopathies
7
Mark A. Ferrante
Introduction each brachial plexus region are known [7], the

sensory nerve conduction studies (NCS) are par-
The brachial plexus originates from the spinal ticularly important for localizing postganglionic,
cord and advances in an inferolateral direction axon loss lesions. The motor NCS are particu-
into the axilla. Its preterminal and terminal nerves larly important for defining lesion severity (and
supply innervation to the entire upper extremity, determining the underlying pathology, which is
as well as to most of the shoulder region. Of the usually axon loss with brachial plexus lesions).
somatic plexuses (cervical, brachial, lumbosa- The needle EMG study refines the NCS findings,
cral, coccygeal), brachial plexopathies are most provides temporal information about the lesion
frequent. The incidence of brachial plexopathies (e.g., the rate of progression), defines the degree
exceeds the combined incidences of the others. of reinnervation, and contributes to clinical man-
There are two major reasons for this vulnerabil- agement and prognosis of the patient. All of
ity: its greater susceptibility to trauma and its vul- these concepts are reviewed in most EDX text-
nerability to disorders involving adjacent books [4].
structures (e.g., blood vessels, lymph nodes, and Because the brachial plexus is such a large
the apex of the lung). Its greater susceptibility to structure, its complete assessment would be time
trauma (especially closed traction) reflects its and cost prohibitive (never mind the unnecessary
large size, its superficial position, and its location patient discomfort). Fortunately, most of its dis-
between two highly mobile structures (the neck orders involve only a single region of the brachial
and arm) (Fig. 7.1) [1–4]. plexus and, hence, can be easily characterized
Although the brachial plexus is the largest without a large EDX study [5, 6]. Because most
and most complex structure of the peripheral of the disorders affecting the brachial plexus are
nervous system (PNS), most brachial plexus dis- site specific (Table 7.1), once the lesion has been
orders are regional [5, 6]. Consequently, regional localized, the differential diagnosis is signifi-
electrodiagnostic (EDX) assessment is usually cantly reduced. In fact, not infrequently, the EDX
sufficient for lesion localization and character- findings dictate the underlying disorder.
ization. Because the sensory axons traversing The regional approach used in our EMG labo-
ratories has been previously reviewed [4, 5, 6]
and is reviewed again below. This approach
M. A. Ferrante (*) requires a thorough understanding of general and
University of Tennessee Health Science Center,
Memphis, TN, USA regional brachial plexus anatomy. For this rea-
e-mail: mferrant@uthsc.edu son, this chapter begins with a detailed review of

https://doi.org/10.1007/978-3-030-74997-2_7
158 M. A. Ferrante
Common carotid a.
Vertebral a.
Thyrocervical trunk
C2 Dorsal scapular a.
C3 Suprascapular a.
Thoracoacromial trunk
C4
C5 Medial cord
C6 Posterior cord
C7 Lateral cord
T1 Musculocutaneous n.
Radial n.
Medial n.
Subclavian a.
Ulnar n. Axillary a.
Subscapular a.
Fig. 7.1 The brachial plexus. The large size, superficial lymph nodes, and clavicle) also render it vulnerable.
location, and position between the neck and arm contrib- Reprinted with permission from Ferrante MA. Brachial
utes to the susceptibility of the brachial plexus to trauma. plexopathies: classification, causes, and consequences.
Diseases of adjacent structures (lung apex, blood vessels, Muscle Nerve 2004;30:547–568
Table 7.1 Site-Specific Brachial Plexus Disorders

Supraclavicular Plexus
Upper Plexus Lower Plexus
Classic postoperative paralysis True neurogenic TOS
Burner syndrome Post-median sternotomy brachial
plexopathy
Rucksack paralysis Pancoast syndrome
Infraclavicular plexus
Lateral cord Medial cord
Axillary lymph node irradiation Clavicular fractures (midshaft)
Terminal nerves
Median terminal Musculocutaneous Radial Axillary terminal
nerve terminal nerve terminal nerve
nerve
Medial brachial fascial Procedures near the coracoid process Crutch palsies Proximal humeral
compartment syndrome (iatrogenic) fractures
Glenohumeral
dislocations
Data compiled from [5, 6]
brachial plexus anatomy, followed by a discussion eneral Anatomy of the Brachial

G
of the EDX assessment of each of its regions. The Plexus
chapter concludes with 8 EDX exercises that
demonstrate the regional approach outlined in The brachial plexus is composed of connective tis-
this chapter. We have been using this approach sue and neural tissue in an approximate 2 to 1 ratio
for approximately 25 years and have found it to [8, 9, 10]. The axons composing the brachial
be quite reliable. Nevertheless, the reader should plexus—which number in excess of 100,000—rep-
be aware that there are other, equally effective, resent cytoplasmic extensions from the cell bodies
approaches. of motor, sensory, and autonomic neurons [11].
7 Brachial Plexopathies 159
The cell bodies of the motor neurons are also innervation ratio of the gastrocnemius muscle
referred to as anterior horn cells (AHCs) because (an example of a muscle that performs courser
of their location within the anterior horn of the spi- movements, such as ankle plantar flexion to
nal cord. Each motor neuron gives off a single, peek over a wall) is much greater than that of the
peripherally-directed axon. The cell bodies of the first dorsal interosseous muscle (a muscle for
sensory neurons are also termed dorsal root gan- which finer control is required, such as during
glion (DRG) cells because of their location within piano playing). Individual sensory axons do not
DRG. The DRG are located within the interverte- arborize distally; each innervates a single sen-
bral foramina of the spinal column. Each sensory sory receptor [4].
neuron gives off two axons, one peripherally-
directed and one centrally-directed. The centrally-
projecting axon is not studied by EDX testing (only lemental Anatomy of the Brachial
E
the peripherally-directed sensory axon and the Plexus
DRG cell are assessed by EDX testing). This
explains why the sensory responses are spared with Roots
radiculopathies (pre-ganglionic lesion).
After the axons exit the spinal column and After the motor and sensory axons exit the spi-
advance peripherally, they collect into groups nal cord, they coalesce, forming ventral and
called roots. In its traditional formation, the bra- dorsal rootlets, respectively. The ventral and
chial plexus is composed of the C5 through T1 dorsal rootlets coalesce to form the primary
roots. As these collections of axons advance, they ventral and dorsal roots. The latter traverse the
repeatedly come together and separate, each time intraspinal canal and enter the intervertebral
exchanging axons such that each element of the foramina. Within the intervertebral foramina
brachial plexus has a unique composition. This and just beyond the DRG, the ventral and dorsal
intermingling among the various axonal collec- roots fuse to form a mixed spinal nerve. The
tions produces the brachial plexus elements: 5 adjective, mixed, reflects the fact that these ele-
roots (C5 through T1), 3 trunks (upper, middle, ments contain both motor and sensory axons.
and lower), 6 divisions (3 anterior and 3 poste- Just outside the intervertebral foramen, each
rior), 3 cords (lateral, posterior, and medial), and mixed spinal nerve passes through a gutter
5 terminal nerves (Fig. 7.2). In addition to the 5 located along the superior aspect of the correlat-
terminal nerves, a number of preterminal nerves ing transverse process. Within these gutters, the
are given off more proximally. The preterminal C5 and C6 mixed spinal nerves are anchored to
and terminal nerves then advance and innervate the transverse process by connective tissue,
their end-organs (e.g., muscle fibers and sensory whereas the C8 and T1 mixed spinal nerves are
receptors). Because each element is composed of not. The connective tissue anchoring of the C7
unique sensory and motor axons, disorders affect- mixed spinal nerve varies. This difference in
ing individual brachial plexus elements result connective tissue anchoring accounts for the
in localizing clinical features [4]. lesion sites occurring with brachial plexus trac-
Distally, once the motor axon enters the mus- tion injuries. Because traction injuries tend to
cle that it innervates, it arborizes into a large disrupt axons at their anchorage points, traction
number of terminal branches, each of which involving C5- and C6-derived axons tends to
innervates a single muscle fiber via a single neu- produce ruptures of the C5 and C6 mixed spinal
romuscular junction (NMJ). The number of nerves, whereas traction involving the C8- and
muscle fibers innervated by a motor axon, T1-derived axons tends to produce root avulsion
termed the innervation ratio, is fairly constant injuries (due to their spinal cord anchorage site).
for each skeletal muscle. In general, it is The effect of traction on C7-derived axons
inversely proportional to the degree of control depends on their degree of anchorage to the
required over that muscle. For example, the transverse process.
160 M. A. Ferrante
Fig. 7.2 The elements “ROOTS”

of the brachial plexus. (APR)
C4
The brachial plexus is
composed of 5 roots, 3 C5
trunks, 6 divisions, 3 TRUNKS Suprascapular n.
C6 UP
cords, 5 terminal nerves, PE
R
and a number of C7 MI
DD
DIVISIONS
preterminal nerves, two LE
of which are shown in T1
the figure. Reprinted LO
W
with permission from ER LA CORDS
TE
Ferrante MA, Tsao PO RA
ST L
BE. Brachial ER TERMINAL
plexopathies. In: Katirji IO BRANCHES
ME R
B, Kaminski HJ, Ruff DI
AL
RL, editors.
Neuromuscular
Disorders in Clinical Musculo-
Long thoracic n. cutaneous n.
Practice. 2nd ed.
Radial n.
New York: Sprin
ger;2013:1029–1062). Median n.
Ulnar n. Axillary n.
Dorsal nerve rootlets
Ventral nerve rootlets

Dorsal root ganglion
Mixed spinal nerve
Posterior primary
ramus
Anterior primary
ramus
Gray ramus
communicans
White ramus
communicans
Sympathetic ganglion
Fig. 7.3 The relationship between the vertebral column mary ramus) and an anteriorly directed branch (the anterior
and the root elements of the brachial plexus. The dorsal primary ramus). Reprinted with permission from Ferrante
and ventral roots fuse to a mixed spinal nerve, which sub- MA. Brachial plexopathies: classification, causes, and
sequently divides into a directed branch (the posterior pri- consequences. Muscle Nerve 2004;30:547–568
Almost immediately after passing over the nerve branches innervate muscles (scalene
transverse process, the mixed spinal nerve and longus colli muscles; C5 through C8),
divides into a posteriorly directed branch whereas others join to form distinct pretermi-
(termed the posterior primary ramus) and an nal nerves (the phrenic nerve [C3 through C5
anteriorly directed branch (termed the ante- APR], the dorsal scapular nerve [C4 and C5
rior primary ramus [APR]) (Fig. 7.3). The C5 APR], and the long thoracic nerve [C5 through
through T1 APR exit between the anterior and C7 APR]).
middle scalene muscles and give off several Although most anatomists define the APR ele-
nerve branches. Some of these APR-derived ments as the roots of the brachial plexus, most
brachial plexologists and other clinicians dealing Trunks

extensively with brachial plexus disorders con-
sider the roots of the brachial plexus to include The 3 trunk elements, which are named for their
all of the structures proximal to the trunk ele- relationship to each other (upper, middle, and
ments. Thus, using this expanded definition, the lower), are formed from the APR near the lateral
roots have intraspinal canal, intraforaminal, and borders of the scalene muscles. The C5 and C6
extraforaminal segments. Because of the greater APR join to form the upper trunk, the C7 APR
clinical utility of the expanded definition, it is continues as the middle trunk, and the C8 and
used throughout the remainder of this chapter. T1 APR coalesce to form the lower trunk. Due
Preganglionic sympathetic axons also exit at to their superficial course, as they pass through
the level of the APR (from the C5 through T1 the posterior cervical triangle, these elements
APR). These fibers are myelinated and termed are susceptible to trauma. Because the lower
white rami communicantes (to reflect the whitish trunk lies adjacent to the apex of the lung and
hue imparted by the myelination). The pregangli- also passes next to the subclavian artery, disor-
onic sympathetic axons enter the nearby sympa- ders involving either of these two structures
thetic ganglia. Postganglionic sympathetic axons, may secondarily affect the lower trunk. Trunk
which are unmyelinated and, hence, termed gray anomalies are uncommon. In one report, the
rami communicantes, exit the sympathetic gan- middle trunk was of customary formation in
glia and enter the C5 through T1 mixed spinal 100%, the upper trunk in 90%, and the lower
nerves. Because the preganglionic sympathetic trunk in 95% [13]. The trunk level of the bra-
fibers supplying the head and neck traverse the chial plexus gives off 2 preterminal nerves—the
C8 and T1 roots on their way to the inferior cervi- suprascapular nerve (almost immediately upon
cal ganglion, a Horner syndrome may be observed its formation from the C5 and C6 roots) and the
with lesions affecting either of these two roots. nerve to subclavius. Each trunk terminates by
Thus, the presence of a Horner syndrome has dividing into two divisions, one anterior and one
localizing value. posterior.
It is important to realize that there are varia-
tions in the root composition of the brachial
plexus. As stated above, traditionally, the bra- Divisions
chial plexus is composed of axons derived from
the C5 through T1 spinal cord segments (i.e., the When the body is oriented in the anatomic posi-
C5 through T1 roots). Nerve root variations tion, the division elements lie behind the middle
include expansions (contributions from C4 or one-third of the clavicle (i.e., they are retrocla-
T2) and 1-segment vertical shifts. The brachial vicular). The anterior divisions primarily inner-
plexus is termed prefixed whenever the C4 con- vate flexors, whereas the posterior divisions
tribution is large and the T1 contribution is small, primarily innervate extensors. Consequently, the
whereas it is termed postfixed whenever the C5 anterior and posterior divisions are not always
contribution is small and the T2 contribution is similar in caliber. The anterior and posterior divi-
large [12]. Importantly, because these changes sions of the upper trunk are similar in size, the
do not affect the internal organization of the bra- posterior division of the middle trunk is larger
chial plexus, the clinical and EDX features that (because the C7 root primarily innervates exten-
permit lesion localization are unaffected [7]. In sor muscles), and the anterior division of the
other words, the clinical and EDX features of an lower trunk is larger (because the C8 and T1
upper trunk lesion are identical regardless of roots primarily innervate flexors) [10]. For this
whether the upper trunk is composed of C5- and reason, it is at the divisional level of the brachial
C6-dereived axons (traditional), C4- and plexus that the segmental nature of the roots and
C5-derived axons (prefixed), or C6- and trunks is lost. In general, there are no preterminal
C7-derived axons (postfixed). nerves given off at the divisional level.
162 M. A. Ferrante
Cords example, the median nerve axons are termed the

terminal median nerve while in the distal axilla
The three posterior divisions join to form the pos- and the median nerve after they exit the axilla.
terior cord, the anterior divisions of the upper and
middle trunk join to form the lateral cord, and the
anterior division of the lower trunk continues as egional Anatomy of the Brachial
R
the medial cord. The cords, which are named for Plexus
their relationship to the axillary artery, are the
longest elements of the brachial plexus and are Because of its large size and the fact that most
located in the proximal portion of the axilla, near brachial plexus disorders involve only a portion of
the axillary lymph node chain. the brachial plexus, it is helpful to divide the bra-
The lateral cord contains C6 and C7 sensory chial plexus into smaller regions. These smaller
axons and C5 through C7 motor axons. It gives regions of the brachial plexus are also termed
off the lateral pectoral preterminal nerve and the plexuses. The initial division is based on the ana-
musculocutaneous terminal nerve before termi- tomical relationship between the clavicle and the
nating as the lateral head of the median nerve. brachial plexus (when the upper extremity is
The posterior cord contains C5 through C7 sen- placed in the anatomical position). This relation-
sory axons and C5 through C8 motor axons. The ship allows the brachial plexus to be divided into
presence of T1-derived axons occurs less than 3 plexuses: (1) the supraclavicular plexus, which
5% of the time [9, 14]. The posterior cord gives contains the roots and trunks; (2) the retroclavicu-
off the thoracodorsal, the upper subscapular, and lar plexus, which contains the divisions; and (3)
the lower subscapular preterminal nerves before the infraclavicular plexus, which contains the
terminating as the axillary and radial terminal cords and terminal nerves. Not only does this
nerves. The posterior cord provides the sensory classification have anatomical significance, but it
axons of the C5 dermatome (via the upper and also has clinical significance—supraclavicular
lower lateral brachial cutaneous nerve branches plexopathies have a higher incidence and tend to
of the axillary and radial nerves, respectively). be more severe. Supraclavicular plexopathies tend
The medial cord contains C8 and T1 sensory to be more severe because a greater force is
axons and C8 and T1 motor axons. It gives off the required to produce them and because they are
medial pectoral, medial brachial cutaneous, and more frequently associated with closed traction
medial antebrachial cutaneous (MABC) preter- injuries [4, 11, 15].
minal nerves and the ulnar terminal nerve before The supraclavicular plexus is further divided
terminating as the medial head of the median into 3 even smaller plexuses. These, like the trunk
nerve. The latter joins the lateral head of the elements, are named for their relationship to each
median nerve to form the median terminal nerve. other: (1) the upper plexus, which consists of the
upper trunk and the C5 and C6 roots; (2) the mid-
dle plexus, which consists of the middle trunk
Terminal Nerves and the C7 root; and (3) the lower plexus, which
consists of the lower trunk and the C8 and T1
The 5 terminal nerves—musculocutaneous, axil- roots (Fig. 7.4). This subdivision also has clinical
lary, median, ulnar, and radial—are located in the significance—upper plexopathies have the high-
distal portion of the axilla. Each originates from est incidence, tend to occur in isolation, and most
a single cord element, except for the median commonly follow trauma (especially closed trac-
nerve, which originates from two cord elements tion) [7, 16]. For a number of reasons, upper
(medial and lateral cords). Once they exit the plexopathies tend to be less severe: (1) they are
axilla, they are no longer considered to be part of closer to the sensory receptors and muscle fibers
the brachial plexus and, at that point, are consid- that they supply; (2) they are more frequently
ered to be upper extremity nerves [5]. Thus, for extraforaminal, which increases the potential for
Fig. 7.4 The divisions

of the supraclavicular “ROOTS” Upper plexus
plexus. The (APR)
C4
supraclavicular plexus Middle plexus
is composed of three C5
smaller plexuses, the TRUNKS Lower plexus
upper plexus (upper C6
trunk and C5 and C6
roots), the middle C7
plexus (middle trunk
and C7 root), and the
T1
lower plexus (lower
trunk and C8 and T1
roots). Reprinted with
permission from
Ferrante MA, Tsao
BE. Brachial
plexopathies. In: Katirji
B, Kaminski HJ, Ruff
RL, editors.
Neuromuscular
Disorders in Clinical ANTERIOR PRIIMARY RAMI C5 C6 C7 C8 T1
Practice. 2nd ed. Proximal Nerve Innervation
New York: Springer; Rhomboid major/minor (dorsal scapular)
2013:1029–1062) Supraspinatus (suprascapular)
Infraspinatus (suprascapular)
Deltoid (axillary)
Biceps (musculocutaneous)
Brachialis (musculocutaneous)
Radial Nerve Innervation
Brachioradialis
Triceps
Anconeus
Extensor carpi radialis
Extensor indicis
Median Nerve Innervation
Pronator teres
Flexor carpi radialis
Flexor pollicis longus
Pronator quadratus
Abductor pollicis brevis
Ulnar Nerve
flexor digitorum profundus (D4,D5)
Adductor polllicis
First dorsal interosseous
POSTERIOR PRIMARY RAMI
Cervical paraspinal muscles
High thoracic paraspinal muscles
predominant contribution
sometimes significant contribution
minor contribution
164 M. A. Ferrante
surgical intervention; and (3) the lesions affect- On examination, because most brachial
ing the upper plexus more frequently have a plexopathies involve axon disruption, “negative”
demyelinating component [11]. Middle plexopa- deficits, such as weakness and numbness, are
thies most commonly follow trauma. Lower expected. Supraclavicular plexopathies produce
plexopathies, which have the lowest incidence, deficits suggesting involvement of one or more
also most commonly follow trauma [16]. Due to roots, whereas infraclavicular plexopathies pro-
their location between the upper plexus and the duce deficits suggesting involvement of one or
lower plexus, lesions involving the middle plexus more terminal nerves.
rarely occur in isolation. In one large series With upper plexopathies, the sensory loss
(n = 417), an isolated middle plexopathy was involves the C5 and C6 dermatomes, including
only observed once [7]. The infraclavicular the lateral aspects of the arm and forearm and the
plexus is not further subdivided. Moreover, infra- dorsolateral aspect of the hand. The weakness
clavicular plexus lesions do not show significant affects the C5 and C6 myotomes, including exter-
regional differences in incidence, severity, prog- nal humeral rotation, shoulder abduction, fore-
nosis, or lesion type [3, 5, 6]. arm flexion and supination, forearm pronation,
When the brachial plexus is considered and forearm extension. Depending on how proxi-
regionally (i.e., upper plexus, middle plexus, mal the lesion is located, weakness involving
lower plexus, lateral cord, posterior cord, and muscles innervated by the dorsal scapular nerve
medial cord regions), it facilitates communica- or the long thoracic nerve may be present. The
tion between physicians, allowing them to biceps and brachioradialis muscle stretch reflexes
regionally localize brachial plexus lesions in the (MSRs) may be hypoactive. With middle plexop-
setting of examination limitations (pain; cogni- athies, the sensory loss and weakness follows a
tive changes; higher priority injuries), as well as C7 distribution (forearm extension and prona-
prior to diagnostic testing (e.g., EDX testing). tion, radial wrist extension and flexion, and, to a
lesser degree, finger extension) and the triceps
MSR may be hypoactive. With lower plexopa-
Brachial Plexus Assessment thies, the sensory loss involves the C8 and T1
dermatomes, including the medial aspects of the
Clinical Assessment arm, forearm, and hand; and the weakness
involves muscles of the C8 and T1 myotomes.
Although the focus of this chapter is on the EDX The finger flexor reflex may be affected and,
assessment of the brachial plexus, because the depending on how proximal the lesion lies, a
EDX examination is an extension of the clinical Horner syndrome may be present.
examination, clinical assessment is briefly dis-
cussed. As always, clinical assessment of the bra-
chial plexus begins with a detailed history of the Electrodiagnostic Assessment
circumstances surrounding the onset of the prob-
lem and is followed by a thorough neurological Through EDX testing, brachial plexus lesions are
examination. In addition to a detailed assessment localized and characterized. Regarding the latter,
of the brachial plexus, the neurological examina- their pathophysiology, severity, rate of progression,
tion must include cervical spinal cord, cervical and degree of reinnervation are defined. These fea-
plexus, spinal accessory nerve, and phrenic nerve tures, in turn, contribute to patient management
assessments. Features of dysautonomia (e.g., and treatment, as well as lesion prognostication.
sudomotor or vasomotor abnormalities; Horner As stated earlier, because most lesions involve
syndrome) and of proximal brachial plexus only one region of the brachial plexus, a regional
involvement (dorsal scapular, phrenic, or long approach to lesion localization can be utilized.
thoracic nerve involvement; Horner syndrome) Because each region of the brachial plexus con-
are sought. tains a unique combination of sensory and motor
axons, lesions involving a single region of the stimulation of the motor axons. It is also referred
brachial plexus produce unique clinical and to as a compound muscle action potential
EDX features. The EDX abnormalities reflect (CMAP). The motor unit action potentials
the sensory and motor axons traversing each (MUAPs) collected during the needle EMG study
region. represents the muscle fiber action potentials of
The responses collected during EDX testing individual motor units.
represent compound electrical potentials, which The motor axons traversing a specific brachial
are composed of either nerve fiber action poten- plexus region determine the CMAP and muscle
tials or muscle fiber actions potentials. The sen- domains of that region, whereas the sensory
sory response represents all of the sensory nerve axons traversing it dictate its SNAP domain. The
fiber action potentials elicited by nerve stimula- unique SNAP, CMAP, and muscle domains of
tion of the sensory axons. It is also referred to as each brachial plexus element are provided
a compound sensory nerve fiber action potential (Table 7.2). These domains are easily derived and
(SNAP). The motor response represents all of the permit the course of the motor axons through the
muscle fiber action potentials elicited by nerve brachial plexus to be determined.
Table 7.2 The SNAP, CMAP, and Muscle Domains of the Brachial Plexus Regions
The Upper Plexus
SNAP Domain^ CMAP Domain Muscle Domain^^
LABC (100%) Musculocutaneous-biceps Levator scapulae
Median-D1 (100%) Axillary-deltoid Rhomboids
S-radial (60%) Radial-EDC Serratus anterior
Median-D2 (20%) Supraspinatus, infraspinatus
Median-D3 (10%) Biceps, brachialis
Deltoid, teres minor
Brachioradialis
Triceps
Pronator teres
Flexor carpi radialis
The Middle Plexus
SNAP domain CMAP domain Muscle domain
Median-D2 (80%) Pronator teres
Median-D3 (70%) Flexor carpi radialis
S-radial (40%) Triceps
Anconeus
Extensor digitorum communis
Serratus anterior
The Lower plexus
SNAP domain CMAP Domain Muscle domain
Ulnar-D5 (100%) Ulnar-ADM Abductor pollicis brevis
MABC (100%) Ulnar-FDI Flexor pollicis longus
Median-D3 (20%) Median-APB Extensor indicis proprius
Radial-EI Extensor pollicis brevis
Extensor carpi ulnaris
(continued)
166 M. A. Ferrante

The Upper Plexus
SNAP Domain^ CMAP Domain Muscle Domain^^
Adductor pollicis
Flexor digitorum profundus-4,5
The Lateral Cord
LABC (100%) Musculocutaneous (biceps) Biceps
Median-D1 (100%) Brachialis
Median-D2 (100%) Pronator teres
Median-D3 (80%) Flexor carpi radialis
The posterior cord
S-radial (100%) Axillary-deltoid Latissimus dorsi
Radial-ED Deltoid; teres minor
Radial-EI Triceps; anconeus
Brachioradialis
Extensor digitorum communis
Extensor carpi ulnaris
Extensor indicis proprius
The Medial Cord
SNAP Domain CMAP Domain Muscle Domain
Ulnar-D5 (100%) Ulnar-ADM Abductor pollicis brevis
MABC (100%) Ulnar-FDI Opponens pollicis
Median-D3 (20%) Median-APB Flexor pollicis longus
Adductor pollicis
Flexor digitorum profundus-4,5
^ The percentages shown in parentheses represent the frequency with which the sensory responses were abnormal for a
specific brachial plexus region. These data imply their dorsal root ganglia (DRG) derivation frequency and permit path-
ways to be generated.
^^The muscle domains provided are not exhaustive but, rather, reflect those muscles considered most helpful by the
author
he Motor Axon Pathways

T muscle begin in the C5 and C6 spinal cord seg-
The motor axons contained within each brachial ments and end in the axillary nerve. Thus, they
plexus element can be determined by knowing must traverse the C5 and C6 roots, the upper
the root innervation of a muscle. Myotomal plexus, and the posterior cord to reach the axil-
charts indicate the root innervations of the mus- lary nerve. Consequently, the Axillary-Deltoid
cles and most any anatomy book indicates the motor NCS and the needle EMG of the deltoid
specific nerve innervation of the muscles. In this muscle both assess all of these brachial plexus
manner, the CMAP and muscle domains for each elements. In summary, the CMAP domain and
element are determined. For example, because the muscle domain of a particular brachial plexus
the deltoid muscle is C5,6-axillary nerve- region is determined by the motor axons travers-
innervated, the axons innervating the deltoid ing it.
he Sensory Axon Pathways

T the C6 DRG [7]. Because the LABC nerve is a
Similarly, knowledge of the cell bodies of origin branch of the musculocutaneous nerve, it can be
of the sensory axons traversing the brachial concluded that the sensory axons must traverse
plexus allows SNAP domains to be determined the brachial plexus from the C6 DRG to the mus-
for each brachial plexus element. In 1995, the culocutaneous terminal nerve. Consequently, a
DRG of origin for the sensory axons subserving low amplitude LABC response may be associ-
the various upper extremity sensory NCS were ated with an axon loss lesion involving the C6
reported [7]. This information allowed the SNAP DRG, the C6 APR, the upper trunk, the lateral
domains for each brachial plexus region, as well cord, or the musculocutaneous terminal nerve (as
as the brachial plexus pathways of the sensory well as the musculocutaneous nerve and the
axons subserving each sensory NCS to be deter- LABC nerve).
mined (Figs. 7.5, 7.6, 7.7, 7.8, 7.9, 7.10, and The median sensory axons innervating the
7.11). These sensory fiber pathways are dis- thumb also derive from the C6 DRG [7].
cussed here. Consequently, a low amplitude median sensory
The sensory axons composing the lateral ante- response recording from the thumb (the
brachial cutaneous (LABC) nerve emanate from Median-D1 response) may be observed with an
C5
C6
C7
C8
T1
Fig. 7.5 The brachial plexus regions assessed by the lateral Electromyography: With Clinical Correlations and Case
antebrachial cutaneous nerve conduction study. Reprinted Studies. New York, Cambridge University Press; 2018
with permission from Ferrante MA. Comprehensive
C5
C6
C7
C8
T1
Fig. 7.6 The brachial plexus regions assessed by the MA. Comprehensive Electromyography: With Clinical
median sensory nerve conduction study, recording from Correlations and Case Studies. New York, Cambridge
the thumb. Reprinted with permission from Ferrante University Press; 2018
168 M. A. Ferrante
C5
C6
C7
C8
T1
median sensory nerve conduction study, recording from Correlations and Case Studies. New York, Cambridge
the index finger. Reprinted with permission from Ferrante University Press; 2018
C5
C6
C7
C8
T1
Fig. 7.8 The brachial plexus regions assessed by the Ferrante MA. Comprehensive Electromyography: With
median sensory nerve conduction study, recording from Clinical Correlations and Case Studies. New York,
the middle finger. Reprinted with permission from Cambridge University Press; 2018
Fig. 7.9 The brachial

plexus regions assessed
by the superficial radial
nerve conduction study. C5
Reprinted with
permission from C6
Ferrante MA. C7
Comprehensive
Electromyography: With C8
Clinical Correlations
T1
and Case Studies.
New York, Cambridge
University Press; 2018
axon loss lesion involving the C6 DRG, the C6 The median sensory axons innervating the
APR, the upper trunk, the lateral cord, or the index finger derive from the C6 DRG and the C7
median terminal nerve (as well as the median DRG. In our study, upper plexus lesions affected
nerve or its sensory branches to the thumb). this response (the Median-D2 response) 20% of
the time, mixed upper and middle plexus lesions
C5
C6
C7
C8
T1
ulnar sensory nerve conduction study, recording from the Correlations and Case Studies. New York, Cambridge
little finger. Reprinted with permission from Ferrante University Press; 2018
C5
C6
C7
C8
T1
Fig. 7.11 The brachial plexus regions assessed by the Electromyography: With Clinical Correlations and Case
medial antebrachial cutaneous nerve conduction study. Studies. New York, Cambridge University Press; 2018
Reprinted with permission from Ferrante MA. Comprehensive
affected this response 80% of the time, and lower response 70% of the time, and lower plexus
plexus lesions did not affect it [7]. This indicates lesions affected it 20% of the time [7].
that it is not an ideal study to assess the upper Consequently, a low amplitude Median-D3
plexus, as it is only affected in 20% of cases. response may be observed with an axon loss
Consequently, a low amplitude Median-D2 lesion involving the C6, C7, or C8 DRG; the C6,
response may be observed with an axon loss C7, or C8 APR; the upper, middle or lower
lesion involving the C6 or C7 DRG, the C6 or C7 trunk; the lateral or medial cord; or the median
APR, the upper or middle trunk, the lateral cord, terminal nerve (as well as the median nerve and
or the median terminal nerve (as well as the its sensory branches to digit 3). Because of its
median nerve and its sensory branches to the wide distribution, we infrequently use this sen-
index finger). sory NCS.
The median sensory axons innervating the The sensory axons composing the superficial
long finger derive from the C6 DRG, the C7 radial nerve derive from the C6 DRG and the C7
DRG, and the C8 DRG. In our study, upper DRG. In our study, upper plexus lesions affected
plexus lesions affected this response (the this response (the S-Radial response) 60% of the
Median-D3 response) 10% of the time, mixed time, mixed middle and lower plexus lesions
upper and middle plexus lesions affected this affected this response 40% of the time, and lower
170 M. A. Ferrante
plexus lesions did not affect it [7]. Consequently, fies the brachial plexus region involved. In our
a low amplitude superficial radial response may EMG laboratories, we begin all upper extremity
be observed with an axon loss lesion involving EDX assessments with three screening sensory
the C6 or C7 DRG, the C6 or C7 APR, the upper NCS –Median-D2, S-Radial, and Ulnar-D5. In
or middle trunk, the posterior cord, or the radial our EMG laboratories, additional sensory NCS
terminal nerve (as well as the radial nerve or the are added to these three screening studies based
superficial radial nerve). on the referral diagnosis (i.e., to address those
The sensory axons composing the ulnar nerve considerations of the referring physician) and
derive from the C8 DRG [7, 17, 18]. Consequently, based on the clinical features identified prior to
a low amplitude ulnar response recording from the start of the EDX study. From that point
the fifth digit (the Ulnar-D5 response) may be forward, the EDX study findings dictate the

observed with an axon loss lesion involving the subsequent studies (i.e., the EDX study is

C8 DRG, the C8 APR, the lower trunk, the medial independent).
cord, or the ulnar terminal nerve of the brachial
plexus (as well as the ulnar nerve or its sensory
branches to the fifth digit). Localizing Strategies
The sensory axons composing the MABC
nerve derive from the T1 DRG [7, 9, 17, 18, 19, Sensory NCS
20]. Consequently, a low amplitude MABC Based on the results of the screening sensory
response may be observed with an axon loss lesion NCS, additional sensory NCS are added.
involving the T1 DRG, the T1 APR, the lower Whenever the amplitude value of the S-Radial or
trunk, the medial cord, or the MABC preterminal the Median-D2 response is abnormal (both assess
(as well as the MABC nerve more distally). C6 or C7 DRG-derived sensory axons), we add
the LABC and the Median-D1 NCS because both
of these NCS only assess sensory axons derived
DX Assessment of the Brachial
E from the C6 DRG. Thus, their involvement or
Plexus by Region sparing shortens the list of potential lesion local-
ization sites. For example, whenever the
Introductory Comments Median-D2 response is reduced in amplitude and
either of the other two added sensory NCS is
Because of its large size, EDX assessment of the abnormal, the lesion localizes to the lateral cord
entire brachial plexus is impractical and, fortu- or upper plexus (i.e., the median nerve and middle
nately, almost always unnecessary because of the plexus sites are excluded). Whenever the S-Radial
regional involvement of most brachial plexus dis- response is reduced in amplitude and either of the
orders. Because each part of the EDX examina- two added sensory NCS is abnormal, the lesion
tion—sensory NCS, motor NCS, and needle localizes to the upper plexus (the radial nerve and
EMG—provides information not provided by the posterior cord localization sites are excluded).
other two parts, each part is mandatory. Although Whenever the amplitude value of the Ulnar-D5
the order of their performance varies among response is abnormal, the MABC NCS is added
EMG laboratories, we prefer to begin all EDX and, when it is also abnormal, the lesion localizes
assessments with the sensory NCS because they to the medial cord or lower plexus (i.e., the ulnar
are more sensitive to axon loss lesions involving nerve localization site is excluded). The ampli-
postganglionic PNS structures (i.e., plexus and tudes of the Ulnar-D5 and MABC responses
nerve lesions) than are the motor NCS. should also be compared to each other. When one
Based on the SNAP domains of the various of them is affected out of proportion to the other,
brachial plexus elements discussed earlier, the it may represent a lesion proximal to the lower
pattern of sensory response abnormalities identi- trunk (e.g., an APR level lesion).
Motor NCS trunk. The relationship between the acute and

Following the sensory NCS, the screening motor chronic changes indicates the rate of progression
NCS—Median-APB and Ulnar-ADM—are per- of the disorder. For example, the presence of
formed. To these two studies, additional motor large numbers of high amplitude fibrillation
NCS are added based on the localizing informa- potentials indicates a relatively recent process,
tion derived from the sensory NCS. The motor whereas the presence of long duration MUAPs
NCS are important in determining the underlying implies chronicity. Regarding the rate of progres-
pathology and, especially, for establishing lesion sion, the presence of large quantities of high
severity. The severity is determined by compar- amplitude fibrillation potentials (typically with
ing the amplitude or negative area under the low and medium amplitude fibrillation poten-
curve value of the distal response to that of the tials) and chronic MUAP changes implies that
contralateral, asymptomatic side. The lure to the underlying disorder is rapidly progressive
grade lesion severity based on fibrillation poten- (e.g., amyotrophic lateral sclerosis), whereas the
tial quantity must be resisted because it is more presence of sparse, low amplitude fibrillation
dependent on the timing of the study than on potentials and large quantities of long duration,
lesion severity. Following reinnervation via col- high amplitude MUAPs implies that the underly-
lateral sprouting, the motor responses underesti- ing disorder is more slowly progressive (e.g.,
mate lesion severity. To avoid missing significant Kennedy disease).
motor axon loss, the needle EMG study is always The needle EMG may also disclose focal
performed, even when the motor responses are demyelinating conduction block lesions that are
normal. located proximal to the motor NCS performed.
For example, whenever the median motor NCS is
he Needle EMG
T normal but the needle EMG study of the abductor
The needle EMG portion of the EDX study is pollicis brevis muscle shows neurogenic recruit-
performed last. Because of the high innervation ment, a demyelinating conduction block must be
ratio of most skeletal muscles, the needle EMG present proximal to the elbow stimulation site.
can show large numbers of fibrillation potentials This is true because neurogenic recruitment only
when there is no motor NCS evidence (or clinical occurs when APs are unable to traverse the lesion
evidence) of motor axon loss. Thus, it is the most (demyelinating conduction block or axon loss).
sensitive portion of the EDX examination for Because the distal motor response is normal,
identifying motor axon loss. For this same rea- axon loss is excluded. Thus, the only possibility
son, the needle EMG study can demonstrate is demyelinating conduction block. Because the
nerve continuity when there are no perceptible distal motor response is normal, it cannot be dis-
movements clinically. tal to the distal stimulation site. Because the dis-
The needle EMG study not only confirms the tal and proximal motor responses were normal, it
impression of the sensory and motor NCS, it fur- cannot be between these two stimulation sites.
ther localizes and characterizes the lesion (e.g., Consequently, it must be located proximal to the
its temporal characteristics). It further refines the proximal stimulation site.
location of the lesion in those areas of the plexus
where distinctions are possible (i.e., where nerve
elements join or separate from each other). For CS Assessment of Specific Brachial
N
example, when the sensory and motor NCS local- Plexus Regions
ize the lesion to either the medial cord or the
lower plexus, involvement of the extensor indicis The Upper Plexus
muscle on needle EMG eliminates the possibility
of a medial cord localization. As another exam- The upper plexus contains motor and sensory
ple, the needle EMG study can also differentiate axons derived from the C5 and C6 spinal cord
a C6 APR lesion from one involving the upper segments. The SNAP, CMAP, and muscle
172 M. A. Ferrante
domains of the upper plexus are provided is typically required when localizing lesions to
(Table 7.2). There are no reliable sensory NCS the middle plexus.
that assess the C5 sensory fibers of the upper On sensory NCS, middle trunk lesions are dif-
plexus. The LABC and Median-D1 NCS assess ferentiated from posterior cord lesions by the
its C6 fibers. These two sensory NCS show good Median-D2 and the Median-D3 sensory NCS
correlation with each other and are typically both (both are spared with posterior cord lesions). On
involved to similar degrees [7]. The S-Radial sen- needle EMG testing, the C6,7-median nerve
sory NCS and the Median-D2 sensory NCS also innervated muscles (e.g., pronator teres; flexor
assess the C6 fibers of the upper plexus, but less carpi radialis) are helpful to differentiate middle
frequently (60% and 20%, respectively) [7]. On plexus lesions (involved) from posterior cord
motor NCS testing, the screening motor NCS are lesions (spared). The C5,6-radial and the C5,6-
of no value in the EDX assessment of this region. axillary nerve-innervated muscles are also help-
Thus, additional motor NCS must be added ful in differentiating a middle plexus lesion
whenever an upper plexus lesion is suspected. (spared) from a posterior cord lesion (involved).
Both the Musculocutaneous-Biceps NCS and the
Axillary-Deltoid NCS assess the upper plexus in he Lower Plexus
T
its entirety (i.e., the C5 root, the C6 root, and the The lower plexus contains motor and sensory
upper trunk). On needle EMG, additional shoul- axons derived from the C8 and T1 spinal cord
der girdle and upper extremity muscles are added segments. The SNAP, CMAP, and muscle
because the screening muscles do not assess the domains of the lower plexus are provided
upper plexus very well. (Table 7.2). On sensory NCS testing, the
On sensory NCS, when involved, the S-Radial Ulnar-D5 and MABC NCS assess its C8 and T1
NCS helps differentiate an upper plexus lesion sensory axons, respectively. With lower trunk
(involved) from a lateral cord lesion (spared). On involvement, both responses are affected,
motor NCS, the Musculocutaneous-Biceps whereas with C8 APR lesions, the Ulnar-D5
response may be affected (depending on lesion response is affected in isolation and with T1 APR
severity), whereas the Axillary-Deltoid response lesions, the MABC response is affected in isola-
is spared. When involved, muscles innervated by tion. On motor NCS, the Ulnar-ADM, the Ulnar-
the dorsal scapular, long thoracic, and suprascap- FDI, the Median-APB, and the Radial-EI NCS
ular nerve are helpful in differentiating an APR- assess the lower plexus. These four motor NCS
level lesion (involved) from an upper trunk lesion assess the C8 and T1 APR to differing degrees—
(spared). When involved, the C5,6-radial axons the Radial-EI NCS assesses solely the C8 APR,
are helpful in differentiating upper plexus lesions the Ulnar-ADM and Ulnar-FDI NCS assess the
(involved) from lateral cord lesions (spared). C8 and T1 more equally, and the Median-APB
NCS assesses the T1 APR to a greater extent than
he Middle Plexus
T the C8 APR [7, 17, 18]. On needle EMG,
The middle plexus contains motor and sensory C8-radial and C8-T1 ulnar and median nerve-
axons derived from the C7 spinal cord segment. innervated muscles are utilized.
The SNAP, CMAP, and muscle domains of the When involved, the C8-radial axons (Radial-EI
middle plexus are provided (Table 7.2). On sen- motor NCS; extensor indicis and extensor polli-
sory NCS testing, the Median-D2, Median-D3, cis brevis muscles on needle EMG study) are
and S-Radial NCS assess its fibers about 80%, especially useful in differentiating a lower plexus
70%, and 40% of the time [7]. There is no motor lesion (involved) from a medial cord lesion
NCS that solely assesses the middle plexus. We (spared).
usually perform the Radial-EDC NCS. Because
of the overlap between the muscle domains of the he Lateral Cord
T
upper plexus, the middle plexus, and the lower The lateral cord contains motor axons derived
plexus, a more extensive needle EMG assessment from the C5 through C7 spinal cord segments and
sensory axons derived from the C6 and C7 spinal domains of the medial cord are provided
cord segments. The SNAP, CMAP, and muscle (Table 7.2). On sensory NCS, it is assessed by the
domains of the lateral cord are provided Ulnar-D5 and MABC NCS. On motor NCS, the
(Table 7.2). On sensory NCS testing, the LABC, Ulnar-ADM, Ulnar-FDI, and Median-APB are
the Median-D1, the Median-D2, and the useful. On needle EMG, C8,T1-median and ulnar
Median-D3 NCS assess its fibers 100%, 100%, nerve-innervated muscles are helpful.
and 100%, and 80% of the time [7]. In general, As previously stated, the C8-radial nerve-
with lateral cord lesions, the LABC, Median-D1 innervated muscles (extensor indicis; extensor
and Median-D2 are affected to similar degrees, pollicis brevis) are helpful in differentiating
whereas with upper plexus lesions, the LABC lower plexus lesions (potentially involved) from
and Median-D1 responses are typically affected medial cord lesions (always spared). It is impor-
to a greater extent than the Median-D2 response tant to understand that sparing of these muscles
[7]. On motor NCS, the Musculocutaneous- does not always indicate that the lesion localizes
Biceps response may be affected (depending on to the medial cord because partial lower plexus
lesion severity), whereas the Axillary-Deltoid lesions may also spare them.
response is spared. Needle EMG abnormalities
are confined to musculocutaneous nerve and he Preterminal and Terminal Nerves
T
C6,7-median nerve innervated muscles (e.g., pro- Sensory NCS are available for all of the terminal
nator teres; flexor carpi radialis). With lateral nerves except the axillary nerve. Motor NCS are
cord lesions, the C5,6 muscles innervated by the available for all 5 terminal nerves. Many of the
axillary, radial, suprascapular, long thoracic, and preterminal nerves are assessable by motor NCS
dorsal scapular nerves are spared. (e.g., Phrenic-Diaphragm; Suprascapular-
Infraspinatus). Because of identical sensory and
he Posterior Cord
T motor axon composition, a terminal nerve lesion
The posterior cord contains motor axons derived of the brachial plexus cannot be differentiated
from the C5 through C8 spinal cord segments and from a proximally-located upper extremity neu-
sensory axons derived from the C5 through C7 ropathy of the same name.
spinal cord segments. The SNAP, CMAP, and
muscle domains of the lateral cord are provided
(Table 7.2). On sensory NCS, it is assessed by the DX Exercises in Brachial Plexus
E
S-Radial NCS. On motor NCS, it is assessed by Localization
the Axillary-Deltoid and both radial motor NCS
(recording extensor digitorum communis and Although the EDX study is an extension of the
extensor indicis). On needle EMG, muscles clinical examination, it is nonetheless an inde-
innervated by the axillary, radial, and thoracodor- pendent study. In order to maintain its indepen-
sal nerves are helpful. dence, it must remain unbiased by the clinical
Posterior cord lesions are differentiated from features once it begins. The EDX findings, not
middle trunk lesions by the Median-D2 and the clinical findings, generate the EDX study
Median-D3 sensory NCS (spared with posterior conclusions. Nonetheless, following the EDX
cord lesions) and by needle EMG of C6,7-median conclusion, it is often helpful to the referring pro-
nerve innervated muscles (e.g., pronator teres; vider to relate the EDX conclusions to the clini-
flexor carpi radialis), which are spared with pos- cal features. Again, this is done, when indicated,
terior cord lesions. after the EDX conclusions have already been
clearly stated.
he Medial Cord
T To convey the fact that the EDX study is an
The medial cord contains motor and sensory independent study, these 8 exercises provide
axons derived from the C8 and T1 spinal cord very little clinical detail so that the indepen-
segments. The SNAP, CMAP, and muscle dence of the EDX study is exemplified. Even in
174 M. A. Ferrante
the setting of the EDX assessment of individu- (Ulnar-D5), and the S-Radial sensory NCS,
als with brachial plexus lesions, very little clini- recording from the dorsolateral aspect of the
cal detail is required. In fact, simply knowing hand (S-Radial)—along with any studies required
which limb is symptomatic is enough to obtain a based on the clinical features collected prior to
detailed history and focused examination. The the start of the EDX study. Whenever the
clinical features obtained generate a differential Median-D2 or S-Radial response is abnormal
diagnosis and, thus, a starting point for the EDX (i.e., the sensory NCS that assess C6 and C7
study. Once initiated, the EDX study continues DRG-derived sensory axons), we add the LABC
along in a dynamic fashion. Additional EDX and the Median-D1 sensory NCS because they
studies are determined by results of the previous only assess the C6 DRG-derived sensory axons
EDX studies. The EDX study continues in this and, thus, clarify the potential lesion localization
manner until the lesion has been fully localized sites. Whenever the Ulnar-D5 response is abnor-
and characterized (pathology, severity, temporal mal (assesses C8 DRG-derived sensory axons),
features). we add the MABC NCS (assesses T1 DRG-
Each of the 8 exercises begins with the infor- derived sensory axons) to better clarify the poten-
mation provided by the referring provider and tial lesion localization sites.
the abbreviated clinical features collected by the Because the overwhelming majority of bra-
EDX provider. The sensory NCS are performed chial plexopathies are related to axon loss and a
first. The sensory NCS permit axon loss lesion minority to demyelinating conduction block,
localization (axon loss lesions represent the only the amplitude values of the responses are
overwhelming majority of brachial plexopa- presented, all of which are rounded to the nearest
thies). The motor NCS are performed next. whole number. Although the latency and conduc-
These studies localize focal demyelinating tion velocity values are helpful for identifying
lesions and, most importantly, assess lesion demyelinating conduction slowing, this patho-
severity. Finally, the needle EMG study is per- physiology does not produce negative symptoms
formed. It is helpful to confirm and fine tune the (i.e., weakness; numbness) but, instead, only iso-
NCS conclusions and to define the temporal lated positive symptoms (e.g., episodic tingling).
characteristics of the lesion. The EDX data are Isolated demyelinating conduction slowing is
presented and discussed as the study unfolds, never observed among individuals with brachial
rather than all at once. This permits a deeper plexopathies. For this reason, and to simplify the
understanding of the dynamic nature of EDX collected data for the 8 exercise presented below,
testing (e.g., the need for additional EDX studies the peak latency values (sensory NCS), the distal
and why they are needed). onset latency values (motor NCS), and the motor
Regarding the sensory NCS, it is important to NCV values are replaced with an “X” in the data
realize the DRG from which the sensory axons tables. Except with severe axon loss, unless all of
subserving the sensory NCS are derived. In this the large-diameter, heavily myelinated fibers are
manner, just like motor axons, the pathways of disrupted, the latency and conduction velocity
the sensory axons through the brachial plexus are values are normal. Thus, in general, with axon
determinable. This information is included in the loss disorders, the latency and conduction veloc-
tables and is derived from our original work pub- ity values do not contribute to the EDX study. In
lished in 1995 [7]. Each exercise begins with 3 the 8 cases discussed below, as expected, the
screening sensory NCS—the median sensory latency and conduction velocity values were nor-
NCS, recording index finger (Median-D2), the mal. Thus, it is easiest to discuss these exercises
ulnar sensory NCS, recording little finger with the values replaced by an “X.” Over a
12-year period, we successfully used this method Because these EDX exercises are included to
of presenting NCS data in our popular American teach brachial plexus lesion localization using
Academy of Neurology course: EDX of the our regional approach, rather than listing all of
Brachial Plexus. For NCS, the amplitude value is the individual needle EMG findings, the muscles
the most important measurement made. With are simply identified as normal or abnormal. Like
needle EMG, however, the duration of the MUAP other EMG laboratories, we consider a muscle to
is the most important measurement collected. be abnormal whenever EDX evidence of acute
MUAP duration is much more sensitive to rein- motor axon loss (e.g., fibrillation potentials),
nervation via collateral sprouting than is MUAP chronic motor axon loss (e.g., increased MUAP
amplitude. Unless significant collateral sprouting duration or, less commonly, increased MUAP
has occurred, the MUAP amplitude is normal, amplitude), or neurogenic recruitment is
whereas the MUAP duration becomes abnormal observed.
with even mild degrees of collateral sprouting, Muscle abbreviations utilized for these exer-
especially when the MUAPs are compared to the cises are: ADM (abductor digiti minimi), APB
contralateral side. The MUAP duration is muscle- (abductor pollicis brevis), ECR (extensor carpi
dependent and age-dependent. For example, for radialis), ECU (extensor carpi ulnaris), ED
the upper extremity, the brachioradialis has the (extensor digitorum), EI (extensor indicis), EPB
shortest duration MUAPs, whereas the triceps (extensor pollicis brevis), FCR (flexor carpi radi-
and deltoid muscles have the longest. This hierar- alis), FDI (first dorsal interosseous), FCU (flexor
chy is addressed in some EDX textbooks carpi ulnaris), FDP-4,5 (flexor digitorum profun-
(Ferrante TB). dus to the fourth vand fifth digits), and FPL
When indicated, contralateral NCS were per- (flexor pollicis longus).
formed. The performance of contralateral stud-
ies helps to identify relative abnormalities. We
consider a study to be relatively abnormal Brachial Plexus Exercises
whenever its amplitude value is more than 50%
smaller than the value recorded from the contra- Exercise 1
lateral side. Often, as will be demonstrated by
many of these exercises, the lesion is localized A 58-year-old right hand dominant female is
during the sensory NCS. When we use the term referred for EDX assessment of left upper
upper plexus (upper trunk and C5 and C6 roots), extremity pain and weakness. These symptoms
middle plexus (middle trunk and C7 root), and began 2 months ago. She reports a history of
lower plexus (lower trunk and C8 and T1 roots), breast cancer, for which she did not receive radia-
we are referring to the expanded definition of tion therapy.
root, which includes the anterior primary ramus The limited clinical features provided indicate
and all of the neural structures proximal to it involvement of the right upper extremity. Thus,
(with the expanded definition, the root element the 3 screening sensory NCS are performed on
is composed of the anterior primary ramus, the the right side. In our EMG laboratories, we begin
mixed spinal nerve, the primary root, and the with 3 sensory NCS—the median sensory NCS,
rootlets exiting from the spinal cord). Using this recording index finger (Median-D2), the ulnar
approach, an avulsion lesion involving the C5 sensory NCS, recording little finger (Ulnar-D5),
root would be considered an upper plexus bra- and the S-Radial sensory NCS, recording from
chial plexopathy. the dorsolateral aspect of the hand (Radial).
176 M. A. Ferrante
Nerve Conduction Studies

Upper extremity nerve conduction study worksheet
Case 1 Left Right
NCS Performed DRG LAT AMP CV nAUC LAT AMP CV nAUC
Sensory
Median-D2 C6,7 X 26
Ulnar-D5 C8 X 18
S-radial C6,7 X 4
DRG Dorsal root ganglion/ganglia, LAT Latency, AMP Amplitude, CV Conduction velocity, nAUC Negative area under
the curve
The sensory NCS are abnormal. The amplitude or middle plexus [7]. The two normal sensory
value of the left superficial radial response is responses support this list of potential lesion
reduced, indicative of axon loss. Because the sen- localizations, but do not shorten it.
sory axons subserving the superficial radial nerve To address these potential localization sites,
derive from the C6 and C7 DRG, the potential the LABC and Median-D1 responses are needed.
lesion sites include the superficial radial nerve, Also, the contralateral S-Radial NCS is needed
the radial nerve, the posterior cord, or the upper (for comparison purposes).

Case 1 Left Right
Sensory
Median-D2 C6,7 X 26
Ulnar-D5 C8 X 18
S-radial C6,7 X 4 X 20
the curve
Based on these findings, additional sensory motor NCS, the following sensory NCS are
NCS are required. Thus, prior to advancing to the performed:

Case 1 Left Right
Sensory
Median-D2 C6,7 X 26
Ulnar-D5 C8 X 18
LABC C6 X NR X 10
Median-D1 C6 X NR X 16
the curve
The LABC and Median-D1 responses are unclear because there are no sensory NCS avail-
absent. Their involvement eliminates the superfi- able to assess these axons.
cial radial nerve, the radial nerve, the posterior With upper plexus lesions, to the routine
cord, and the middle plexus as lesion localization motor NCS, we bilaterally add the axillary NCS,
sites. Thus, at this point, the lesion is axon loss in recording deltoid (Axillary-Deltoid) and muscu-
nature and involves the upper plexus, at least its locutaneous NCS, recording biceps (Musculocut-
C6 DRG derived sensory axons. Whether the C5 Biceps) to assess lesion severity.
DRG-derived sensory axons are affected is

Case 1 Left Right
Sensory
Median-D2 C6,7 X 26
Ulnar-D5 C8 X 18
LABC C6 X NR X 10
Median-D1 C6 X NR X 16
Motor Stim Site
Median-APB X 12
12 X
Ulnar-ADM X 13
13 X
Musculocut-biceps X 3 X 6
3 X
Axillary-deltoid X 4 X 9
the curve
The screening motor NCS, as expected, are The abnormal muscles are all within the mus-
normal. The amplitude values of the musculocu- cle domain of the upper plexus. Sparing of the
taneous and axillary motor responses are abnor- spinati muscles and the serratus anterior suggest
mal and indicate an axon loss lesion. The that the lesion involves the upper trunk because
relationship between the distal motor responses the long thoracic nerve exits the plexus from the
on the two sides indicates that approximately C5, C6, and C7 anterior primary rami and the
50% of the musculocutaneous motor axons inner- suprascapular nerve exits just after the formation
vating the biceps muscle are disrupted of the upper trunk (i.e., it is usually spared with
(1–3/6 = 0.5) and approximately 56% of the axil- upper trunk disorders). The presence of large
lary motor axons innervating the deltoid muscle amplitude fibrillation potentials and mildly long
are disrupted (1–4/9 = 0.56). it is important to duration MUAPs indicates recent denervation
appreciate that reinnervation through collateral and some reinnervation, respectively, consistent
sprouting improves the motor response because it with a lesion that occurred 2 months ago.
reflects the number of muscle fibers innervated,
not the number of motor axons composing the EDX Conclusion
nerve. Given that the symptoms started 2 months 1. Upper Plexopathy
ago, we use the term “approximately.” Had the
symptoms started more than 3 months ago, we The above is axon loss in nature, involves the
would replace “approximately” with “at least” to sensory and motor axons, and is at least moderate-
indicate that the degree of motor axon loss might severe in degree.
be an underestimate due to the presence of col-
lateral sprouting. This should be supported by the Final Comments
needle EMG as long duration MUAPs.
• The amplitude values of the sensory NCS are
he Needle EMG Study
T essential for localizing axon loss brachial
• Abnormal muscles: Deltoid, brachioradialis, plexopathies
biceps, pronator teres, FCR –– The LABC and Median-D1 sensory NCS
• Normal muscles: Trapezius, serratus anterior, are extremely useful for assessing the upper
supraspinatus, infraspinatus, triceps, EDC, plexus because the sensory axons subserv-
EIP, FCU, FPL, APB, FDI, paraspinals. ing them derive from the C6 DRG [7]. Thus,
178 M. A. Ferrante
their involvement could not be explained by • The because the dorsal scapular and long tho-
a middle plexus lesion. racic nerves exit the plexus from its anterior
–– Because the sensory axons subserving the primary ramus level, they are spared with
Median-D2 sensory NCS are only abnor- upper trunk lesions. Because the suprascapu-
mal in 20% of upper plexus lesions [7], lar nerve exits from the upper trunk just after
they are not useful for screening this ele- its formation, it is frequently spared with
ment of the brachial plexus. upper trunk lesions.
• The musculocutaneous and axillary motor • When involved, the C5,6-axillary nerve inner-
responses are helpful for determining the vated muscles (deltoid; teres minor) and the
severity of the lesion. This can be calculated C5,6-radial nerve innervated muscles (e.g.,
for axon loss lesions by comparing the distal brachioradialis) are helpful for differentiating
motor response amplitude values (or negative an upper plexus lesion (involved) from a lat-
AUC values) of the two sides. After enough eral cord lesion (spared).
time has elapsed for reinnervation via collat-
eral sprouting to occur, the calculated value is
an underestimate because the calculated value Exercise 2
reflects the number of innervated muscle fibers
not the number of functioning motor axons. A 41-year-old female is referred for EDX assess-
Thus, we study the muscle on needle EMG for ment of distal upper extremity sensory and motor
evidence of reinnervation via collateral sprout- abnormalities that followed a fall onto her out-
ing. For lesions of less than 3 months duration stretched left arm 4 weeks earlier.
without evidence of reinnervation via collat- The distal weakness suggests C8 and T1 fiber
eral sprouting (i.e., absence of long duration involvement, given that it includes the hand, but
MUAPs), we use the term “approximately,” otherwise, there are no clinical clues. We do
whereas with lesions of greater than 3 months know that the left upper extremity is the symp-
with evidence of reinnervation via collateral tomatic limb. Thus, the screening sensory NCS
sprouting (i.e., the presence of long duration are performed on that limb first.
MUAPs), we use “at least.”

Case 2 Left Right
Sensory
Median-D2 C6,7 X 30
Ulnar-D5 C8 X NR
S-radial C6,7 X 21
the curve
The left Ulnar-D5 sensory response is absent, whenever the Ulnar-D5 sensory response is
indicative of an axon loss lesion that is localized abnormal, we add the MABC NCS to better
to the ulnar nerve, the medial cord, or the C8 assess the medial cord and lower plexus. When
fibers of the lower plexus [7]. The other responses the MABC is unaffected, we add the DUC
are normal and do not affect this list of potential NCS. We also need to add the contralateral
lesion localizations. In our EMG laboratories, Ulnar-D5.

Case 2 Left Right
Sensory
Median-D2 C6,7 X 30
Ulnar-D5 C8 X NR X 14
S-radial C6,7 X 21
MABC T1 X NR X 12
the curve
The MABC response is absent, indicative of tions. This differentiation requires C8-radial
an axon loss process involving the MABC nerve, motor axon assessment via the motor NCS or the
the medial cord, or the T1 fibers of the lower needle EMG study. The C8-radial motor axons
plexus. Thus, this cannot be an ulnar neuropathy traverse the lower plexus and the posterior cord.
and, for this reason, there is no need to perform Thus, their involvement would indicate a lower
the DUC NCS. Also, because the ulnar response plexus lesion. However, their sparing does not
is abnormal, this cannot be an MABC lesion. indicate a medial cord lesion because a partial
Thus, because the ulnar and the MABC responses lower plexus lesion would still be possible. The
are both affected, the lesion must involve the C8 radial motor axons innervate the extensor
medial cord or the lower plexus (both its C8 and indicis muscle. Thus, the Radial-EI NCS is added
its T1 fibers). to the routine motor NCS. If the screening ulnar
Thus, at this point, there is an axon loss lesion motor NCS is abnormal, the Ulnar-FDI NCS will
involving the medial cord or the lower plexus. be added to better assess lesion severity. Finally,
More accurate localization is not possible with any abnormal motor responses on the symptom-
the sensory NCS as they do not differentiate atic side will be compared to the contralateral
between medial cord and lower trunk localiza- side for severity assessment.

Case 2 Left Right
NCS Performed Stim site LAT AMP CV nAUC LAT AMP CV nAUC
Sensory
Median-D2 C6,7 X 30
Ulnar-D5 C8 X NR X 14
S-radial C6,7 X 21
MABC T1 X NR X 12
Motor
Median-APB X 4 X 13
4 X
Ulnar-ADM X 4 X 12
4 X
Ulnar-FDI X 5 X 9
5 X
Radial-EI X 1 X 4
1 X
the curve
180 M. A. Ferrante
The left Median-APB, Ulnar-ADM, Ulnar- C8-radial motor axons, when involved, are
FDI, and Radial-EI responses are severely helpful in localizing the lesion to the lower
reduced in amplitude, consistent with an axon plexus because they are never involved with
loss process (as already indicated by the sensory medial cord lesions. Importantly, their lack of
NCS) that is severe in degree (as suggested by the involvement does not indicate a medial cord
absent sensory NCS), and that involves the lower lesion because a partial lower plexus could be
plexus (since the Radial-EI response is affected). responsible.
The lesion involves 69% of the median motor • The extensor indicis and extensor pollicis bre-
axons to the APB muscle (1–4/13 = 0.69), 67% of vis are good C8-radial muscles. The extensor
the ulnar motor axons to the ADM muscle indicis is assessable by motor NCS and by
(1–0.33 = 0.67), 44% of the motor axons to the needle EMG, whereas the extensor pollicis
FDI muscle (1–5/9 = 0.44), and 75% of the motor brevis is usually studied by needle EMG.
axons to the EI muscle (1–1/4 = 0.75). • When the Radial-EI response is normal on
motor NCS, the EI and EPB should be studied
The Needle EMG Study on needle EMG because the needle EMG is
• Abnormal muscles: APB, FPL, FCU, FDP- more sensitive to axon loss than the motor
3,4, FDI, ADM, EIP, EPB NCS (especially in the 21- to 35-day window
• Normal muscles: Deltoid, biceps, triceps, bra- when fibrillation potentials are at their
chioradialis, pronator teres, paraspinals densest).
The needle EMG study shows involvement of

muscles in the muscle domain of the lower Exercise 3
plexus. The extensor indicis and the extensor pol-
licis brevis are both involved, consistent with the A 26-year-old female is referred for EDX assess-
lower plexus localization identified on motor ment of left upper extremity pain and numbness.
NCS. According to the patient, she has a several-year
history of intermittent numbness along the medial
EDX Conclusion aspect of her left forearm and hand. She reports
1. Lower plexopathy that the tingling can be precipitated by lying in a
supine position. She also reports a 10-year his-
The above is axon loss in nature, involves the tory of aching pain along the medial aspect of her
sensory and motor nerve fibers, and is severe in left arm and forearm.
degree. The distribution of the pain and tingling
along the medial aspect of the arm, forearm, and
Final Comments hand suggest C8 and T1 nerve fiber involve-
ment. To address this presentation, the screen-
• When the sensory NCS localize the lesion to ing sensory NCS are performed on the left upper
the medial cord or the lower plexus, the extremity.

Case 3 Left Right
Sensory
Median-D2 C6,7 X 51
Ulnar-D5 C8 X 16
S-radial C6,7 X 59
the curve
The screening sensory NCS are normal. normal (the lower limit of normal is 12 micro-
However, it is important to assess the relationship volts for a patient of this age), and that the ampli-
of the sensory responses to each other so as not to tude value of the superficial radial response is
miss a relative abnormality. Notice that the roughly 3.5 times the lower limit of normal (the
amplitude value of the median response is lower limit of normal is 17 microvolts for a
roughly 2.5 times the lower limit of normal (the patient of this age). Thus, the ulnar sensory
lower limit of normal is 20 microvolts for a response is questionably lower than the other two
patient of this age), that the amplitude value of responses. To address this, this NCS should be
the ulnar response is 1.25 times the lower limit of performed on the contralateral side.

Case 3 Left Right
Sensory
Median-D2 C6,7 X 51
Ulnar-D5 C8 X 16 X 41
S-radial C6,7 X 59
the curve
The contralateral ulnar response is roughly 3.5 nerve, the medial cord, or the C8 fibers of the
times the lower limit of normal, which is in line lower plexus. As discussed in Exercise 2, for
with the other responses. Moreover, the ampli- localization purposes, the MABC NCS is added.
tude value of the contralateral ulnar study is more Because this is not one of our routine screening
than twice that of the ipsilateral response, render- studies, we usually add it bilaterally, especially in
ing the ipsilateral response relatively abnormal. the setting of a relatively abnormal ulnar
The relatively abnormal left ulnar response response.
indicates an axon loss process involving the ulnar

Case 3 Left Right
Sensory
Median-D2 C6,7 X 51
S-radial C6,7 X 59
MABC T1 NR X 15
the curve
The absent MABC, when considered in the the MABC axons to a much greater extent than
setting of an abnormal ulnar response, limits the the ulnar axons, the other possibility is that the
potential lesion localization sites to the medial lesion is located in the proximal portion of the
cord or the lower plexus (both the C8 and T1 lower plexus (i.e., at the anterior primary ramus
fibers must be affected). level, where the C8 and T1 fibers are separate
It is important to notice the degree of involve- from each other). At this site, they could more
ment of the two abnormal responses—the ulnar readily be affected to varying degrees because the
response is relatively abnormal and the MABC majority of MABC fibers emanate from the T1
response is absent. Although this might represent DRG and the majority of the ulnar sensory fibers
a lower trunk lesion that happens to be affecting emanate from the C8 DRG [7].
182 M. A. Ferrante
Similar to Exercise 2, the C8-radial motor bilaterally to the routine motor NCS. Because the
axons can be useful in differentiating between ulnar sensory response was abnormal, both ulnar
medial cord and lower plexus lesion localiza- motor NCS are performed bilaterally.
tions. Thus, the Radial-EI motor NCS is added

Case 3 Left Right
NCS Performed LAT AMP CV nAUC LAT AMP CV nAUC
Motor Stim site
Median-APB Wrist X 2 X 12
Elbow 2 X
Ulnar-ADM Wrist X 12 X 14
AE 11 X
Ulnar-FDI Wrist X 10 X 15
AE 10 X
Radial-EI Forearm X 2 X 4
ACF 2 X
the curve
The median motor response is very low in also argues against a lower trunk localization
amplitude, indicating a severe axon loss process. and, instead, favors an APR-level lesion involv-
The two ulnar motor responses are normal, but ing the T1 APR to a greater extent than the C8
clearly lower than the contralateral side. However, APR. this is the typical pattern of NCS abnor-
they do not meet our criteria for relative abnormalities observed with true neurogenic thoracic
mal (i.e., less than 50% of the contralateral side). outlet syndrome (TN-TOS).
The amplitude value of the radial motor response For these reasons, the needle EMG is expanded
is abnormal. Its involvement excludes a medial to better evaluate the lower plexus.
cord localization. Thus, at this point, there is an
axon loss process involving the lower plexus. he Needle EMG Study
T
The degree of median motor response involve- • Abnormal muscles
ment (very severe) when considered in relation to –– APB (the most severely affected), FPL,
the degree of ulnar sensory nerve fiber involve- FDI, adductor pollicis, EI, EPB
ment (relatively abnormal) argues against a lesion • Normal muscles
involving a single element because, in general, –– Deltoid, brachioradialis, biceps, triceps,
when an element containing both sensory and pronator teres, FDP-3,4,
motor axons is affected, whenever the motor • paraspinals.
response from that element is more than 50%
reduced, the sensory response from that element The extensor indicis and extensor pollicis bre-
is typically absent or very low in amplitude. Thus, vis muscle are involved, confirming a lower
although a fascicular process is still possible, the plexus localization. Because the APB muscle was
lower trunk is a less likely localization site. the most severely affected and because it pre-
Finally, it is important to recognize that the dominantly receives motor axons from the T1
T1 > C8 pattern on sensory NCS (i.e., absent spinal cord segment, this pattern of needle EMG
MABC response; relatively abnormal Ulnar-D5 abnormalities, like the sensory NCS and the
response) is also present here (i.e., the APB mus- motor NCS, indicates that the severity of T1
cle is primarily innervated by motor axons nerve fiber involvement exceeds the severity of
derived from the T1 spinal cord segment). This the C8 nerve fiber involvement.
DX Study Impression
E • The T1 > C8 pattern of EDX abnormalities is
1. Left Lower Plexopathy (suspect true neuro- typically seen at all levels of the EDX study
genic thoracic outlet syndrome) –– Sensory NCS: MABC more affected than
Ulnar-D5
The above is axon loss in nature and severe in –– Motor NCS: Median-APB more affected
degree. The abnormalities localize to the lower than ulnar motor NCS
plexus and the pattern of abnormalities suggests –– Needle EMG: APB affected out of propor-
more proximal lower plexus involvement at the tion to the other muscles of the lower
level of the anterior primary rami. The constella- plexus muscle domain
tion of EDX abnormalities identified are essen- • Because this is a very slowly progressive
tially pathognomonic of true neurogenic thoracic process, maximum reinnervation has typi-
outlet syndrome. cally already occurred. Thus, surgical inter-
As you know, this disorder is a very slowly vention is required (i.e., there is no place for
progressive one that permits reinnervation to conservative treatment with this condition).
keep pace with denervation. For this reason, it The normal first thoracic rib is ideally left in
does not respond to conservative therapy and, place. A supraclavicular approach to section
hence, surgical intervention is the recommended the band is preferred over a transaxillary
treatment. This is best done by a neurosurgeon approach because the latter has been associ-
specializing in brachial plexus disorders using a ated with significant surgical complications
supraclavicular approach and leaving the normal [22].
first thoracic rib in place. Please contact my • At surgery, this patient was found to have a
office for recommendations. fibrocartilaginous band extending from her
first thoracic rib to a rudimentary C7 rib. The
Final Comments band deflected the T1 APR to a greater extent
than the C8 APR, as expected from the EDX
• Again, when the sensory NCS localize the findings.
lesion to the medial cord or lower plexus, the
C8-radial motor axons are useful for differen-
tiating these two sites. Exercise 4
• The pattern of T1 > C8 should be appreciated
because it is the pattern observed with true A 71-year-old male is referred for EDX assess-
neurogenic thoracic outlet syndrome, a disor- ment of a suspected postoperative left ulnar
der usually related to a taut fibrocartilaginous neuropathy. According to the patient, he noted
band that extends from the first thoracic rib to left grip weakness following open heart surgery
the C7 vertebral body (to either a C7 cervical 3–4 weeks ago. It is associated with numbness
rib or an elongated transverse process) and along the medial aspect of his left hand.
that displaces the lower plexus upward. The The distribution of the sensory symptoms
contact site is usually at the APR level of the implies a lesion along the pathway form the ulnar
brachial plexus and, therefore, the traction on nerve to the C8 root. This pathway could also
the more inferiorly located T1 APR is greater account for the grip weakness.
than that on the more superiorly located C8 To address this presentation, the screening
APR [21, 22]. sensory NCS are performed on the left.
184 M. A. Ferrante

Case 4 Left Right
Sensory
Median-D2 C6,7 X 14
Ulnar-D5 C8 NR
S-radial C6,7 X 18
the curve
The left ulnar response is absent, indicating an define this list of potential lesion sites, the MABC
axon loss process that is localized to the left ulnar NCS is added.
nerve, medial cord, or lower plexus. To better

Case 4 Left Right
Sensory
Median-D2 C6,7 X 14
Ulnar-D5 C8 NR
S-radial C6,7 X 18
MABC T1 X 11
the curve
The MABC sensory response is normal, argu- process, further NCS are required, including
ing against a medial cord or lower plexus process bilateral DUC and contralateral Ulnar-D5 and
involving the T1 fibers. To better localize this MABC NCS.
Case 4 Left Right
Sensory
Median-D2 C6,7 X 14
Ulnar-D5 C8 NR X 8
S-radial C6,7 X 18
MABC T1 X 11 X 12
DUC C8 NR X 7
the curve
The left Ulnar-D5 and DUC responses are At this point, an axon loss process has been
absent, indicative of an axon loss process that identified that involves the ulnar nerve proximal
localizes to the ulnar nerve, the medial cord, or to the wrist, the medial cord, or the C8 fibers of
the C8 APR. given that both ulnar responses are the lower plexus.
absent and the MABC response is normal, it is Similar to Exercise 2 and Exercise 3, the C8
less likely that this represents a partial lower radial motor axons should be included on the motor
trunk lesion. Involvement of the DUC response NCS (to help differentiate between ulnar nerve/
indicates that the lesion is proximal to the depar- medial cord and lower plexus localization. Also, the
ture site of this nerve (i.e., it is above the wrist). ulnar motor NCS should be performed bilaterally.

Case 4 Left Right
Motor Stim site
Median-APB 7 9
7
Ulnar-ADM 4 10
4
Ulnar-FDI 2 8
2
Radial-EI 1 3
the curve
The median motor response is normal. The DX Study Impression

E
ulnar motor responses are severely reduced in 1. Lower Plexopathy (median sternotomy bra-
amplitude, indicative of an axon loss process chial plexopathy)
involving the ulnar nerve, medial cord, or lower
plexus. Sparing of the MABC response argues The above is axon loss in nature and severe in
against a lower trunk process but does not exclude degree. The temporal relationship to the median
a C8 APR process because the MABC NCS is sternotomy and the pattern of EDX abnormalities
predominantly subserved by T1 DRG-derived (i.e., best localizing to the C8 anterior primary
sensory axons. ramus) strongly suggest that this represents
Because the Radial-EI response is reduced in median sternotomy brachial plexopathy, a disor-
amplitude, both an ulnar neuropathy and a medial der associated with the rib retraction required by
cord are excluded. Thus, the NCS indicate an surgical procedures requiring median sternotomy
axon loss process involving the lower plexus, (median sternotomy causes first thoracic rib-
most likely at the C8 APR level. related trauma of the C8 anterior primary ramus).
The needle EMG study of the left upper Although the majority of these lesions are pre-
extremity should be expanded to include addi- dominantly demyelinating conduction block
tional muscles in the muscle domain of the lower (about two-thirds), this lesion is predominantly
plexus. axon loss.
Final Comments
he Needle EMG Study
T
• Abnormal muscles • This case exemplifies the importance of adher-
–– FCU, FDP-3,4, ADM, FDI, FPL, EIP, ing to the admonition to always surround the
EPB abnormal studies with normal studies. Had the
• Normal muscles EDX provider mistakenly assumed that the
–– Deltoid, biceps, triceps, pronator teres, patient had a typical postoperative ulnar neu-
APB, paraspinals ropathy and only performed standard NCS
and needle EMG assessments to verify that
The involved muscles all belong to the muscle impression (i.e., not assessed the C8-radial
domain of the lower plexus. Again, the C8-radial motor axons), the patient would have been
muscles are extremely helpful in identifying misdiagnosed as having an ulnar neuropathy.
lower plexus pathology. Had an unnecessary ulnar transposition been
186 M. A. Ferrante
performed with resultant neurological wors- the patient, following pacemaker placement, she
ening, a medicolegal issue would be generated noted weakness of elbow flexion and pain and
(misdiagnosis with harm). numbness along the lateral aspect of the left fore-
• This particular pattern of NCS findings— arm. Examination shows an infraclavicular scar
involvement of the ulnar sensory and motor at the surgical site.
nerve fibers with sparing of the MABC sen- The sensory and motor symptoms following
sory and median motor nerve fibers—has long the surgical procedure suggest involvement of the
been associated with C8 anterior primary peripheral nervous system and the infraclavicular
ramus lesion related to median sternotomy. location of the scar suggest an infraclavicular
This entity is termed median sternotomy bra- lesion. Forearm flexion weakness implies muscu-
chial plexopathy [5, 6]. locutaneous nerve, lateral cord, or upper plexus
involvement. The numbness is in the cutaneous
distribution of the LABC nerve. Thus, this might
Exercise 5 represent a musculocutaneous neuropathy or a
lateral cord lesion, but is clinically unlikely to
A 56-year-old right hand dominant female is represent an upper plexus lesion.
referred for EDX assessment of left upper To address this presentation, the screening
extremity weakness and numbness. According to sensory NCS are performed on the left.

Case 5 Left Right
Sensory DRG
Median-D2 C6,7 X 14
Ulnar-D5 C8 X 22
S-radial C6,7 X 20
the curve
The Median-D2 response is abnormal. It is distribution of sensory and motor symptoms elic-
reduced in amplitude, indicative of an axon loss ited clinically.
process that localizes to the median nerve, lateral As stated previously, whenever the Median-D2
cord, or to the C6 or C7 fibers of the upper or response is reduced in amplitude, the LABC and
middle plexus. The presence of median sensory Median-D1 NCS are added bilaterally. In addi-
fibers excludes an isolated musculocutaneous tion, the Median-D2 and S-Radial NCS should be
nerve as a consideration, as was postulated by the performed on the contralateral side.

Case 5 Left Right
Sensory Drg
Median-D2 C6,7 X 8 32
Ulnar-D5 C8 X 22
S-radial C6,7 X 20 24
LABC C6 5 16
Median-D1 C6 12 28
the curve
The left LABC and Median-D1 responses are time and the upper plexus only 10% of the time
abnormal, indicative of an axon loss process [7]. In this individual, it was deferred given that
involving the lateral cord or the upper plexus. this issue would likely be resolved during the
Given that the sensory axons subserving the motor NCS and the needle EMG study.
Median-D2 NCS only traverse the upper plexus In addition to the routine motor NCS on the
20% of the time, the lateral cord is favored. In left, the Axillary-Deltoid and the
this setting, the Median-D3 NCS may also be Musculocutaneous-Biceps motor NCS were
helpful, as it assesses the lateral cord 80% of the added bilaterally.

Case 5 Left Right
Motor Stim site
Median-APB X 7
7 X
Ulnar-ADM X 8
8 X
Musculocut-biceps X 2 X 5
2 X
Axillary-deltoid X 9.2 X 8.6
the curve
The routine motor NCS are normal, as is the DX Study Impression

E
axillary motor response. The musculocutaneous 1. Lateral Cord Lesion
response is moderately to severely reduced. This
pattern of motor NCS abnormalities supports a The above is axon loss in nature, involves the
lateral cord localization, as was previously sug- sensory and motor nerve fibers, and is moderate-
gested by the sensory NCS. severe in degree.
The needle EMG study is expanded to include
muscles belonging to the upper plexus muscle Final Comments
domain that are not a part of the lateral cord mus-
cle domain. These include C5,6 muscles inner- • Unlike upper plexus lesions, with lateral cord
vated by the suprascapular (supraspinatus; lesions, the Median-D1 and the Median-D2
infraspinatus), axillary (deltoid; teres minor), and sensory responses tend to be more uniformly
radial (brachioradialis) nerves. affected (they are more uniformly affected
with upper plexus lesions). In addition, the
he Needle EMG Study
T radial sensory response is never affected by a
• Abnormal muscles lateral cord lesion (it is affected by upper
–– Biceps, pronator teres, FCR plexus lesions 60% of the time) [7].
• Normal muscles • With lateral cord lesions, the musculocutane-
–– Infraspinatus, deltoid, brachioradialis, FDI, ous motor response may be affected (depend-
EI, FPL, triceps, paraspinals ing on severity), but the axillary motor
response is always spared. With upper plexus
The abnormal muscles all belong to the mus- lesions, both of these responses may be
cle domain of the lateral cord. Sparing of. affected, depending on lesion severity [7].
the suprascapular, axillary, and radial nerve • On needle EMG, distinguishing between a lat-
innervated C5,6 muscles further support this. eral cord lesion and an upper plexus lesion is
localization. best done by studying C5,6-suprascapular
188 M. A. Ferrante
nerve, C5,6-axillary nerve, and C5,6-radial his arm over his shoulder level. He also reports left
nerve innervated muscles. hand numbness. He denies associated bulbar or
lower extremity involvement. He denies numbness
along the lateral aspect of the arm, proximally.
Exercise 6 The wrist and finger drop suggest radial nerve
distribution weakness. The shoulder abduction
A 31-year-old right hand dominant male is referred weakness suggests axillary nerve distribution
for EDX assessment of progressive left upper weakness. The distribution of the hand numbness
extremity weakness. According to the patient, he suggests superficial radial nerve involvement.
first noted left upper extremity weakness about Together, these features suggest posterior cord
12 years ago. The weakness initially produced a involvement. We begin the EDX study with
mild finger and wrist drop. Since then, it has screening sensory NCS on that side.
slowly worsened and he now can trouble raising Nerve Conduction Studies.
Case 6 Left Right
Sensory DRG
Median-D2 C6,7 32
Ulnar-D5 C8 24
S-radial C6,7 NR
the curve
The S-Radial response is absent, indicative of plexus localization cannot be excluded because
an axon loss process involving the S-Radial or the those fibers derive predominantly from the
radial nerve, the posterior cord, or the upper or C6 DRG 20% of the time [7]. Further localiza-
middle plexus because the sensory axons sub- tion requires additional sensory NCS. Thus, as
serving the S-Radial predominantly derive from discussed earlier, whenever the screening sensory
the C6 or the C7 DRG. The normal median NCS identify n abnormality involving a sensory
response argues against a middle plexus localiza- NCS whose axons derive from the C6 or C7
tion because the sensory fibers subserving the DRG, we bilaterally add the LABC and the
Median-D2 response derive from the C7 DRG Median-D1 NCS. In addition, the contralateral
about 80% of the time [7]. However, middle S-Radial NCS is added.
Case 6 Left Right
Sensory DRG
Median-D2 C6,7 32
Ulnar-D5 C8 24
S-radial C6,7 NR X 36
LABC C6 26 19
Median-D1 C6 25
the curve
Because the amplitude value of the Median-D1 Sparing of the LABC and the Median-D1 sen-
response was what would be expected when sory responses excludes an upper plexus localiza-
compared to the Median-D2 response (i.e., more tion. Thus, the potentials localization sites are the
than two-thirds the value of the Median-D2 superficial radial or radial nerve or the posterior
response), we did not add the contralateral cord. To hone this list further, the Axillary-
Median-D1 NCS for comparison purposes. We Deltoid motor NCS is added. Also, to better
did add the contralateral LABC NCS. define the severity of the lesion, the Radial-ED
motor NCS is added (and possibly the Radial-EI NCS include the upper plexus or the middle
NCS). For severity assessment, contralateral plexus as potential lesion localization sites, is not
comparison studies are also required. Because required. This response assesses the musculocu-
the upper plexus has been excluded as a possibil- taneous nerve, the lateral cord, and the upper
ity, the Musculocutaneous-Biceps motor NCS, plexus—none of which are lesion localization
previously included when the screening sensory considerations.

Case 6 Left Right
Motor Stim site
Median-APB X 18
17 X
Ulnar-ADM X 10
10 X
Radial-EDC X NR
X
Axillary-deltoid X NR
the curve
The radial and axillary motor responses are DX Study Impression

E
absent. For this reason, the contralateral motor 1. Left Posterior Cord Lesion
NCS are of no benefit in grading severity
(regardless of their value, the lesion involves The above is axon loss in nature, involves the
100% of the motor axons and is severe). Thus, sensory and motor nerve fibers, and is extremely
they were not required and, hence, not per- severe in degree. The relationship between the
formed. The motor response abnormalities are acute (sparse, low amplitude fibrillation poten-
consistent with a posterior cord localization, tials) and the chronic features (many long dura-
although a lesion involving both the axillary tion MUAPs) are indicative of a slowly
and the radial nerves near their site where they progressive process. As you know, imaging to
separate from each other (i.e., at the point exclude a structural process may be of further
where the posterior cord divides to become the diagnostic utility.
axillary nerve and the radial nerve) cannot be
excluded. Final Comments
The needle EMG study, in addition to the
screening muscles, is expanded to include • In the setting of posterior cord lesions, the
additional muscles within the muscle domain addition of the LABC and Median-D1 sensory
of the posterior cord, as well as one C5,6 mus- NCS is helpful in excluding a supraclavicular
cle within the upper plexus domain that is not process and thereby decreasing the list of
also in the posterior cord domain (e.g., the potential lesion localization sites to the poste-
infraspinatus). rior cord or the radial or superficial radial
nerve.
he Needle EMG Study
T • Following the sensory NCS, the motor NCS
• Abnormal muscles are helpful in differentiating a posterior cord
–– Deltoid, teres minor, triceps, brachioradia- lesion from one restricted to either the radial
lis, TC, ECR, ED, EI nerve or the axillary nerve.
• Normal muscles • On needle EMG examination, muscles should
–– Infraspinatus, biceps, pronator teres, FDI, be added to differentiate a posterior cord
FPL, paraspinals lesion from an upper plexus lesion (e.g., C5,6
190 M. A. Ferrante
long thoracic, dorsal scapular, or suprascapu- ness and atrophy. According to the patient, she
lar nerve innervated muscles, of which the first noted left hand weakness about 10 years ago.
infraspinatus is the easiest to assess). In addi- Since that time, it has slowly worsened but has
tion, muscles belonging to the axillary and not progressed proximally. More recently, she
radial nerve muscle domains are included to noted left axillary pain. The latter radiates dis-
differentiate a posterior cord from one involv- tally to the hand.
ing just one of its branches. Clinically, the axillary pain suggests that the
• Posterior cord lesions are differentiated from underlying etiology of the left hand weakness
middle plexus lesions by assessing C7 motor and atrophy is located proximally, possibly in the
axons that traverse the lateral cord (e.g., those axillary region. The distribution of the weakness
innervating the pronator teres and FCR (hand intrinsic muscle weakness) suggests C8
muscles). and T1 nerve fiber involvement. The clinical fea-
tures—the location of the pain and the distribu-
tion of the weakness—suggest possible medial
Exercise 7 cord or lower plexus involvement.
To address this presentation, screening sen-
A 32-year-old right hand dominant female was sory NCS of the left upper extremity are per-
referred for EDX assessment of left hand weak- formed first.

Case 7 Left Right
Sensory DRG
Median-D2 C6,7 X 38
Ulnar-D5 C8 X 4
S-radial C6,7 X 33
the curve
The low amplitude value of the Ulnar-D5 added bilaterally. Should it be normal, the DUC
response indicates an axon loss process involving NCS will be added bilaterally. For comparison
the ulnar nerve, the medial cord, or the C8 fibers purposes, the ulnar response is performed on the
of the lower plexus. To shorten this list of poten- contralateral side.
tial lesion localization sites, the MABC NCS is
Case 7 Left Right
Sensory DRG
Median-D2 C6,7 X 38
Ulnar-D5 C8 X 4 28
S-radial C6,7 X 33
MABC T1 NR 17
the curve
The absent MABC response eliminates the To address this, the screening motor NCS are
ulnar nerve from the list of possible lesion local- expanded to include the Radial-EI motor NCS
ization sites. Because the sensory response is bilaterally. Although radial motor response
nearly absent, we would expect the ulnar motor involvement localizes the process to the lower
response to be at least mildly involved. plexus, radial motor response sparing does not
localize the lesion to the medial cord because a added bilaterally (to better define lesion sever-
partial lower plexus lesion could also spare it ity). To better define lesion severity, those routine
(i.e., the radial motor response contributes to motor responses that are abnormal, will be com-
lesion localization when it is abnormal, but not pared to the contralateral side.
when it is normal). The Ulnar-FDI motor NCS is

Case 7 Left Right
Motor Stim site
Median-APB X 4 X 10
4 X
Ulnar-ADM X 5 X 11
5 X
Ulnar-FDI X 8 X 14
8 X
Radial-EI X 5 X 5
5 X
the curve
The amplitude values of the left Median- This pattern of abnormal muscles is most con-
APB response and the left Ulnar-ADM response sistent with a medial cord lesion (i.e., involvement
are reduced, consistent with an axon loss pro- of C8 and T1 median and ulnar nerve innervated
cess involving the medial cord or the lower muscles, with sparing of C8 radial nerve inner-
plexus. Although asymmetric. The two Ulnar- vated muscles). Of course, a partial lower trunk
FDI responses are normal because the ampli- lesion cannot be excluded with certainty.
tude value of the response is above the lower
limit of normal (i.e., not an absolute abnormal) DX Study Impression
E
and more than 50% of the contralateral response 1. Probable Medial Cord Lesion
value (i.e., not a relative abnormal). Still, the
asymmetry implies axon loss and, thus, the nee- The EDX study identifies a brachial plexopa-
dle EMG study of this muscle is expected to be thy that is axon loss in nature and that involves
abnormal. the sensory and motor nerve fibers. The distribu-
Again, sparing of the Radial-EIP response tion of the EDX abnormalities is most consistent
does not exclude a lower plexus localization (it with a medial cord localization, although a partial
may be a partial lower plexus lesion sparing the lower plexus lesion could produce a similar pat-
C8-radial nerve fibers) or rule in a medial cord tern and, hence, cannot be excluded.
localization.
The routine needle EMG study can now be Final Comments
performed, with special attention to C8 radial Differentiation between lower plexus and medial
nerve innervated muscles (EI; EPB) and the FDI cord lesions is best accomplished by studying the
(to look for axon loss). C8-radial motor axons. Although lesions involv-
ing the lower plexus may affect them, lesions
he Needle EMG Study
T involving the medial cord never do. Thus,
• Abnormal muscles: FCU, FDI, ADM, FPL, although their involvement excludes a medial
APB, FPL cord localization, the converse statement—that
• Normal muscles: Deltoid, triceps, biceps, pro- C8-radial muscle sparing localizes the lesion to
nator teres, EI, EPB, paraspinals the medial cord—is inaccurate.
192 M. A. Ferrante
Exercise 8 C6-derived motor axons (biceps; brachioradialis)

and C8,T1-derived motor axons (thenar emi-
A 17-year-old male violinist is referred for EDX nence muscles). No single PNS element could
assessment of left upper extremity numbness and account for this distribution. Although it is pos-
weakness. According to the patient, he first noted sible that the thenar eminence atrophy is congeni-
muscle wasting involving the left thenar emi- tal, the patient is certain that it was not present
nence about 1 year ago. At that time, he also prior to 1 year ago.
noted numbness along the lateral aspect of his On examination, it would be helpful to sepa-
left forearm and thumb. On examination by the rately assess the dorsal (superficial radial nerve,
referring physician, there was also biceps and radial nerve, posterior cord distribution) and ven-
brachioradialis weakness. Based on the distribu- tral (median nerve, lateral cord distribution)
tion of the weakness and the numbness, a tenta- aspects of the thumb to determine if a single
tive diagnosis of left C6 radiculopathy had been region or both regions (upper plexus distribution)
provided by the referring physician. are involved. This was done, but the information
Clinically, the sensory loss involves is not being provided because we have enough
C6-derived axons, so the list of possible lesions information to move forward with the EDX study.
sites includes the lateral cord and the upper The screening sensory NCS are performed on
plexus, whereas the weakness involves C5- or the symptomatic (left) limb.

Case 8 Left Right
Sensory DRG
Median-D2 C6,7 X 7
Ulnar-D5 C8 X 14
S-radial C6,7 X 21
the curve
The amplitude response value of the As always, because the median screening
Median-D2 response indicates an axon loss lesion response is abnormal, the LABC and median-D1
involving the median nerve, the lateral cord, or the sensory NCS are added bilaterally. The contralat-
upper or middle plexus. The normal radial eral Median-D2 is also added. Although the
response argues against a supraclavicular local- radial and ulnar responses are normal, in a
ization but, because the sensory fibers subserving 17-year-old the amplitude values could have been
the Median-D2 and S-Radial sensory NCS could much higher. Thus, they are also added. In addi-
theoretically emanate from either the C6 DRG or tion, we need to assess the C8 fibers because of
the C7 DRG (i.e., one could be affected in isola- the thenar eminence muscle weakness and
tion), a supraclavicular site cannot be excluded. atrophy.

Case 8 Left Right
Sensory DRG
Median-D2 C6,7 X 7 X 24
LABC C6 X 4 X 17
Median-D1 C6 X 6 X 18
the curve
The left LABC and Median-D1 responses are To address this list of possibilities, the
reduced in amplitude, arguing for a lateral cord or Musculocutaneous-Biceps and Axillary-Deltoid
upper plexus localization. Sparing of the left motor NCS are added bilaterally and the contra-
radial response does not differentiate between lateral Median-APB motor NCS is added to
these two possibilities as it could be spared with address the thenar muscle atrophy.
both if the sensory axons subserving it predomi-
nantly emanate from the C7 DRG.

Case 8 Left Right
Motor Stim site
Median-APB Wrist X 2 X 8
Elbow 2 X 8 X
Ulnar-ADM Wrist X 8 X 9
Elbow 8 X 9 X
Musculocutaneous-BC Axilla X 4 X 8
SCF 4 X 8 X
Axillary-deltoid SCF X 14 X 12
the curve
The amplitude value of the median motor he Needle EMG Study

T
response is reduced, indicating an axon loss pro- • Abnormal muscles
cess involving the median nerve, the medial cord, –– Biceps, brachialis, pronator teres, FCR,
or the lower plexus. However, the medial cord and FPL, APB
lower plexus localizations are excluded by the • Normal muscles
normal Ulnar-D5 sensory response, leaving only –– Infraspinatus, deltoid, triceps, brachioradi-
the median nerve as an explanation. The low alis, EI, FDI, paraspinals
amplitude Musculocutaneous-Biceps response
indicates an axon loss process involving the mus- The needle EMG shows abnormalities in the
culocutaneous nerve, the lateral cord, or the upper distribution of the lateral cord (biceps; brachia-
plexus. Sparing of the Axillary-Deltoid response lis; pronator teres) and the terminal median
argues against an upper plexus localization. nerve (APB, FPL, pronator teres, FCR). Note
Thus, the sensory NCS argues for a lateral that the brachioradialis is normal (it was
cord lesion (but cannot exclude upper plexus reported to be weak by the referring
with certainty) and the motor NCS argue for a physician).
lateral cord and median nerve lesion (but cannot
exclude upper plexus with certainty. Given that DX Study Impression
E
we are seeking a single focus that could account 1. Probable Lateral Cord Brachial Plexopathy
for all of the abnormalities, and given that the lat- with extension into the Median Terminal
eral cord and the terminal median nerve are adja- Nerve
cent structures, a lateral cord lesion with
extension into the terminal median nerve is sug- The above is axon loss and best localizes to
gested. Thus, on needle EMG, we will need to the lateral cord with extension into the terminal
look at the lateral cord and median nerve muscle median nerve. Based on the motor responses, the
domains closely (as well as the upper plexus lesion is severe in degree. An MRI study of the
muscle domain given that the needle EMG study brachial plexus may be of further diagnostic util-
is more sensitive to motor axon loss than are the ity. Evidence of brachioradialis involvement was
motor NCS). not noted.
194 M. A. Ferrante
Final Comments 7. Ferrante MA, Wilbourn AJ. The utility of various sen-
sory nerve conduction responses in assessing brachial
plexopathies. Muscle Nerve. 1995;18:879–89.
• Magnetic resonance imaging of the brachial 8. Bonnel F. Microscopic anatomy of the adult human
plexus disclosed a mass involving the lateral cord brachial plexus: an anatomical and histological basis
that, as predicted by the EDX study, extended for microsurgery. Microsurgery. 1984;5:107–17.
9. Slingluff CL Jr, Terzis JK, Edgerton MT. The quan-
into the terminal median nerve. At surgery, the titative microanatomy of the brachial plexus in
lesion extended from the proximal aspect of the man: reconstructive relevance. In: Terzis JK, editor.
lateral cord (proximal to the departure site of the Microreconstruction of nerve injuries. Philadelphia:
musculocutaneous nerve) to the proximal aspect WB Saunders; 1987. p. 285–324.
10. Wolock B, Millesi H: Brachial plexus: applied
of the terminal median nerve. Pathology showed anatomy and operative exposure. In Gelberman
the lesion to be a perineurioma. RH, editor. Operative nerve repair and reconstruc-
• The strength of the brachioradialis muscle is tion, volume 2. Philadelphia: Lippincott; 1991;
not easily assessed. Even when the forearm is p 1255–1272.
11. Wilbourn AJ. Brachial plexus lesions. In: Dyck
oriented in the neutral position (i.e., forearm PJ, Thomas PK, editors. Peripheral neuropathy.
pronation so that the thumb is superior), the 4th ed. Philadelphia: Elsevier Saunders; 2005.
biceps and brachialis are the predominant fore- p. 1339–73.
arm flexors. Moreover, in this case, the biceps 12. Swash M. Diagnosis of brachial root and plexus
lesions. J Neurol. 1986;233:131–5.
and brachialis muscles are both affected. 13. Leffert RD. Brachial plexus injuries. London:
• The author assesses the brachioradialis muscle Churchill Livingstone; 1985.
by having the patient first position both fore- 14. Hollinshead WH. Anatomy for surgeons, vol 3: the
arms in their neutral position and flexed against back and limbs. 3rd. ed. Philadelphia: Harper & Row;
1982.
the examiner’s forearm, which is held perpen- 15. Ferrante MA. The management of periph-
dicular to the patient’s forearms. The examiner eral nerve trauma. Curr Treat Options Neurol.
then uses his other hand to palpate the con- 2018 Jun 1;20(7):25. https://doi.org/10.1007/
tracting brachioradialis muscles of the two s11940-018-0507-4).
16. Brunelli GA, Brunelli GR. A fourth type of bra-
sides for differences in bulk and consistency. chial plexus injury: middle lesion (C7). Ital J Orthop
In this individually, the brachioradialis mus- Traumatol. 1992;18:389–93.
cles of the two sides had identical features. 17. Maggiano H, Levin KH, Wilbourn AJ. Relationship
between the medial antebrachial cutaneous sensory
and median motor responses in brachial plexopathies
(abstract). Muscle Nerve. 1993;16:1113–4.
18. Nishida T, Price SJ, Minieka MM. Medial ante-
References brachial cutaneous nerve conduction in true neuro-
genic thoracic outlet syndrome. Electromyogr Clin
1. Stewart JD. Focal peripheral neuropathies. 2nd ed. Neurophysiol. 1993;33:285–8.
New York: Raven Press; 1993. p. 506. 19. Walsh JF. The anatomy of the brachial plexus. Am J
2. Wilbourn AJ. Brachial plexus disorders. In: Dyck PJ, Med Sci. 1877;74:387–98.
Thomas PK, editors. Peripheral neuropathy, vol. 2. 20. Gilliatt RW. Thoracic outlet syndromes. In: Dyck PJ,
3rd ed. Philadelphia: WB Saunders; 1993. p. 911–50. Thomas PK, Lambert EH, Bunge R, editors. Peripheral
3. Ferrante MA. Brachial plexopathies: classifica- neuropathy. 2nd ed. Philadelphia: Saunders; 1984.
tion, causes, and consequences. Muscle Nerve. p. 1409–24.
2004;30:547–68. 21. Tsao BE, Ferrante MA, Wilbourn AJ, Shields RW
4. Ferrante MA. Comprehensive electromyography: Jr. Electrodiagnostic features of true neurogenic tho-
with clinical correlations and case studies. New York: racic outlet syndrome. Muscle Nerve. 2014;49:724–7.
Cambridge University Press; 2018. https://doi.org/10.1002/mus.24066.
5. Ferrante MA, Tsao BE. Brachial plexopathies. 22. Ferrante MA, Ferrante ND. The thoracic outlet
In: Katirji B, Kaminski HJ, Ruff RL, editors. syndromes: part 1. Overview of the thoracic outlet
Neuromuscular disorders in clinical practice, vol. 2. syndromes and review of true neurogenic thoracic
2nd ed. New York: Springer; 2014. p. 1029–62. outlet. Muscle Nerve. 2017;55:782–93. https://doi.
6. Ferrante MA. Brachial plexopathies. Continuum. org/10.1002/mus.25536.
2014;20:1323–42.
Radiculopathies
8
Etiology tes mellitus, or spinal tumors. Below is a more

extensive list of the causes of radiculopathies.
There are several causes of radiculopathy, how- Causes of Radiculopathy:
ever, the vast majority of radiculopathies are com-
pressive in etiology. Typically, in patients younger • Compression: Disc herniation, degenerative
than 40–50 years of age, nerve root compression spine disease/spondylosis [4, 5]
occurs from disc protrusion. Whereas, in patients • Vascular: Vasculitis (e.g. in diabetes mellitus),
older than 40–50 years of age, nerve root compres- epidural/subdural hematomas, dural fistulae/
sion is due to bony impingement from spondylo- arteriovenous malformations, spinal epidural
sis. Compressive radiculopathy is more commonly cavernous hemangiomas [3, 5]
seen in cervical and lumbosacral regions. Lumbar • Infections: e.g. Tuberculosis, mycoplasma, her-
spinal stenosis most commonly affects L4-5 fol- pes zoster virus (HZV), herpes simplex virus
lowed by L3-4, L5-S1 and L1-2 in descending (HSV), cytomegalovirus (CMV), Epstein-Barr
order [1]. The likelihood of nerve root compres- virus (EBV), Lyme, syphilis, HIV [6–10]
sion by disc rupture at the lumbosacral levels is • Iatrogenic: including complication of spinal
increased by extrathecal dural and foraminal liga- anesthesia [11], spinal cord stimulator place-
ments that anchor nerve roots and reduce plasticity ment [12]
[2, 3]. Radiculopathy in the thoracic region due to • Trauma: e.g. Root avulsion, fracture of verte-
disc protrusion is uncommon (this region benefits bral body [13]
from less range of movements, especially given • Toxic: e.g. Procainamide polyradiculoneurop-
the effects of ribs) and is more likely to be second- athy [14]
ary to local infection such as herpes zoster, diabe- • Autoimmune: e.g. Chronic Inflammatory
Demyelinating Polyradiculoneuropathy
The original version of this chapter was revised. A
(CIDP) [15]
correction to this chapter can be found at
https://doi.org/10.1007/978-3-030-74997-2_14 • Metabolic: e.g. Diabetic polyradiculopathy,
adrenal insufficiency [16]
K. A. Karwa (*) • Inflammatory: e.g. Sarcoidosis [17–19]
Neurology of the Rockies, Englewood, Colorado, USA • Mass (non-neoplastic): e.g. Epidural abscess,
J. A. Morren spinal cysts [20–22]
Neuromuscular Center, Neurological Institute, • Neoplastic: Epidural/vertebral metastases, lep-
Cleveland Clinic, and Cleveland Clinic Lerner tomeningeal metastases, malignant angiotrophic
College of Medicine of Case Western Reserve lymphoma; benign such as meningioma, epen-
e-mail: morrenj@ccf.org dymoma, schwanommas, [23–25]
© Springer Nature Switzerland AG 2021, corrected publication 2022 195
https://doi.org/10.1007/978-3-030-74997-2_8
196 K. A. Karwa and J. A. Morren
• Radiation at the T1-2 vertebral level, and the L4 nerve root

• Neurodegenerative: Amyotrophic lateral scle- exits at L4-5 vertebral level. At lumbosacral lev-
rosis (involvement may extend beyond ante- els, all lumbar and sacral spinal nerve roots are
rior horn cell proper), juvenile amyotrophy constituted at the T12-L1 vertebral level, where
[26] the spinal cord ends as the conus medullaris. The
roots then course down the canal as the cauda
equina, until they exit at their respective neural
Anatomy foramina. Depending on the nature and location
of intraspinal compression, roots may be injured
Neural foramina are formed between each pair of at any disk level, from the L1-2 level to the level
vertebral bodies and are bounded superiorly and of their exit through the neural foramen. For
inferiorly by pedicles, anteriorly by interverte- example, the L5 root can be compressed by a
bral disc and vertebral body, and posteriorly by central disk protrusion at the L2-3 or L3-4 level,
facet joint.(See Fig. 8.1) [3]. Through the neural a posterolateral disk protrusion at the L4-5 level,
foramina pass the spinal nerve roots, recurrent or foraminal stenosis at the L5-S1 level. (Figs. 8.2
meningeal nerves and radicular blood vessels. and 8.3) [3, 27].
The blood supply to the nerve roots arises from a The cell bodies of the motor nerve roots are
capillary network derived from radicular arteries located in the anterior horn of the spinal cord
[3]. There are 31 pairs of spinal roots: 8 cervical, (anterior horn cells), and are therefore within the
12 thoracic, 5 lumbar, 5 sacral, 1 coccygeal. The spinal canal, whereas the sensory dorsal root gan-
number designation of the exiting cervical spinal glion (DRG) is typically located outside the spi-
root corresponds with the number of the vertebral nal canal proper. The DRG is in a protected
body below it, for example the C3 nerve root position within the neural foramen. This location
exits the spinal canal at C2-3 neural foraminal is of significance as nerve conduction studies
level. The cervical spinal column is comprised of demonstrate preservation of sensory nerve action
7 vertebral bodies, as a result at the C7-T1 inter- potentials (SNAP) in radiculopathies. However,
vertebral level, the exiting nerve root is numbered there are cadaveric and radiographic (including
C8. Thus all thoracic, lumbar and sacral nerve magnetic resonance imaging studies) that
roots exit below the vertebral body of the same demonstrate about 11%-38% of L5 DRG and 3%
number [3]. For example the T1 nerve root exits of L3 and L4 DRG are intraspinal canal in loca-
Fig. 8.1 Diagram of

cross section of the
spine at the cervical Ligamentum flavum
level [3]
Dura
Dorsal root Arachnoid
ganglion Pia
Dorsal ramus Superior articular

facet
Ventral ramus
Posterior CCF
Nucleus pulposis ©2000
long lig.
Anterior Annulus fibrosis
long lig.
8 Radiculopathies 197
Fig. 8.3 Far lateral disc herniation compresses the nerve

Fig. 8.2 Posterolateral disc herniation compresses the root exiting foramen through foramen at that vertebral
nerve root descending to exit the intervertebral foramen level (ie. L4-5 disc compressing the L4 nerve root) [27]
one level below corresponding vertebrae (i.e L4 -5 disc
herniation compresses L5 nerve root) [27]
(i.e. the dermatomal distribution). They are also
associated with sensory loss and weakness fol-
tion [28, 29]. In addition, the C5 and C6 DRG lowing the distribution of the nerve root. Due to
also have a tendency to reside in intraspinal loca- significant dermatomal overlap dense numbness
tions [30]. Intraspinal location of DRG can result is uncommon and is more classically seen in a
in degeneration of sensory nerves and loss of peripheral nerve lesion rather than radiculopathy.
SNAP on nerve conduction studies which can Similarly, weakness can be seen in muscles sup-
present diagnostic dilemmas in the case of plied by the same nerve root, however, it is very
radiculopathies. unusual for a radiculopathy to result in complete
Mild injury to the nerve root may result in muscle paralysis due to myotomal overlap.
focal demyelination leading to conduction block Conduction block and axon loss only produce
or conduction velocity slowing along nerve root symptoms and neurologic deficits if a sufficient
fibers. Axon loss at the root level results in wal- number of nerve fibers are affected. Conduction
lerian degeneration along the entire course of velocity slowing alone is insufficient to produce
affected nerve fibers [3]. significant weakness or sensory loss, although
sensory modalities requiring timed volleys of
impulse transmission along their pathways, such
Clinical Features as vibration and proprioception, can be altered
[3]. Deep tendon reflexes may be absent depend-
Radiculopathies present clinically as pain and/or ing on the nerve root involved in the muscle ten-
paresthesia originating from the spine and gener- don reflex [31]. Involvement of a single nerve
ally following the distribution of the nerve root root is termed monoradiculopathy, whereas mul-
tiple, usually contiguous nerve root level involve- presenting similar to C5 and other half similar to
ment is termed polyradiculopathy. C7 radiculopathies [32].
C7 Radiculopathy: Typically presents with
pain in neck, shoulder, middle finger and hand;
Cervical Radiculopathies numbness typically seen in index, middle finger,
palm. Weakness is classically seen in C7 inner-
The clinical diagnosis of cervical radiculopathy vated muscles—elbow and wrist extension, fore-
is supported by neck or shoulder pain radiating arm pronation.
into the arm. Cervical radiculopathies involving An important part of the clinical diagnosis of
the C7 nerve root is most common, while pure T1 C7 radiculopathy rests on the finding of a dimin-
radiculopathy occurs the least commonly [32]. ished triceps deep tendon reflex, but several stud-
Provocative tests such as Spurling’s test (neck ies have shown that the reflex is abnormal in less
compression maneuver or foraminal compression than 70% of patients [32, 34].
test) and relief tests such as shoulder abduction C8 Radiculopathy: Typically presents with
relief sign have been used at the bedside to assist pain in shoulder, medial forearm, fourth and fifth
with diagnosis of cervical radiculopathy. One digits, medial hand; numbness typically seen in
study reviewed various provocative and relief medial forearm, fourth and fifth digits, medial
signs and reported that for roots C6-8 neck com- hand. Weakness is classically seen in C8 inner-
pression maneuvers had a specificity of 100% for vated muscles—finger and wrist extension, distal
radicular pain and 92-100% for neurological and finger flexion, finger abduction and adduction.
radiological signs, respectively. For shoulder There can be loss of triceps reflex.
abduction relief sign, the specificity was 100% T1 radiculopathy: Typically presents with pain
for neurological signs and 80% for radiological in medial arm, medial forearm, axillary chest
signs. The sensitivity of these tests for radicular wall; numbness typically seen in medial forearm,
symptoms, neurological signs and radiological fourth and fifth digits, medial hand. Weakness is
signs varied from 40–60% [33]. classically seen in T1 innervated muscles—thumb
There are classical clinical attributes in soli- abduction, distal thumb flexion, finger abduction
tary cervical root lesions which are detailed and adduction. T1 radiculopathy is the most
below: uncommon isolated root lesion affecting the arm.
C5 Radiculopathy: Typically presents with Although all C8 muscles of the hand are said to
neck or shoulder pain, with numbness generally have some T1 contributions, the abductor pollicis
following axillary nerve distribution. Weakness brevis muscle seems to be the only muscle with
is classically seen in C5 innervated muscles— predominantly T1 innervation [32, 35].
shoulder abduction, external rotation, elbow flex-
ion, forearm supination. There can be loss of
biceps and brachioradialis reflex. Thoracic Radiculopathies
C6 Radiculopathy: Typically presents with
pain in neck, shoulder, lateral upper arm, lateral The classic presentation of thoracic radiculopa-
forearm, thumb and lateral hand; numbness typi- thies involve pain and paresthesias radiating from
cally seen in lateral forearm, thumb and index the posterior thorax in the dermatomal distribu-
fingers. Weakness is classically seen in C6 inner- tion of the involved nerve root. Sensory loss may
vated muscles—shoulder abduction, external be present only in a portion of the involved der-
rotation, elbow flexion, forearm supination and matome. Muscle weakness is often subtle and
pronation. There can be loss of biceps and bra- can be demonstrated by having patient cough or
chioradialis reflex. C6 radiculopathies have the attempt a sit up which may result in a bulging
most variable presentation with half of the cases intercostal or abdominal muscle [5].
Lumbosacral Radiculopathies gastrocnemius, soleus and gluteus maximus.

The ankle reflex may be absent or reduced [5].
Lumbosacral radiculopathies typically present as The biceps femoris short head, long head and
pain and/or paresthesias radiating from lower medial gastrocnemius are near exclusively
back into the lower limbs. Symptoms are often innervated by the S1 root although some stud-
worsened by Valsalva maneuvers, straight leg ies do suggest significant L5 innervation [3,
raising test and reverse straight leg raising test. 37–39].
However, straight leg raising test and reverse S2-4 Radiculopathy: Typically occurs as a
straight leg raising are not quite specific and may polyradiculopathy. Typically presents with pain
be seen in lumbosacral plexopathies, femoral and/or paresthesias and/or loss of sensation in the
neuropathies and diseases of hip joint [5]. Lumbar perineal and medial buttocks and is associated
disc herniation leading to electromyographically- with urinary and fecal incontinence. There can be
determined motor radiculopathy occurs at L4-5, absence of anal wink and bulbocavernosus
L5-S1 and L3-4 levels 55%,43% and 2% of the reflexes [5].
time respectively [3, 36].
L1 radiculopathy: Extremely uncommon.
Typically presents with pain and/or paresthesias Differential Diagnosis of Cervical
and/or loss of sensation in inguinal region. No Radiculopathy
corresponding muscle weakness in the lower
limb, or loss of reflexes [5]. 1. Neuralgic Amyotrophy: This can often pres-
L2 Radiculopathy: Very uncommon. Typically ent similar to an acute cervical radiculopathy.
presents with pain and/or paresthesias and/or loss Classically patient presents with debilitating
of sensation in anterolateral thigh with weakness pain in shoulder or scapula that is followed by
of hip flexors [5]. progressive weakness. The motor component
L3 Radiculopathy: More likely to occur than can involve spinal accessory, suprascapular,
L1 or L2 however, less common than L4-S1 lev- long thoracic, axillary, and musculocutaneous
els. Typically presents with pain and/or paresthe- nerves. Most patient recover although atrophy
sias and/or loss of sensation in medial thigh or and muscle weakness may persist. This condi-
knee with weakness of hip flexors, quadriceps, tion can be hard to differentiate from a C5-6
and adductors. The knee jerk may be reduced or radiculopathy [4, 40].
absent [5]. 2. Rotator cuff tear: Typically results in weak-
L4 Radiculopathy: Disc herniation is fairly ness of shoulder abductor, external rotation
common at this level. Typically presents with and can be associated with suprascapular
pain and/or paresthesias and/or loss of sensation mononeuropathy [41].
in the medial leg with weakness of quadriceps, 3. Amyotrophic lateral sclerosis: May initially
hip adductors, occasionally tibialis anterior. The present as a radiculopathy (although radicular
knee jerk is often reduced or absent [5]. pain typically absent) especially if predomi-
L5 Radiculopathy: Typically presents with nant C8-T1 muscle involvement (the weak-
pain and/or paresthesias and/or loss of sensation ness and atrophy may produce the so-called
in lateral lower leg and great toe with weakness “split-hand pattern”).
including that of the tibialis anterior, extensor 4. Multifocal motor neuropathy: Early on, when
hallucis, ankle invertors and evertors, and gluteus an isolated nerve trunk is involved it may also
medius. There is no specific deep tendon reflex mimic involvement of an isolated nerve root
abnormality [5]. [4].
S1 Radiculopathy: Typically presents with 5. Neurogenic thoracic outlet syndrome:
pain and/or paresthesias and/or loss of sensa- Classically involves T1> > C8 nerve root with
tion in the sole/plantar aspect, lateral aspect of severe axon loss in the abductor pollicis brevis
the foot and lateral three toes with weakness of muscle and medial antebrachial SNAP [42].
6. Median sternotomy brachial plexopathy: if the DRG is involved in the pathologic process
Typically occurs in the setting of coronary such as with a variant intraspinal location of
artery bypass graft and cardiac valve repair DRG. In patients with radiculopathy, nerve con-
surgeries (operations requiring sternotomy) duction studies are typically normal (unless
with severe axon loss in C8> > T1 nerve root lesion is severe/has marked motor axon loss) and
[42]. electrodiagnosis is primarily established by nee-
7. Ulnar mononeuropathy: Can present similar dle electrode examination (NEE) [31]. There can
to C8-T1 radiculopathy. However, weakness be abnormalities in routine motor nerve conduc-
is limited to ulnar innervated muscles with tion studies especially in C8-T1 and L5-S1 with
sparing of median and radial innervated significant axon loss. Late responses include
C8-T1 muscles [43]. F-waves and H-reflex. The absence of motor
F-wave, in the presence of normal motor nerve
conduction is suggestive of recent/acute demye-
ifferential Diagnosis of Lumbosacral
D lination, classically seen in acute demyelinating
Radiculopathies neuropathies however, this finding may also be
seen in an isolated motor radiculopathy.
1. Ilioinguinal and genitofemoral neuropathy: Abnormalities in F-waves are not specific and
Can present similar to L1 radiculopathy [44] can be seen in several disorders and alone is
2. Lateral femoral cutaneous neuropathy: Can insufficient to make an electrodiagnosis of radic-
present similar to L2 radiculopathy, however ulopathy. An evidence-based review demon-
classically a pure sensory neuropathy with no strated the peroneal (fibular) and tibial motor
associated weakness [44]. F-waves have a low sensitivity in diagnosis of
3. Femoral neuropathy: Can present similar to lumbosacral radiculopathy [45]. Although the
L2/L3/L4 radiculopathy. However, classically H-reflex is the electrodiagnostic equivalent of the
only femoral innervated muscles are weak ankle deep tendon reflex, in normal individuals
with essentially preserved strength in hip there may be a discordance between presence of
abductors/adductors [44]. the ankle reflex and ability to elicit the H reflex
4. Common peroneal (fibular) neuropathy: Can [46]. The tibial H-reflex is routinely used in clini-
present similar to L5 radiculopathy, however, cal practice and is a very sensitive test for tibial
there is preservation of ankle inversion which S1 sensory pathway including intraspinal course
is typically weak in a L5 radiculopathy [44]. of S1 nerve root [3]. In the H-reflex electrical
5. Sciatic neuropathy: Can present similar to mul- stimulus travels orthodromically along 1a affer-
tilevel lumbosacralradiculopathy, but glutei ents to the spinal cord activating the motor neu-
muscles are spared in sciatic neuropathy [44]. ron in the same segment and resulting in a
6. Tibial neuropathy: Can present similar to S1 peripheral motor response [47, 48]. The H-reflex
radiculopathy, but hamstrings and glutei mus- is absent or low in 80% patients with surgically
cles are spared in tibial neuropathy [44]. proven S1 radiculopathy [49].
Theoretically, somatosensory evoked poten-
tials (SEPs) should be a valuable tool in the
Electrodiagnostic Evaluation assessment of conduction abnormalities along
sensory fibers at the root level. Electrical stimuli
Nerve Conduction Studies are delivered on the skin surface to a mixed sen-
sory and motor nerve trunk, a sensory nerve
Nerve conduction studies are an essential part of trunk, or the skin in a specific dermatomal distri-
the work up of patients with suspected radicu- bution. Responses are recorded over the spine
lopathies. Due to the intraspinal location of the and scalp, and latencies are measured to assess
DRG, the sensory nerve responses are classically the conduction time along large-diameter sensory
preserved. The SNAP amplitude may be affected fibers across various segments of the peripheral
and central conduction pathways primarily sub- The Needle Electrode Examination
serving proprioception and vibratory sense [3].
Unfortunately, a number of limitations diminish NEE is the most specific and sensitive electrodiag-
the value of this technique including variable nostic test for the identification of axon loss radicu-
amplitude measurements in normal individuals; lopathy. NEE can identify one or more of the
activation of nerve fibers belonging to more than following abnormalities: increased insertional
one root segment, time consuming procedure and activity (usually in the form of unsustained positive
subject to technical artifacts [50]. Given these sharp waves just after needle movement); further
limitations, SEPs obtained from nerve trunk evidence of active/ongoing motor axon loss (typi-
stimulation have been shown to add little diag- cally in the form of fibrillation potentials); reduced
nostic value [51, 52]. (“neurogenic”) recruitment pattern of motor unit
Tables 8.1 and 8.2 demonstrate “screening” firing; and features of chronic motor unit action
nerve conduction studies that can be performed potential (MUAP) reinnervation, such as increased
to evaluate cervical and lumbosacral radiculopa- duration, increased amplitude, and polyphasia [3].
thies, respectively. The NEE can help localize nerve root involvement
by identifying motor axon loss changes in at least
Table 8.1 Nerve conduction studies evaluating for cervi- two muscles that share the same nerve root how-
cal radiculopathy ever, involve separate peripheral nerve distributions.
Nerve When a single nerve root is localized, muscles of
Nerve Recording Site Root surrounding myotomes must be examined to rule
Median sensory Index/digit 2 C6-7 out additional nerve root involvement.
Median sensory Digit 3 C7 Corresponding paraspinal muscle evaluation should
Ulnar sensory Digit 5 C8 be routinely performed as it is very helpful in ruling
Radial sensory Snuffbox C5-C6 out generalized polyneuropathy or plexopathy (also
Lateral antebrachial Lateral forearm C5-6
the first muscles to be affected early in the process).
cutaneous
Medial antebrachial Medial forearm T1
NEE can also determine degree of active/ongoing
cutaneous and chronic motor axon loss, thereby establishing
Median motor Abductor pollicis brevis T1 the chronicity of the process. In patients with non-
Ulnar motor Abductor digiti minimi C8-T1 specific arm or leg paresthesias, screening of several
Radial motor Extensor digitorum C7-8 muscles can be performed to help identify an under-
(communis) lying radiculopathy. Tables 8.3 and 8.4 outline the
Musculocutaneous Biceps C5-6
motor
“screening” NEE for radiculopathy [3].
Axillary Deltoid C5-6 Anatomic, clinical, and electromyographic
myotomal charts are used to correlate the pattern
Table 8.2 Nerve conduction studies to evaluate for lum- of EMG abnormalities in a limb with a specific
bosacral radiculopathy root level involvement. Anatomical and clinical
Nerve charts although useful, are not entirely applicable
Nerve Recording Site Root to the NEE. Muscles are chosen for the NEE
Sural sensory Ankle S1 because of specific attributes of root innervation
Superficial peroneal Ankle L5 and accessibility.
(fibular) sensory
NEE can easily isolate muscles that are diffi-
Saphenous sensory Ankle L4
Peroneal (fibular) Extensor digitorum L5 cult to examine clinically and with assistance of
motor brevis (-S1) myotomal chart, play a key role in root localiza-
Tibial motor Abductor Hallucis S1 tion. Thus, electromyographically derived myoto-
Peroneal (fibular) Tibialis anterior (L4-)L5 mal charts are quite useful in the electrodiagnosis
motor of radiculopathy [3, 32, 49, 53]. Tables 8.5 and 8.6
Femoral motor Rectus Femoris L3-4
demonstrate myotomal innervation patterns in
H-reflex Soleus S1
upper and lower extremities respectively [54].
Table 8.3 Screening muscles to evaluate cervical radicu- Table 8.4 Screening muscles to evaluate lumbosacral
lopathy* [3] radiculopathy* [3]
Muscle Nerve Root Nerve Trunk Nerve
First dorsal C8(−T1) Ulnar Muscle Root Nerve Trunk
Interosseus Abductor Hallucis S1 Tibial
Abductor (C8-)T1 Median Medial gastrocnemius S1 Tibial
pollicis brevis Biceps Femoris, short S1 Peroneal
Flexor Pollicis C8(−T1) Anterior Interosseus head (fibular)
longus (median) division of
Extensor Indicis C8 Posterior Interosseus sciatic
Proprius (radial) Extensor digitorum L5 (S1) Peroneal
Pronator Teres C6-7 Median brevis (fibular) (deep
Triceps C6-7(-C8) Radial branch)
Biceps C5-6 Musculocutaneous Tibialis anterior (L4) L5 Peroneal
Deltoid C5-6 Axillary (fibular) (deep
branch)
C7 Paraspinal Overlap
Tibialis posterior (or L5 Tibial
flexor Digitorum longus)
Gluteus Medius L5 (S1) Superior
gluteal
Rectus Femoris (L2)L3-4 Femoral
S1 Paraspinal Overlap
Table 8.5 Myotomal Inner ANTERIOR PRIMARY RAMI C5 C6 C7 C8 T1

vatation Upper Extremity* PROXIMAL NERVES
[54] Rhomboid major/minor (dorsal scapular)
Supraspinatus (suprascapular)
Infraspinatus (suprascapular)
Deltoid (axillary)
Biceps brachii (musculocutaneous)
Brachialis (musculocutaneous)
RADIAL NERVE
Triceps
Anconeus
Brachioradialis
Extensor Carpi Radialis
Extensor Pollicis Brevis
Extensor Indicis Proprius
MEDIAN NERVE
Pronator Teres
Flexor Carpi Radialis
Flexor Pollicis Longus
Pronator Quadratus
Abductor Pollicis Brevis
ULNAR NERVE
Flexor Carpi ulnaris
Flexor digitorum Profundus Dig 4,5
Abductor Digiti Minimi
Adductor Pollics
First Dorsal Interosseus
Table 8.6 Myotomal Inner ANTERIOR PRIMARY RAMI L2 L3 L4 L5 S1 S2

vation Lower extremity [54] PROXIMAL NERVES
Iliacus (lumbar plexus-femoral)
Adductor Longus (obturator)
Vastus Lateralis (femoral)
Rectus Femoris (femoral)
Tensor Fascia Lata (superior gluteal)
Gluteus Medius (superior gluteal)
Gluteus Maximus (inferior gluteal)
SCIATIC NERVE
Semitendinosus/Semimembranosus
(tibialdivision of sciatic)
Biceps Femoris, shorthead (peroneal
(fibular) division of sciatic)
Biceps Femoris long head (tibial
division of sciatic)
PERONEAL (FIBULAR) NERVE

Tibialis Anterior
Extensor Hallucis
Peroneus Longus
Extensor Digitorum Brevis
TIBIAL NERVE
Tibialis Posterior
Flexor Digitorum Longus
Gastrocnemius, lateral
Gastrocnemius, medial
Soleus
Abductor Hallucis
Abductor Digiti Quinti Pedis
Usually dominant contribution

Sometimes significant contribution
Minor/equivocal contribution
Determining the Age tional spontaneous activity in the form of

of a Radiculopathy fibrillation potentials indicates a process at
There are characteristic electrophysiological least ~3 weeks of age (may be earlier in proxi-
changes that occur within the muscle in the set- mal limb and paraspinal muscles).
ting of conduction block and axon loss injury B. Acute Demyelinating Radiculopathy: There is
which help identify the age of a radiculopathy. evidence of a prominent conduction block
Table 8.7 summarizes the typical changes in axon lesion at the root level as the cause for weak-
loss radiculopathy. ness. In these cases, CMAP (with stimulation
point distal to the root-level conduction block)
A. Acute Axonal radiculopathy: Motor unit is of normal amplitude and NEE of the muscle
potentials are of normal configuration and demonstrates reduced recruitment pattern,
size, the presence of abnormal insertional or normal motor unit configuration/morphology
spontaneous activity in the form of trains of and the absence of positive sharp wave and
brief sharp spikes or positive waves indicates fibrillation potentials (which may be collec-
recent motor axon loss. Increased insertional tively referred to as “denervation potentials”).
activity alone suggests that the process may be The corresponding late responses (F-wave
only several weeks old. The presence of addi- and/or H-reflex responses) may be prolonged
Table 8.7 Findings in Needle Electrode Examination at progressive stages of axon loss radiculopathy * [3]
Reduced Recruitment Insertion PSP Fib Poly/Var Neuro CRD
<3 week ++ +/++ + − − − −
3-6 week ++ ++ ++ +++ − − −
6-26 week ++ + +/− ++ +++ − −
Chronic/active ++ − +/− + ++ ++ −
Chronic/remote +/++ − − − − +++ +
Abbreviations: Fib fibrillation potentials in myotomal limb muscles; Insertion abnormal insertional activity in myoto-
mal muscles; CRD complex repetitive discharges; Neuro neurogenic motor unit potential changes (increased duration
and amplitude); Poly/Var polyphasic motor unit potential changes/motor unit potential variation/variability; PSP para-
spinal muscle fibrillation potentials; −negligible/equivocal amount; +, mild amount; ++, moderate amount; +++, great-
est amount [3]
or absent. Ttogether, these findings suggests Determining the Severity

conduction block. If this pattern is seen in mul- of a Radiculopathy
tiple muscles of a specific myotome, a diagno- The severity of an axon loss is based on the degree of
sis of radiculopathy can be made. This strategy motor unit loss in the specific root distribution. This
is not reliable for the diagnosis of conduction is determined by a subjective/semi-objective mea-
block if the onset of weakness is less than surement of the degree of reduced recruitment of
~4 weeks before the electrodiagnostic study, motor unit potentials. Of note, reduced recruitment
because an acute axon loss lesion may share (without definite MUAP morphological changes) is
these features, including not clearly manifest- not necessarily caused by axon loss unless the
ing denervation potentials for ~3 or more CMAP elicited from the same muscle is also reduced
weeks after onset of symptoms [3]. in amplitude (although, less commonly, this may
C. Chronic-active Radiculopathy: NEE demon- also result from distal conduction blocks). Thus,
strates neurogenic MUAP with chronic defining the severity of an axon loss radiculopathy
reinnervation changes along with consider- requires evaluation of the CMAPs in the myotome in
abledenervation potentials. In root distribu- question (when possible) and the degree of reduced
tions where the myotome includes muscles recruitment of MUAPs. Quantifying the degree of
in distal and proximal regions of a limb, the denervation potentials does not correlate as well
presence of a chronic and ongoing axon loss with the degree of axon loss [3].
process can be even more clearly defined
when fibrillation potentials are seen in distal
and proximal muscles in the root distribu- Electrodiagnostic Pitfalls
tion. In lesions where fibrillation potentials and Limitations
are seen in distal muscles only, the presence
of an ongoing axon loss process is less cer- The value of electrodiagnostic studies in estab-
tain. Some inactive but severe axon loss pro- lishing a diagnosis of radiculopathy is highly
cesses never fully reinnervate, especially in variable and depends on patient selection, nerve
muscles farthest from the injury site, leaving root involved and study used for electrodiagnos-
some muscle fibers denervated indefinitely. tic testing [3, 55–57].
D. Chronic-remote Radiculopathy: NEE demon-
strates neurogenic MUAP with chronic reinner-
vation changes, in the absence of denervation imitations of Nerve Conduction
L
potentials. These changes in motor unit action Studies in Diagnosis of Radiculopathy
potentials are permanent, reflecting the histo-
pathologic changes in the reinnervated muscle, There are several limitations to the use of nerve
and remain unchanged unless the motor unit is conduction studies in the diagnosis of radiculop-
injured again. After a significant motor axon athy. Nerve conduction studies are more useful to
loss process has occurred, MUAPs never return exclude other clinical conditions mimicking
to their preinjury morphology. radiculopathies.
1. In most radiculopathies, typically there is belly from which the CMAP is generated
damage only to a portion of nerve root fibers, must be in the myotomal distribution of the
thus there may not be significant axon loss to injured root. Illustratively, this is as follows:
result in abnormalities in (motor) nerve con- A severe C8 radiculopathy is expected to
duction studies. produce some change in the ulnar CMAP
2. Early in the course of a radiculopathy patients amplitude, recording from either the abduc-
present with pain/paresthesia. tor digiti minimi or the first dorsal interos-
Sensory radiculopathy can only rarely be seus. In the C5 myotome, the
reliably localized segmentally by electrodiag- musculocutaneous and axillary nerve trunks
nostic techniques. Pain is primarily mediated can be stimulated to assess CMAPs from the
through C-type sensory fibers that are too small biceps and deltoid muscles, respectively.
to be studied by routine electrodiagnostic tech- However, muscles in the C6 and C7 myoto-
niques, and because the peripheral processes of mal distributions are not well spatially iso-
sensory root fibers remain intact with intraspi- lated from muscles of other myotomes, and
nal lesions, SNAPs typical remain normal. The therefore CMAPs derived from them are
intraspinal location of most lesions makes it generally less reliable [3].
impossible to perform direct nerve conduction
studies on the nerve root proximal to the dam-
aged segment, precluding the diagnosis of con- imitations of Needle Examination
L
duction block or focal conduction velocity in Diagnosis of Radiculopathy
slowing along the damaged segment.
3. Sensory NCS performed along peripheral Although needle examination plays a key role in
nerve trunks are characteristically normal in the diagnosis of radiculopathy, there are several
radiculopathy. When DRG reside in an intra- limitations that may pose diagnostic challenges.
spinal location they become vulnerable to
compression/injury by disk protrusion and 1. Most muscles are innervated by more than
spondylosis (or other intraspinal canal patho- one myotome making it challenging to iso-
logical process). L5 radiculopathy can uncom- late a single root involvement. For example,
monly be associated with loss of the superficial L2, L3, and L4 root lesions cannot be reli-
peroneal (fibular) SNAP [58]. However, an ably distinguished from each other because
intraspinal location of DRG alone does not if the overlap of innervation of the anterior
result in abnormal SNAP, for example S1 (and medial) thigh muscles.
radiculopathy is almost never associated with 2. The problem in reliable localization is com-
sural SNAP amplitude loss. Although S1 DRG pounded if there is absence of proximal and
are even more commonly intraspinal than L5 distal muscles to examine for example C5-6
DRG, their intraspinal location is caudal to the and L2-4 radiculopathies.
L5-S1 disk space where most compressive S1 3. Reinnervation occurs in proximal muscles
radiculopathies occur. When nerve root dam- before distal muscles, in chronic radiculopa-
age occurs distal to the neural foramen, SNAP thies there may be residual denervation only
amplitude is affected [3, 58, 59]. in distal muscles making it challenging to
4. Motor nerve conduction are often normal in differentiate from distal neuropathy espe-
radiculopathies as typically a loss of close to cially in older individuals when sensory
50% of motor axons in a nerve trunk is required responses can be absent.
to reliably establish a significant reduction in 4. Electrodiagnostic localization of a specific root
the compound muscle action potential (CMAP) lesion does not specify the vertebral level of
amplitude compared with the same response on damage/injury in lumbosacral radiculopathies.
the uninvolved side [60]. 5. NEE can be normal in acute lesions and mild
5. To identify an abnormality of CMAP ampli- demyelinating radiculopathy. In acute demy-
tude in a motor radiculopathy, the muscle elinating radiculopathy abnormalities are
only seen if associated with prominent con- these patients, 55% had an EMG abnormality and
duction block. 57% had an MRI abnormality that correlated with
6. NEE cannot identify a pure sensory the clinically estimated level of radiculopathy.
radiculopathy. The two studies agreed in 60% of patients,
7. Fascicular sparing can result patchy involve- with normal in 11 and abnormal in 17; however,
ment of muscles within the same myotome only one study was abnormal in a significant
and often requires sampling of several mus- minority (40%), suggesting that the two studies
cles to increase yield of study. were complementary diagnostic modalities. The
8. Paraspinal muscle denervation potentials are agreement was higher in patients with abnormal
not specific to radiculopathies and can also findings on neurologic examination [63].
be seen in processes affecting anterior horn
cells and in muscle disorders, such as necro-
tizing myopathy. Case Studies
9. Paraspinal muscle involvement cannot pre-
cisely localize the segmental level of root Case 1: A 48 year-old right-handed woman was
damage because the segmental innervation referred for an EMG/NCS to evaluate for a possible
of paraspinal muscles can overlap by as lumbosacral radiculopathy. Patient typically exer-
much as four to six segments [61]. cises regularly at the gym. Approximately 4 weeks
10. Clear evidence of paraspinal denervation with ago she was lifting weights when she experienced
cervical and lumbosacral radiculopathies is sudden onset back pain radiating down into her
seen only in approximately 50% of cases due right leg. On neurological examination she appears
to overlapping segmental innervation of para- to have positive straight leg raising test on lifting
spinal muscles and the tendency for muscles her right leg up to 45 degrees. On formal muscle
close to the site of the nerve lesion to reinner- strength testing she had normal strength in her left
vate sooner and more completely than muscles leg, her right leg appeared to be strong proximally,
at greater distance from the point where nerve ankle and toe dorsiflexion, foot inversion and ever-
regeneration must begin [3, 32, 49]. sion was approximately 4+/5 as per Medical
11. In paraspinal muscles close to a prior lami- Research Council (MRC) grading scale. She had
nectomy/similar spinal surgery site, denerva- difficulty standing on heels with relative preserva-
tion potentials may persist indefinitely tion of standing on toes. Sensory exam reveals
because of iatrogenic denervation [3]. subtle decreased sensation over dorsum of right
foot to pinprick and all deep tendon reflexes are
For cervical radiculopathy, a systematic preserved.
evidence- based literature review concluded that Approach to electrodiagnostic evaluation: In
needle EMG examination provided confirmatory this case, history and physical examination is
evidence of cervical root pathology in 30% to 72% classic for of a lumbosacral radiculopathy involv-
of patients presenting with appropriate symptoms ing the right L5 nerve root. Also on the differen-
or signs. Needle EMG abnormalities were highly tial is a proximal lesion of the sciatic nerve or
correlated with weakness. Good agreement between lower lumbosacral plexus. In order to reach elec-
imaging studies and needle EMG was seen in 65% trodiagnosis of a lumbosacral radiculopathy, one
to 85% of cases [62]. For lumbosacral radiculopa- must (typically) obtain normal sensory nerve
thy, an evidence-based review concluded that nee- responses with L5 and S1 nerve root thus ruling
dle EMG of the limb is probably effective in clinical out a sciatic mononeuropathy and plexopathy.
diagnosis (class II evidence) [45]. Needle examination must sample muscles from
One study retrospectively analyzed 47 patients several nerve roots, in addition, it is important to
with a clinical history compatible with either cer- sample L5-S1 muscles that are supplied by dif-
vical or lumbosacral radiculopathy who were eval- ferent nerves. The summary of her electrodiag-
uated with an EMG and a spine MRI. Among nostic study findings is presented in Table 8.8.
8
Sensory nerve conductions
Amplitude (μV) Peak Lat (ms) Normal values
Nerve stimulated Stimulation site Recording site L R L R Amp (μV) Peak Lat (msec) Temperature (°C)
Sural Posterior leg Lateral malleolus 8.45 3.3 >4 <4 31.2
Superficial peroneal (fibular) Lateral leg Dorsum of foot 6.20 3.6 >4 <4 31.5
Radiculopathies
Motor nerve conductions

Amplitude (mV) Onset Lat (ms) Cond Vel (m/s) Distance (mm) Normal values F-wave Lat (ms)
Nerve Stimulation Recording L R L R L R L R Amp (mV) Distal Lat Cond Vel L R Temperature
stimulated site site (msec) (m/s) (°C)
Peroneal Ankle EDB 5.26 4.00 N/A 70 >4 <6 > 40 31.7
(fibular) Pop fossa 4.54 13.00 48.9 440
Tibial Ankle AH 11.44 4.00 N/A 80 >5 <5.9 > 40 30.8
Pop fossa 8.30 13.00 48.9 440
Peroneal Fib Head TA 4.50 2.60 N/A 80 >3 <4.1 > 40 31.0
(fibular) Pop fossa 4.02 4.30 58.8 100
Tibial Ankle AH <66 57.5 31.2
Tibial H-reflex Soleus 1.50 1.60 32.5 33.80 >1 <35 31.5
M response 7.26 7.16 5.0 6.00 >7 <7
Needle EMG
Insertion Spontaneous activity Motor unit action potentials
Muscle Activity Fibs PSW Fasc Other Rec pattern Duration Amp Polys Descript
L tibialis anterior Normal 0 0 0 Normal Normal Normal None NC
R. lumbar 1 PSP Normal 0 0 0
R sacral 1 PSP Normal 2+ 0 0 Normal Normal Normal None NC
R gluteus medius Normal 0 2+ 0 Rapid, Reduced 2- Normal Normal None NC
R gluteus maximus Normal 0 0 0 Normal Normal Normal None NC
R rectus femoris Normal 0 0 0 Normal Normal Normal None NC
R semitendinosus Normal 0 2+ 0 Rapid, Reduced 2- Normal Normal None NC
R biceps femoris, short head Normal 0 0 0 Normal Normal Normal None NC
R tibialis anterior Normal 2+ 0 0 Rapid, Rapid, Reduced 3- Normal Normal None NC
R extensor hallucis longus Normal 1+ 0 0 Rapid, Reduced 2- Few 1+ Normal None NC
R medial gastrocnemius Normal 0 0 0 Normal Normal Normal None NC
R flexor digitorum longus Normal 0 0 0 Rapid, Reduced 3- Few 1+ Normal None NC
Abbreviations: μV microvolt; mV millivolt; msec milliseconds; m/s meter/second; mm millimeter; L left; R right; mV millivolt; Lat Latency; Cond Vel Conduction Velocity; Sup
207
Peroneal (fibular) Superficial Peroneal (fibular); Pop Popliteal; EDB Extensor Digitorum Brevis; AH Abductor Hallucis; TA Tibialis Anterior; Fib fibrillation potentials; PSW
positive sharp waves; Fasc fasciculations; Rec Pattern Recruitment pattern; Amp Amplitude; Polys polyphasic motor unit potential changes; PSP paraspinal muscle; NC no com-
ment; Descript description; fibrillation potentials: 1+ mild amount; 2+ moderate amount; 3+ great amount
Interpretation of electrodiagnostic studies: extensors, thumb abductors. Examination is

somewhat limited due to pain.
1. Right sural and superficial sensory nerve con- Approach to electrodiagnostic evaluation: In
duction studies are within normal limits which this case, the history may suggest a possible right
would rule out a generalized large fiber poly- ulnar mononeuropathy at the elbow, however, on
neuropathy or a lumbosacral plexopathy (or exam patient appears to have diffuse hand muscle
multiple mononeuropathies). weakness including median and radial innervated
2. Motor nerve conduction studies including hand muscles. Electrodiagnostic studies should be
right peroneal (fibular) recording from exten- performed to primarily differentiate between right
sor digitorum brevis (EDB) and tibialis ante- ulnar mononeuropathy, right (lower trunk) bra-
rior (TA) muscles and tibial nerve recording chial plexopathy and right C7-T1 radiculopathy.
from abductor hallucis (AH) muscle are The summary of his electrodiagnostic study find-
within normal limits. Tibial H-reflex is with- ings is presented in Table 8.9.
out definite abnormality bilaterally. Interpretation of electrodiagnostic studies:
3. Extensive needle examination performed in
right lower extremity with additional study in 1. Sensory nerve conduction studies including
left lower extremity reveals significant active/ right median (digit II) and bilateral ulnar (digit
ongoing motor axon loss changes in the form of V), dorsal ulnar cutaneous and medial ante-
fibrillation and positive sharp wave potentials brachial cutaneous nerves are within normal
and/or neurogenic recruitment pattern in several limits which would rule out a brachial plexop-
muscles including right gluteus medius, semi- athy, or multiple mononeuropathies.
tendinosus, tibialis anterior, extensor hallucis 2. Motor nerve conduction studies reveal normal
and S1 paraspinal muscles. MUAP morphology right median recording from abductor pollicis
is essentially normal in the muscle sampled brevis (APB) muscle and bilateral ulnar motor
throughout. Of note, although the S1 paraspinal nerves recording from first dorsal interosseous
muscle reveals fibrillations, all of the muscles (FDI) as well as Abductor digiti minimi (ADM)
with associated denervation potentials are pre- muscles including proximal stimulations up to
dominantly L5 innervated muscles and one must Erb’s point. Bilateral ulnar F-wave distal laten-
keep in mind that there is significant overlap in cies are within normal limits. No evidence of
innervation of paraspinal muscles. Abnormalities conduction block or other demyelination fea-
in paraspinal muscles have greater value in local- tures along bilateral ulnar motor nerves.
izing lesion at or proximal to the root rather than 3. On extensive needle examination performed
specific root involvement. in right upper extremity with additional stud-
ies in left upper extremity, there appears to be
Taken together these findings are supportive neurogenic (motor axon loss-related) changes
of a subacute intraspinal canal lesion involving of different ages/chronicities in the muscles
the right L5 nerve root, axon loss in type and at studied. For example, there appears to be
least moderate in degree electrically. mixed active/ongoing on chronic motor axon
Case 2: A 66 year-old right-handed man loss changes involving right FDI, APB, flexor
presents for an EMG referral with a long- digitorum profundus (to digits 4,5), and flexor
standing history of neck pain who, over the carpi ulnaris, with predominantly chronic
past 3 months, has been experiencing pain, tin- motor axon loss changes involving right tri-
gling, numbness travelling from right elbow ceps, pronator teres, ADM, flexor pollicis lon-
into digits III, IV, V. On neurological examina- gus, extensor indicis (proprius) and extensor
tion patient appears to have diffuse dysesthetic digitorum (communis) muscles.
sensory perception distortion along the right
forearm and hand, but no classic dermatomal Taken together these findings are most con-
sensory loss pattern. He appears to have mild sistent with chronic intraspinal canal lesions
decreased strength (4+/5 MRC grading) in involving the right C7-T1 nerve roots/segments,
right hand muscles specifically finger flexors, axon loss in type, overall moderate-to-severe in
8
Sensory nerve conductions
Amplitude (μV) Peak Lat (ms) Normal values
Nerve stimulated Stimulation site Recording site L R L R Amp (μV) Peak Lat (msec) Temperature (°C)
Median Wrist Digit II 24.66 3.0 >10 <3.8 33.0
Ulnar Wrist Digit V 20.21 22.95 3.0 2.7 >5 <3.2 33.1
Radiculopathies
Radial Forearm Snuffbox 21.73 2.2 >10 <2.8 33.7

Ulnar Wrist Dorsal hand 10.26 8.45 2.8 2.6 >5 <3.2 33.6
Medial antebrachial cutaneous Proximal Elbow Forearm 7.18 7.23 2.6 2.6 >5 <3.2 34.2
Motor nerve conductions
Amplitude (mV) Onset Lat (ms) Cond Vel (m/s) Distance (mm) Normal values F-wave Lat (ms)
Nerve Stimulation Recording Amp Distal Lat Cond Vel
stimulated site site L R L R L R L R (mV) (msec) (m/s) L R Temperature (°C)
Median Wrist APB 11.48 3.1 N/A 50 >5 <4.0 >50 34.0
Elbow 10.72 8.3 55.8 290
Ulnar Wrist FDI 9.27 9.50 3.00 3.00 N/A N/A 210 210 >7 <4.5 >50 33.7
Dist elbow 9.05 9.31 6.80 6.90 55.3 53.8 310 310
Prox elbow 8.00 8.42 9.00 8.80 51.7 53.4
Ulnar Wrist ADM 9.92 9.80 2.25 2.00 N/A N/A 50 50 >7 <3.1 >50 34.2
Dist elbow 9.03 9.11 5.80 5.65 59.2 57.5 210 210
Prox elbow 8.81 8.90 8.15 8.00 52.5 51.7 310 310
Axilla 8.57 8.61 10.95 10.60 51.7 52.3 450670 450
Erb’s point 8.05 8.10 14.00 13.75 57.0 57.0 670
Ulnar Wrist ADM <34 29.65 29.90 33.6
(continured)
209
210
Needle EMG
Insertion Spontaneous activity Motor unit activity
Muscle Activity Fibs PSW Fasc Other Rec pattern Duration Amp Polys Descript
L triceps Normal 0 0 0 Normal Normal Normal None NC
L flexor pollicis longus Normal 0 0 0 Normal Normal Normal None NC
L first dorsal interosseus Normal 0 0 0 Normal Normal Normal None NC
R cervical 7 PSP Normal 0 0 0 Normal Normal Normal None NC
R deltoid Normal 0 0 0 Normal Normal Normal None NC
R biceps Normal 0 0 0 Normal Normal Normal None NC
R triceps Normal 0 0+ 0 Rapid, Reduced 2- Many 2+ Many 1+ Many 1+ NC
R extensor digitorum Normal 0 0 0 Rapid, Reduced 3- Many 2+ Many 2+ None NC
communis
R extensor indicus Normal 0 0+ 0+ Rapid, Reduced 2- Many 1+ Few 1+ Some 1+ NC
proprius
R pronator teres Normal 0 0+ 0 Rapid, Reduced 2- Some 1+ Few 1+ None NC
R flexor pollicis longus Normal 0 0+ 0 Rapid, Reduced 2- Some 1+ Some 1+ None NC
R flexor carpi ulnaris Normal 1+ 0 1+ Rapid, Reduced 3- Most1+ Most 1+ None NC
R flexor digitorum Normal 1+ 2+ 0 Rapid, Reduced 3- Many 1+ Some 1+ None NC
profundus 4,5
R abductor pollicis brevis Normal 0 2+ 0 Rapid, Reduced 2- Some 1+ Some 1+ None NC
R abductor digiti minimi Normal 0 0 0+ Rapid, Reduced 2- Many 1+ Some 1+ None NC
R first dorsal interosseus Normal 0 1+ 1+ Rapid, Reduced 3- Many 2+ Many 2+ Some 1+ NC
R thoracic 1 PSP Normal 0 0 0 Normal Normal Normal None pain-
limited
Abbreviations: μV microvolt; mV millivolt; msec milliseconds; m/s meter/second; mm millimeter; L left; R right; mV millivolt; Lat Latency; Cond Vel Conduction Velocity; APB
Abductor pollicis brevis muscle; FDI First Dorsal Interosseus muscle; ADM Abductor Digiti minimi; Fib fibrillation potentials; PSW positive sharp waves; Fasc fasciculations;
Rec Pattern. Recruitment pattern; Amp Amplitude; Polys polyphasic motor unit potential changes; PSP paraspinal muscle; NC no comment; Descript description; fibrillation
potentials: 1+ mild amount; 2+ moderate amount; 3+ great amount
K. A. Karwa and J. A. Morren
degree electrically, and with a superimposed 17. Atkinson R, Ghelman B, Tsairis P, Warren RF, Jacobs
B, Lavyne M. Sarcoidosis presenting as cervical
subacute component mostly in the C8-T1 radiculopathy: a case report and literature review.
distributions. Spine. 1982 Oct;7(5):412–6.
18. Vargas DL, Stern BJ. Neurosarcoidosis: diagnosis
and management. Semin Respir Crit Care Med. 2010
Aug;31(4):419–27.
References 19. Koffman B, Junck L, Elias SB, Feit HW, Levine
SR. Polyradiculopathy in sarcoidosis. Muscle Nerve.
1. Binder DK, Schmidt MH, Weinstein PR. Lumbar 1999 May;22(5):608–13.
spinal stenosis. Semin Neurol. 2002 Jun;22(2):157–66. 20. Schutta H. Spinal tumors. In: Joynt RG, editor.
2. Spencer DL, Irwin GS, Miller JA. Anatomy and sig- Clinical neurology. Philadelphia: Lippincott Williams
nificance of fixation of the lumbosacral nerve roots in & Wilkins; 1994. p. 1–126.
sciatica. Spine. 1983 Sep;8(6):672–9. 21. Hlavin ML, Kaminski HJ, Fenstermaker RA, White
3. Levin KH. Approach to the patient with sus- RJ. Intracranial suppuration: a modern decade
pected radiculopathy. Neurol Clin. 2012 May of postoperative subdural empyema and epidural
1;30(2):581–604. abscess. Neurosurgery. 1994;34(6):974–80. discus-
4. Levin KH. Cervical radiculopathy. In: Neuromuscular sion 980-981
disorders in clinical practice. 2nd ed. New York: 22. Schutta H. Diseases of the dura mater. In: Joynt RG,
Springer; 2014. p. 981–1000. editor. Clinical neurology. Philadelphia: Lippincott
5. Raynor E, Baruchow S, Nardin R, Kleiner-Fisman Williams & Wilkins; 1993. p. 67–91.
G. Lumbosacral and thoracic radiculopathies. In: 23. Black P. Spinal metastasis: current status and recom-
Neuromuscular disorders in Clinical Practice. 2nd ed. mended guidelines for management. Neurosurgery.
New York: Springer; 2014. p. 1001–28. 1979 Dec;5(6):726–46.
6. Thomas JE, Howard FM. Segmental zoster paresis--a 24. Taylor JW, Schiff D. Metastatic epidural spinal cord
disease profile. Neurology. 1972 May;22(5):459–66. compression. Semin Neurol. 2010 Jul;30(3):245–53.
7. So YT, Olney RK. Acute lumbosacral polyradiculopa- 25. Engelhard HH, Villano JL, Porter KR, Stewart AK,
thy in acquired immunodeficiency syndrome: experi- Barua M, Barker FG, et al. Clinical presentation, his-
ence in 23 patients. Ann Neurol. 1994 Jan;35(1):53–8. tology, and treatment in 430 patients with primary
8. Logigian EL. Peripheral nervous system Lyme bor- tumors of the spinal cord, spinal meninges, or cauda
reliosis. Semin Neurol. 1997 Mar;17(1):25–30. equina. J Neurosurg Spine. 2010 Jul;13(1):67–77.
9. Masjuan J, Corral I, Fernández-Ruiz 26. Hirayama K, Tomonaga M, Kitano K, Yamada T,
LC. Mycobacterial acute lumbosacral polyradicu- Kojima S, Arai K. Focal cervical poliopathy causing
lopathy as the initial manifestation of AIDS. J Acquir juvenile muscular atrophy of distal upper extremity:
Immune Defic Syndr Hum Retrovirology Off Publ Int a pathological study. J Neurol Neurosurg Psychiatry.
Retrovirology Assoc. 1997 Jun 1;15(2):175. 1987 Mar;50(3):285–90.
10. Corral I, Quereda C, Casado JL, Cobo J, Navas 27. Hardy R, Plank N. Clinical diagnosis of herniated
E, Pérez-Elías MJ, et al. Acute polyradiculopa- lumbar disc. In: Lumbar Disc Disease. New York:
thies in HIV-infected patients. J Neurol. 1997 Raven Press; 1982. p. 17–28.
Aug;244(8):499–504. 28. Hamanishi C, Tanaka S. Dorsal root ganglia in the
11. Yuen EC, Layzer RB, Weitz SR, Olney RK. Neurologic lumbosacral region observed from the axial views of
complications of lumbar epidural anesthesia and anal- MRI. Spine. 1993 Oct;18(13):1753–6.
gesia. Neurology. 1995 Oct;45(10):1795–801. 29. Kikuchi S, Sato K, Konno S, Hasue M. Anatomic and
12. Mammis A, Bonsignore C, Mogilner AY. Thoracic radiographic study of dorsal root ganglia. Spine. 1994
radiculopathy following spinal cord stimulator place- Jan 1;19(1):6–11.
ment: case series. Neuromodulation Technol Neural 30. Yabuki S, Kikuchi S. Positions of dorsal root ganglia
Interface. 2013 Sep 1;16(5):443–8. in the cervical spine. An anatomic and clinical study.
13. Liveson JA. Thoracic radiculopathy related to col- Spine. 1996 Jul 1;21(13):1513–7.
lapsed thoracic vertebral bodies. J Neurol Neurosurg 31. Preston D, Shapiro B. Radiculopathy. In:
Psychiatry. 1984 Apr;47(4):404–6. Electromyography and neuromuscular disorders:
14. Sahenk Z, Mendell J, Rossio J, Hurtubise clinical-
electrophysiological correlates. 3rd ed.
P. Polyradiculopathy accompanying procainamide- China: Elsevier Saunders; 2013. p. 448–67.
induced lupus erythrematosus: evidence for drug- 32. Levin KH, Maggiano HJ, Wilbourn AJ. Cervical
induced enhanced sensitization to peripheral nerve radiculopathies: comparison of surgical and EMG
myelin. Ann Neurol. 1977;1:378–84. localization of single-root lesions. Neurology. 1996
15. Goldstein JM, Parks BJ, Mayer PL, Kim JH, Sze G, Apr;46(4):1022–5.
Miller RG. Nerve root hypertrophy as the cause of lumbar 33. Viikari-Juntura E, Porras M, Laasonen EM. Validity
stenosis in chronic inflammatory demyelinating polyra- of clinical tests in the diagnosis of root compression in
diculoneuropathy. Muscle Nerve. 1996 Jul;19(7):892–6. cervical disc disease. Spine. 1989 Mar;14(3):253–7.
16. Bastron JA, Thomas JE. Diabetic polyradiculopa- 34. Yoss RE, Corbin KB, Maccarty CS, Love
thy: clinical and electromyographic findings in 105 JG. Significance of symptoms and signs in local-
patients. Mayo Clin Proc. 1981 Dec;56(12):725–32.
ization of involved root in cervical disk protrusion. conventional electromyography. Arch Neurol. 1986
Neurology. 1957 Oct;7(10):673–83. Dec 1;43(12):1264–71.
35. Levin KH. Neurologic manifestations of compressive 52. Aminoff M, Goodin D. Electrophysiological evalua-
radiculopathy of the first thoracic root. Neurology. tion of lumbosacral radiculopathy; electromyography,
1999 Sep 22;53(5):1149–51. late responses and somatosensory evoked potentials.
36. Knutsson B. Comparative value of electromyographic, Neurology. 1985;35:1514–8.
myelographic and clinical-neurological examinations 53. Phillips LH, Park TS. Electrophysiologic mapping of
in diagnosis of lumbar root compression syndrome. the segmental anatomy of the muscles of the lower
Acta Orthop Scand Suppl. 1961;49:1–135. extremity. Muscle Nerve. 1991 Dec;14(12):1213–8.
37. Liguori R, Krarup C, Trojaborg W. Determination of 54. Wilbourn AJ, Aminoff MJ. AAEM Minimonograph
the segmental sensory and motor innervation of the 32: the electrodiagnostic examination in patients
lumbosacral spinal nerves. An electrophysiological with radiculopathies. Muscle Nerve. 1998 Dec
study. Brain J Neurol. 1992 Jun;115(Pt 3):915–34. 1;21(12):1612–31.
38. Thage O. The myotomes L2-S2 in man. Acta Neurol 55. Johnson E, Melvin J. Value of electromyography
Scand. 1965;41(Suppl 13):241–3. in lumbar radiculopathy. Arch Phys Med Rehabil.
39. Young A, Getty J, Jackson A, Kirwan E, Sullivan M, 1971;52:239–43.
Parry CW. Variations in the pattern of muscle inner- 56. LaJoie W. Nerve root compression: correlation of
vation by the L5 and S1 nerve roots. Spine. 1983 electromyographic, myelographic, and surgical find-
Sep;8(6):616–24. ings. Arch Phys Med Rehabil. 1972;53:390–2.
40. Feinberg JH, Radecki J. Parsonage-Turner Syndrome. 57. Tullberg T, Svanborg E, Isacsson J. A preoperative
HSS J. 2010 Sep;6(2):199–205. and postoperative study of the accuracy and value
41. Bachasson D, Singh A, Shah S, Lane JG, Ward of electrodiagnosis in patients with lumbosacral disc
SR. The role of the peripheral and central nervous herniation. Spine. 1993;18:837–42.
Systems in Rotator Cuff Disease. J Shoulder Elb Surg 58. Levin KH. L5 radiculopathy with reduced superficial
Am Shoulder Elb Surg Al. 2015 Aug;24(8):1322–35. peroneal sensory responses: intraspinal and extraspi-
42. Levin KH, Wilbourn AJ, Maggiano HJ. Cervical nal causes. Muscle Nerve. 1998 Jan;21(1):3–7.
rib and median sternotomy-related brachial 59. Wiltse LL, Guyer RD, Spencer CW, Glenn WV,
plexopathies: a reassessment. Neurology. 1998 Porter IS. Alar transverse process impingement of the
May;50(5):1407–13. L5 spinal nerve: the far-out syndrome. Spine. 1984
43. Preston DC. Compressive and entrapment neuropa- Feb;9(1):31–41.
thies of the upper extremity. In: Neuromuscular 60. Berger AR, Sharma K, Lipton RB. Comparison of
disorders in clinical practice. 2nd ed. New York: motor conduction abnormalities in lumbosacral radic-
Springer; 2014. p. 871–902. ulopathy and axonal polyneuropathy. Muscle Nerve.
44. Katirji B. Compressive and entrapment neuropathies 1999 Aug;22(8):1053–7.
of the lower extremity. In: Neuromuscular disorders 61. Gough JG, Koepke GH. Electromyographic determi-
in clinical practice. 2nd ed. New York: Springer; nation of motor root levels in erector spinae muscles.
2014. p. 903–52. Arch Phys Med Rehabil. 1966 Jan;47(1):9–11.
45. Cho SC, Ferrante MA, Levin KH, Harmon RL, So 62. American Association of Electrodiagnostic Medicine,
YT. Utility of electrodiagnostic testing in evaluating So YT. Guidelines in electrodiagnostic medicine.
patients with lumbosacral radiculopathy: an evidence- Practice parameter for needle electromyographic
based review. Muscle Nerve. 2010 Aug;42(2):276–82. evaluation of patients with suspected cervical radicu-
46. Katirji B, Weissman JD. The ankle jerk and the lopathy. Muscle Nerve Suppl. 1999;(8):S209–21.
tibial H-reflex: a clinical and electrophysiological 63. Nardin RA, Patel MR, Gudas TF, Rutkove SB,
correlation. Electromyogr Clin Neurophysiol. 1994 Raynor EM. Electromyography and magnetic reso-
Sep;34(6):331–4. nance imaging in the evaluation of radiculopathy.
47. Burke D, Gandevia SC, McKeon B. Monosynaptic Muscle Nerve. 1999 Feb;22(2):151–5.
and oligosynaptic contributions to human ankle jerk
and H-reflex. J Neurophysiol. 1984 Sep;52(3):435–48.
Recommended Reading
48. Fisher M. H reflexes and F waves: physiology and
clinical indications. Muscle Nerve. 1992;15:1223–33. Levin KH. Approach to the patient with suspected radicu-
49. Tsao BE, Levin KH, Bodner RA. Comparison of lopathy. Neurol Clin. 2012 May 1;30(2):581–604.
surgical and electrodiagnostic findings in single root Levin KH. Cervical radiculopathy. In: Neuromuscular
lumbosacral radiculopathies. Muscle Nerve. 2003 disorders in clinical practice. 2nd ed. New York:
Jan;27(1):60–4. Springer; 2014. p. 981–1000.
50. Wilbourne A, Aminoff M. Radiculopathies. In: Raynor E, Baruchow S, Nardin R, Kleiner-Fisman
Brown W, Bolton C, editors. Clinical electromyogra- G. Lumbosacral and thoracic radiculopathies. In:
phy. 2nd ed. Boston: Butterworth- Heinemann; 1993. Neuromuscular disorders in clinical practice. 2nd ed.
p. 177–209. New York: Springer; 2014. p. 1001–28.
51. Yiannikas C, Shahani BT, Young RR. Short-latency Preston D, Shapiro B. Radiculopathy. In:
somatosensory-evoked potentials from radial, Electromyography and neuromuscular disorders:
median, ulnar, and peroneal nerve stimulation in the clinical electrophysiological correlates. 3rd ed. China:
assessment of cervical spondylosis: comparison with Elsevier Saunders; 2013. p. 448–67.
Motor Neuron Diseases
9
Michelle M. Dompenciel
Etiology women, but this is rapidly changing as the inci-

dence can equal between men and women with
Motor neuron diseases (MND) are very challeng- increasing age. Genetic influence plays an impor-
ing to diagnose, and it is imperative that the cor- tant role as more gene mutations are found, some
rect diagnosis is reached early after the onset of linked with environmental risk factors causing
symptoms, because of the poor prognosis associ- degeneration of the motor neurons. ALS cases
ated with the disease. Amyotrophic Lateral were initially described and studied by Jean-
Sclerosis (ALS) is the most frequent form of Martin Charcot in 1869 as a pure motor neuron
MND. It causes gradual dysfunction of the upper disease with a very distinct pathology, and the
motor neurons (UMN) and lower motor neurons term amyotrophic lateral sclerosis was later
(LMN). The median survival of ALS is about introduced in his 1874 research paper. Nowadays,
2–3 years after onset of symptoms, typically ALS is considered a multi-systemic disease that
related to respiratory muscle weakness/failure. can be at times associated with non-motor symp-
However, because the disease is variable, there toms, causing dysfunction of the fronto-temporal
are a few exceptions, with some patients living lobes, cerebellar circuits (as may sometimes be
past the typical estimated life expectancy. seen in Madras MND), autonomic nervous sys-
UMN symptoms comprise spasticity, weak- tem, basal ganglia [1], dorsal columns, and even
ness, and pathologic hyperreflexia, and the cases described as related to idiopathic sensory
expression of symptoms varies between patients neuropathy [2]. Rare forms of ALS that can be
depending on which motor neurons are affected. inherited in endemic areas and which can present
LMN signs include fasciculations, cramps, mus- with ALS-Parkinsonism-Dementia complex have
cle atrophy and weakness. ALS patients are typi- also been reported. Madras motor neuron disease
cally diagnosed in late middle age (average age (MMND) is another rare subtype of motor neu-
of about 55 years at diagnosis), but more cases ron disease presenting typically in the young,
have been diagnosed as being genetic/familial having weakness and wasting of limb muscles,
affecting much younger adult patients as well. together with multiple lower cranial nerve palsies
ALS is typically more common in men than in and sensorineural hearing loss. Infrequently,
there may be cerebellar involvement, with cere-
bellar atrophy described in at least 1 case [3].
M. M. Dompenciel (*) A small percentage of ALS patients may mani-
Department of Neurology, Cleveland Clinic,
Weston, FL, USA fest with frontotemporal dementia (FTD) with
e-mail: dompenm@ccf.org cognitive deficits, personality changes, and

https://doi.org/10.1007/978-3-030-74997-2_9
214 M. M. Dompenciel
behavioral changes (up to 50% of ALS patients lesions on imaging, or family history of hereditary
with at least some of these features). Even though forms of spastic paraplegia, will make the diagno-
the majority of cases of ALS are sporadic, now it sis of PLS more convincing. Typically, the progno-
is considered a genetically heterogeneous disor- sis of PLS is better when compared to classic
der with a complex genetic etiology [4]. The most ALS. The etiology is considered mostly similar to
frequently mutated disease genes discovered are: ALS with a potential combination of genetic and
C9ORF72, SOD1 (the first gene mutation identi- environmental factors.
fied for ALS), NEK1 (sporadic and familial cases), Progressive Muscular Atrophy (PMA) is another
TDP-43 (mostly dominant forms of inheritance subtype of MND presenting with purely LMN
cases), and FUS (mostly dominant inheritance symptoms of: fasciculations, cramps, reduced/
pattern). C9ORF72 DNA expansion gene absent reflexes, flaccid muscle weakness, and atro-
accounts for more ALS cases with a genetic influ- phy. It also carries a better prognosis than
ence (seen in up to ~40%), including a predisposi- ALS. Appropriate diagnostic testing and close clini-
tion to developing FTD, and to a lesser extent cal observation are needed because some patients
seen in sporadic cases (up to ~7%). More genes with initial physical examination suggestive of
have been discovered that are associated with the PMA could progress to develop UMN signs, hence
development of ALS, and having an understand- eventually meeting El Escorial criteria for
ing of their role in the disease will affect future ALS. EDX evaluation is important to differentiate
therapeutic avenues. Nevertheless, there are limi- between PMA and multifocal motor neuropathy
tations with genetic testing in patients with sus- with conduction block (MMNCB), which is another
pected family history of ALS, mostly because of disorder mostly affecting the motor fibers with spar-
variable expression and incomplete penetrance of ing of the sensory fibers. MMNCB is an immune-
the genes [4]. ALS has been linked to excessive mediated demyelinating motor neuropathy, and it is
stimulation of glutaminergic NMDA (activation imperative for it to be excluded during EDX testing
of glutamate receptors causing elevation of neuro- and laboratory investigation (associated with GM1
nal intracellular calcium, leading ultimately to ganglioside antibody). This is especially important
cell death) and AMPA (alpha-amino-3-hydroxy- since most MMNCB, patients show improvement
5-methyl-4-isoxazolepropionate) receptors, with immune-modulating therapies (particularly IV
impaired axonal transport, increased oxidative immunoglobulin).
stress, glial cell dysfunction, reactive astrocytes, Progressive Bulbar Palsy (PBP) presents with
among other hypotheses, ultimately leading to selective damage of the motor nerves supplying
motor neuron degeneration [5]. the bulbar muscles, affecting speech and swal-
Primary Lateral Sclerosis (PLS) selectively lowing, and may affect the facial muscles as well.
affects the UMN with a clinical presentation of Most cases are sporadic and some familial ones
spasticity, pathologic hyperreflexia, weakness, and have been described. Diagnosis is usually delayed
even pseudobulbar affect [6]. It affects about 1–3% because the initial symptoms are mistaken as gas-
of patients diagnosed with MND, with also a slight trointestinal or ENT-related conditions. Patients
male predominance. Symptoms can take years to can present with tongue muscle atrophy with fas-
progress; most commonly exhibiting progressive ciculations, drooling, spastic speech, and brisk
paraplegia, spastic bulbar weakness, or hemiplegia facial reflexes. It can remain limited to the bulbar
[7]. Overlaps with other diseases have also been muscles, but in some cases, it may be the initial
documented [8]. The Pringle criteria suggests that presentation of the ALS type of MND. Close
the diagnosis is based on clinical findings, appro- clinical observation and EDX information over
priate laboratory testing (infectious, metabolic, or time are integral parts of securing the diagnosis.
toxic), EDX results not meeting El Escorial crite- A small study published in 2016 suggested early
ria, and at least 3 years of observation. This is to changes on imaging that could potentially assist
ensure that the correct diagnosis is made, as it can in the future when distinguishing among the dif-
be easily mistaken with other diseases/mimics. ferent MND variants. The study proposed early
Lack of LMN involvement on EDX, structural disease changes seen in diffusion tensor imaging
9 Motor Neuron Diseases 215
(DTI) and magnetic resonance spectroscopic

(MRS) studies in patients with bulbar-onset and
limb-onset ALS. Extra-motor involvement by the
corpus callosum is a feature seen in bulbar-onset
patients, when compared to limb-onset ALS, and
can suggest poor outcome in such patients [9].
Anatomy
The upper motor neurons (pyramidal tracts) orig-

inate in the brain’s primary motor cortex, and
those tracts carry voluntary motor activity from
Fig. 9.1 Hematoxylin and eosin stained slide showing
the cortex to the lower motor neurons. These loss of neurons in the anterior horn cell region with reac-
tracts will descend in the spinal cord to synapse tive astrocytes (arrow represents a motor neuron).
with the lower motor neurons at each spinal nerve Courtesy of Dr. Richard Prayson/Section Head of
Neuropathology at Cleveland Clinic
root level. Each of those axons will innervate sev-
eral fibers of a skeletal muscle. The major UMN/
pyramidal pathway is the corticospinal tracts,
which travel down the anterior horn to connect
with interneurons and exit the spinal cord to con-
vey voluntary muscle control to the extremities
and trunk. The other pyramidal pathway is the
corticobulbar tract, which connects to the cranial
nerve motor nuclei, like the nucleus ambiguus
(supplying motor fibers of the vagus and glosso-
pharyngeal nerves), and motor fibers of the tri-
geminal, facial, and accessory nerves. Damage to
the nucleus ambiguus will affect speech and
swallowing because of its control on the pharynx,
larynx, and soft palate muscles. Once the nerve
exits the spinal cord or brainstem (in the case of Fig. 9.2 Hematoxylin and eosin stained slide of the cer-
cranial nerve motor nuclei) it becomes a lower vical spinal cord with atrophy of the anterior spinal root-
lets (solid arrows) when compared to the posterior rootlets
motor neuron. Electrodiagnostic evaluation (dashed arrow). Courtesy of Dr. Richard Prayson/Section
(EDX) will specifically assess the function of the Head of Neuropathology at Cleveland Clinic
lower motor neurons. ALS affects both the upper
and lower motor neurons (see Figs. 9.1 and 9.2),
and remains a clinical diagnosis. EDX will assist ever, not fully understood. Hence, increased risk
in detecting lower motor neuron dysfunction, for ALS has also been associated with history of
with upper motor neuron involvement primarily traumatic brain injuries [10]. Typically, ALS
assessed during physical examination. starts with symptoms affecting one body seg-
From a histopathological point of view, astro- ment, and depending on the location and degree
cytes are vital in supporting and repairing the of spinal cord motor neuron loss, progressive
nervous tissue, and when they become reactive, weakness will ultimately involve adjacent
they promote motor neuron autophagy. It has myotomes. The disease will continue to spread to
been stipulated that motor neuron degeneration other extremities, or bulbar muscles, producing
has been linked to a reactive state of astrocytes, at weakness and respiratory complications, leading
times triggered by environmental factors like to death. Prompt diagnosis is paramount in order
traumatic central nervous system injuries; how- to offer available treatment to slow the disease
progression. Two FDA-approved medications are This can be considered in cases where the initial
available for the treatment of ALS: Riluzole and symptoms are bulbar, and the patient starts to
Edaravone. Riluzole may modulate and inhibit develop new symptoms spreading to other limbs.
glutamate neurotransmission, decreasing Clinical examinations should be performed at
glutamate-related excitotoxicity. Edaravone has least every 6 months for progression. Four
been associated with decreasing oxidative stress. regions have been established to describe the
Free radicals/oxidative stress have been linked to involvement/spread of clinical symptoms: bulbar,
motor nerve cell death, increasing the risk of ALS cervical, thoracic, and lumbosacral. The diagno-
development. sis becomes more evident when the features
spread within the same region, or involve other
regions. Moreover, if there is sensory, sphincter,
Clinical Features or autonomic dysfunction, then alternative diag-
noses should be considered. A detailed neuro-
Motor neuron diseases can be very difficult to logical examination, family history, past medical
diagnose because they can share clinical features, history, medications/toxin exposure history, and
at its earliest presentation, with other diseases/ onset/evolution of symptoms review need to be
mimics. At onset, the majority of ALS patients carefully taken into consideration when diagnos-
will have subtle features of weakness in either an ing MND. More importantly, electrophysiologi-
upper or lower extremity. Symptoms then evolve cal studies are always recommended to confirm a
to muscle atrophy, and continue to spread to other lower motor neuron process, and are essentially
myotomes. Depending on the location of motor equivalent to clinical LMN findings. Primary
neuron involvement, it can clinically mimic a Lateral Sclerosis (PLS) often presents as progres-
mononeuropathy such as at the ulnar nerve, or a sive leg weakness, cramps, and stiffness. The dis-
lumbar radiculopathy presenting with foot drop. ease course is prolonged, and has a better
The clinical absence of sensory symptoms should prognosis than ALS. On examination, patients
indicate to the clinician that a motor neuron pro- will develop pathologic hyperreflexia and marked
cess could be the etiology. If bulbar motor nerves spasticity. Some patients can develop cognitive
are involved at presentation, then the patients changes and pseudobulbar affect and dysarthric
may have spastic and/or flaccid speech, dysar- speech. At least 3 years are required for clinical
thria, dysphagia, leading to the development of observation, looking for progression or develop-
tongue atrophy with fasciculations and drooling. ment of features suggestive of LMN involve-
Most of these cases are initially extensively eval- ment, according to The Pringle criteria. EDX
uated by other specialists looking for other causes evaluation must show lack of a LMN process.
of dysphagia and dysarthria. Progressive Muscular Atrophy (PMA) also has a
The revised El Escorial criteria (see Table 9.1) prolonged course of symptom development. On
were published to assist in the correct diagnosis physical examination, the patients will show:
of ALS. Based on the guidelines, there has to be reduced or absent reflexes, fasciculations, muscle
clinical evidence of disease progression, and weakness, and ultimately muscle atrophy.
absence of alternative causes. Signs of upper and Limited forms of the disease have also been
lower motor neuron involvement must be pres- described, like flail arm or leg syndromes. The
ent, which may include that supported by electro- clinician needs to perform close observation over
physiological evaluation. Neuroimaging is time, looking for UMN signs or features that
always recommended to exclude mimics. The meet El Escorial criteria, to exclude the possibil-
revised El Escorial criteria classify cases as: sus- ity of disease progression to classic
pected, possible, probable, or definite ALS. Progressive Bulbar Palsy (PBP) presents
ALS. Appropriate laboratory evaluations are with early symptoms of speech, drooling, and
needed to rule-out other etiologies when the swallowing dysfunction. Patients can develop
diagnosis is in question. At times, repeating EDX tongue and facial weakness with atrophy and fas-
testing is required to discern disease progression. ciculations. Generally, the symptoms remain lim-
9
Table 9.1 World Federation of Neurology El Escorial criteria for ALS

LMN signs UMN signs Regions Clinically definite Clinically probable Clinically possible Clinically suspected
– Weakness – Spasticity -Bulbar UMN and LMN UMN and LMN features: →UMN and LMN signs →LMN or UMN signs only
– Atrophy – Pathologic (craniobulbar) features: → 2 regions in 1 region OR in 1 or more regions
– Fasciculations hyperreflexia -Cervical →bulbar and at →some UMN signs must be rostral →UMN signs in 2 or →absence of other possible
– Extensor -Thoracic least 2 other to (above) LMN signs more regions OR etiologies
plantar -Lumbosacral spinal regions → “Clinically probable, laboratory- →UMN and LMN signs
response OR supported ALS”: UMN signs in 1 or in 2 regions without
– Pseudobulbar →3 spinal more regions coupled with LMN UMN signs rostral to the
features regions signs by EMG in at least 2 regions LMN signs
→absence of →absence of other possible →absence of other
other possible etiologies possible etiologies
etiologies
Based on the revised El Escorial criteria (“Clinically Suspected” category carried forward from the original criteria). On electromyography, cervical and lumbar region requires
involvement of at least 2 muscles innervated by different nerve roots. Bulbar and thoracic region requires involvement of at least 1 muscle per region. ALS Amyotrophic lateral
sclerosis; UMN upper motor neuron; LMN lower motor neuron; EMG electromyography
217
ited to the bulbar muscles, but in some cases, it tioned, EDX evaluation will specifically assess the
can be the initial presentation of ALS. function of the lower (not upper) motor neurons.
Clinically, if there is evidence of widespread One caveat of EDX testing can be seen in multiple
LMN process (at least 2 or more regions), then ALS sclerosis patients when the plaque involvement is
should be suspected, provided that the appropriate near/at root exit zones, and the patient also has a
diagnostic testing (neuroimaging, laboratory, or more typical central nervous system lesion(s).
genetic testing if warranted) was performed to Clinically, the patient will express UMN and LMN
exclude other possible etiologies. Cognitive testing involvement, mimicking a motor neuron disease
should also be considered to assess ALS variants process. Although rare, it may present on EDX
like FTD-ALS. More forms of ALS are being testing as a pure LMN process, like a polyradicu-
described leading to the belief that it is a multi-sys- lopathy. Clinical examination, onset of symptoms
temic disease. It has to be recognized that ALS can review, and neuroimaging will certainly aid in dif-
be associated, in some cases, with mild sensory, ferentiating between the two entities. Post-polio
autonomic, and cerebellar, among other symptoms. syndrome should be easy to assess, because of
prior history of infection and slow muscle weak-
ness and atrophy progression over many years.
Differential Diagnosis Often patients present to the neurologist with
muscle twitching or fasciculations, having great
Motor neuron diseases have a myriad of symp- concern about the implications of this isolated
toms that can be confused with many other dis- symptom. In these cases, fasciculation potentials
eases at onset [11]. Using the revised El Escorial are often detected on EDX evaluation in the absence
criteria can assist in the proper clinical evaluation of any other significant changes. Close clinical
of ALS and its mimics. All of these patients should observation over time would typically confirm
undergo EDX evaluation, laboratory testing, and benign fasciculation syndrome, rather than a more
neuroimaging studies to exclude other disease sinister motor neuron process. In particular, lack of
possibilities. Requesting imaging studies is very unequivocal weakness or progressive muscle atro-
important because a structural lesion can present phy suggests a benign disorder like this.
with both UMN and LMN features. Some exam- Certain muscle diseases may mimic a disorder
ples of structural lesions are: cervical compressive of motor neuron dysfunction. This raises the impor-
myelopathy/myeloradiculopathy, brainstem or tance of appropriate laboratory including electrodi-
spinal cord tumors, also tandem UMN lesions agnostic testing, and in some cases muscle biopsy
with LMN lesions from plexopathy, or polyradicu- to confirm a diagnosis. Inclusion body myositis
lopathy, among others. However, some of these (IBM) is an idiopathic inflammatory disorder that
examples may have sensory loss clinical features, can present with asymmetric limb weakness (typi-
and should alert the clinician against the case for cally, deep finger flexors and quadriceps muscles),
ALS. Laboratory studies are recommended to with some difficulties in swallowing due to bulbar
exclude metabolic, toxic (organic pesticides, lead, muscle involvement. IBM can share some EDX
mercury, arsenic, among others), infectious, or features with ALS, hence ideally a muscle biopsy
nutritional causes. Vitamin B12 deficiency, thyroid should be performed in suspected cases for diagno-
dysfunction, copper deficiency, hyperparathyroid- sis confirmation. Oculopharyngeal muscular dys-
ism, heavy metals toxicity, vitamin E deficiency, trophy is another muscle disease that can present
Lyme disease, HIV myelopathy, and tropical spas- with progressive muscle weakness of the throat,
tic paraparesis (human T-lymphotropic virus type facial, ocular, and eyelid muscles. It can mimic
1 infection), are some other examples. Some of bulbar-onset ALS, specifically when the extraocu-
them can present with largely UMN symptoms, lar muscle symptoms are very subtle at onset. In
like HIV myelopathy and tropical spastic parapa- this case, genetic testing will help in the evaluation.
resis. EDX testing can only complement the physi- Isolated neck extensor myopathy is one of the eti-
cal examination, and should not be used in ologies of dropped head syndrome that will show
isolation to diagnose ALS. As previously men- signs of electrical “irritability” on needle electro-
myography testing in the cervical paraspinal mus- ciency (Tay-Sachs disease), are examples of
cles, and can be confused with MND at onset. hereditary diseases that may present with some
However, it is usually limited and does not spread features of MND clinically or electrodiagnosti-
to other myotomes, such as in ALS. Diseases of the cally. Hereditary spastic paraparesis can present
neuromuscular junction may present with LMN with UMN disorder, whereas spinal muscular atro-
features. Myasthenia gravis may present with bul- phy will present as slowly progressive muscle
bar symptoms, and at onset can be mistaken for weakness because of anterior horn cell/LMN
bulbar-onset ALS. To assist in differentiation, involvement. Kennedy’s disease patients will man-
blood evaluation [e.g. for acetylcholine receptor ifest with muscle cramps, tongue weakness/fas-
and MuSK (muscle-specific kinase) antibodies], ciculations, speech disturbance, and limb
and repetitive nerve stimulation on electrodiagnos- weakness. There is dysfunction of the motor neu-
tic testing (or single fiber EMG), can be performed rons at the brainstem and spinal cord, which can be
to establish the diagnosis of myasthenia gravis. confused with classic ALS, but these patients will
One should not rely only on symptom improve- also show endocrine dysregulation, and genetic
ment with cholinesterase inhibitors to differentiate testing will confirm the diagnosis. Hexosaminidase
between them, because some MND patients may A deficiency/adult or late-onset patients can
express transient symptom improvement with express speech and swallowing problems, but
these medications. prominent psychiatric and cognitive deficits can
Immune-mediated processes should always be differentiate it from bulbar-onset ALS.
investigated further because some could be poten- Paraneoplastic processes can also manifest
tially treatable. Multifocal motor neuropathy with with clinical features of MND. Lymphoma can
conduction block (MMNCB) presents with a present with lower extremity LMN features.
lower motor neuron dysfunction, and needs to be Radiation therapy can manifest with muscle
excluded from the progressive muscular atrophy weakness and atrophy, even many years after
MND variant. MMNCB is a purely motor demye- radiation exposure, and clinically exhibits a pure
linating neuropathy that is slowly progressive, and LMN process. EDX evaluation will be important
also begins distally as in many ALS cases. in this case because myokymic discharges are
Clinically, they can be differentiated by more mul- very commonly seen in radiation-induced pro-
tifocal individual motor nerves being affected in cesses, particularly plexopathy.
MMNCB, rather than progressively involving
adjacent myotomal distributions as in ALS/
MND. Anti-GM1 antibody presence, and motor Electrodiagnostic Evaluation
conduction block (between distal and proximal
motor segments) on EDX evaluation, are typical of Nerve Conduction Studies
MMNCB patients. The distinction between these
two processes must be made clear because a trial Nerve conduction studies and needle electromy-
of intravenous immunoglobulin therapy should be ography play an important role in the diagnostic
considered in MMNCB patients. Stiff person syn- process of motor neuron diseases, but essentially
drome patients will develop painful cramps and can only evaluate the presence of lower motor
spasticity, thus clinically mimicking a UMN dis- neuron dysfunction. Therefore, ALS is a clinical
ease. Typically, it affects the truncal muscles, but diagnosis, supported by the presence of UMN
there are other variants that are segmental or lim- dysfunction (signs disclosed on neurological
ited to a limb. Blood evaluation for glutamic acid exam) and LMN dysfunction (exam and/or EDX
decarboxylase (GAD) antibodies, and paraneo- findings). EDX evaluation will also serve to
plastic testing, should be performed to exclude exclude potentially treatable alternative etiolo-
underlying malignancy. gies, including a demyelinating motor/motor-
Hereditary spastic paraparesis, spinal muscular predominant polyneuropathy. Careful testing of
atrophy, Kennedy’s disease (spinal and bulbar several motor nerves should be performed to
muscular atrophy), and hexosaminidase A defi- increase the probability of finding a motor con-
duction block or focal/segmental demyelination. and above-elbow sites), peroneal (fibular) (record-
At times, proximal nerve stimulation can be con- ing at extensor digitorum brevis; stimulating at the
sidered, or contralateral studies, to look for perti- ankle, below the fibular neck, and lateral popliteal
nent features including motor conduction block. fossa), and tibial (recording at abductor hallucis;
In addition, if the late-responses are abnormal stimulating at the ankle and popliteal fossa).
and the motor studies are normal, contralateral or Consider peroneal (fibular) motor studies recording
proximal motor nerve studies are recommended. at the tibialis anterior muscle if the peroneal (fibu-
Upper and lower extremities must be assessed lar) motor study recording at the extensor digitorum
on nerve conduction studies (NCS). Features of brevis muscle is abnormal. If the CMAP amplitudes
motor axonal loss are classic findings of LMN are low at the median/abductor pollicis brevis or
involvement in ALS. Decreased compound muscle ulnar/abductor digiti minimi, a brief post-exercise
action potentials (CMAP), with relatively normal stimulation should be performed to evaluate the
distal latencies and conduction velocities are typi- presence of a presynaptic disorder of neuromuscu-
cal findings seen with motor axonal loss. If there is lar junction transmission. The ulnar/first dorsal
involvement of the largest and fastest conducting interosseous muscle response is also recommended,
axons, then there could be mild slowing of the con- especially as it pertains to the demonstration of a
duction velocities; however, not to the degree seen “split-hand pattern” which may be seen in ALS.
in a demyelinating process. Only the fastest con- Sensory nerve action potential (SNAP) studies
ducting fibers are measured on conduction veloci- should include the following nerves: median (stim-
ties and latency testing on NCS. If there is marked ulating at the wrist; recording at second digit), ulnar
motor axonal loss, the CMAP will drop, but the (stimulating at the wrist; recording at fifth digit),
distal latencies and conduction velocities should radial (stimulating at the forearm; recording at base
remain essentially normal (or almost normal), of the thumb), superficial peroneal (fibular) (stimu-
because there will be a few of the fastest conduct- lating at the lateral leg; recording at the ankle), and
ing fibers still left unaffected. These fibers can only sural (stimulating at the posterior portion of the calf;
drop to ~75% of the lower limit of normal conduc- recording at the posterior ankle). Late responses are
tion velocity because these myelinated fibers can- important and should include: F-waves (median,
not conduct slower than this range. Distal latencies ulnar, peroneal (fibular), and tibial nerves), and tib-
can be prolonged, but will not be greater than ial H-reflexes. Any abnormality should be com-
~130% of the upper limit of normal. Applying pared to the contralateral side. More proximal
these concepts to the evaluation of MND is impor- motor nerve stimulation could be considered look-
tant to exclude a demyelinating neuropathy. When ing for conduction block, but may be limited due to
there is a complete motor conduction block, there location and supramaximal stimulation pitfalls at
is a drop of more than 50% of the CMAP ampli- the axilla or Erb’s point. Late responses could also
tude or area, when comparing the distal and proxi- be minimally abnormal in MND/ALS, mostly
mal stimulation sites (with or without associated reflecting the reduced number of motor neurons
temporal dispersion). This tends to become marked available for the response, but are not typically
when the nerve is studied utilizing a long distance expected to be absent or significantly delayed, as
between stimulation sites. Sensory nerve conduc- may be seen in a severe polyradiculopathy.
tion studies should be normal in MND/ALS, except
in those cases where there is a superimposed pro-
cess like a mononeuropathy or polyneuropathy, in Needle Electrode Examination
which case relevant investigations should be per-
formed looking for other etiologies. Needle electromyographic assessment must be
Routine motor studies should be performed on comprehensive and must show evidence of wide-
the following nerves: median (recording at abductor spread denervation and re-innervation, specifically
pollicis brevis; stimulating at the wrist and antecu- in the majority of the four regions discussed previ-
bital fossa), ulnar (recording at the abductor digiti ously, and in at least two muscles of different spinal
minimi; stimulating at the wrist and at below-elbow nerve root innervation within each limb region.
Table 9.2 Recommended muscle selections for needle electrode examination- motor neuron disease protocol
Upper extremity Lower extremity Craniobulbar Paraspinal muscles
→first dorsal interosseous →tibialis anterior →tongue →cervical
→abductor digiti minimi →medial gastrocnemius Consider: →thoracic (must be
→abductor pollicis brevis →tibialis posterior or →sternocleidomastoid performed- typically,
→flexor pollicis longus (if flexor digitorum longus →masseter mid or low thoracic
question of inclusion body →rectus femoris or vastus →facial muscles levels)
myositis) lateralis →lumbosacral
→extensor indicis proprius →gluteus medius
→pronator teres
→biceps brachii
→triceps
→deltoid
Abnormal spontaneous activity (fibrillation, posi- recruitment is reduced (including the rapid firing
tive sharp wave, and/or fasciculation potentials) are frequency of affected MUAPs), but in myopa-
usually very prominent in a motor neuron disease thies there is typically “early” recruitment (of
process. However, fasciculation potentials alone are MUAPs which are polyphasic, but short in dura-
not sufficient to be considered as evidence of active/ tion and low in amplitude).
ongoing denervation, as they can be seen in other The recommended protocol for needle electro-
diseases, or may be a benign finding in some cases. myography should include at least two limbs (dis-
Nonetheless, in MND, fasciculation potentials tend tal and proximal muscles of different spinal nerve
to be large with multiple turns and/or phases com- root innervation), thoracic paraspinal muscles (typ-
prising a complex “bizarre-appearing” morphology. ically at the mid and low thoracic levels), and may
Noteworthy is the added pathological/diagnostic also include craniobulbar muscles (important when
significance that is conferred by fasciculation poten- excluding the possibility of superimposed cervical
tials when there is superimposition of chronic motor or lumbosacral polyradiculopathy). Active/ongo-
axon loss changes in the same muscle (added diag- ing denervation findings in the thoracic paraspinal
nostic yield from the Awaji criteria, compared to the muscles are commonly seen in most patients with
revised El Escorial criteria). Complex repetitive dis- MND/ALS, and several segments should be exam-
charges (CRDs) can be seen in chronic lower motor ined to increase diagnostic yield. Please refer to
neuron processes, but are not a particularly com- Table 9.2 for our recommended protocol of muscle
mon feature in MND. Abnormal needle EMG find- selection for needle electrode examination in motor
ings must show involvement of different myotomes, neuron disease cases.
with careful evaluation of possible sparing of indi-
vidual nerves that could suggest another process,
such as MMNCB. Electrodiagnostic Pitfalls
Careful evaluation of motor unit action poten- and Limitations
tials (MUAPs) are key in the assessment of a
lower motor neuron process. Features of chronic Sensory nerve action potentials are essential
axon loss will be manifested by MUAP configu- when demonstrating that there is definite electro-
rational changes- high amplitude, long duration, diagnostic evidence of a motor neuron process.
and may include increased polyphasia. There is As mentioned previously, SNAPs are expected to
often evidence of motor unit instability, as typi- be normal in lower motor neuron disease.
cally evidenced by “moment-to-moment ampli- However, if the patient has a superimposed
tude variation”. Decreased recruitment would mononeuropathy, or polyneuropathy (or plexopa-
also reflect the loss of motor units. Recruitment thy), then the results can seem confounding
analysis will be essential when differentiating a because of reduced SNAPs. In this case, history,
lower motor neuron process from a myopathic physical examination, and additional testing may
process (including one with overalapping dener- assist in the differential diagnostic investigation.
vation/neurogenic) features. With LMN lesions, Motor nerve studies must be evaluated with cau-
tion, because the examiner has to specifically Needle electromyography also has some limi-
exclude MMNCB. If there is any indication of tations during the evaluation of a lower motor
selective motor nerves being affected, with spar- neuron disease process. Accordingly, the assess-
ing of other individual motor nerves, MMNCB ment has to be comprehensive and should involve
(or multifocal motor neuropathy) has to be con- sufficient coverage of the majority of regions (cra-
sidered. Since a complete motor conduction niobulbar, cervical, thoracic, and lumbosacral).
block has been established as greater than 50% There should be the aforementioned electrical
drop in CMAP amplitude or area between distal evidence of active/ongoing and chronic axon loss
and proximal nerve stimulation sites, there needs (i.e. overlapping features of denervation and re-
to be vigilance to prevent spurious responses innervation), spanning different nerve roots/myo-
with similar changes. Accordingly, if supramaxi- tomes, which cannot be reasonably explained by
mal nerve stimulation was not achieved (or if any other etiologies. Thoracic paraspinal muscles
there are technical factors related to large body are of paramount importance when differentiating
habitus), then responses may exhibit a motor motor neuron disease from a polyradiculopathy,
conduction block pattern, leading to misdiagno- as typically they will be abnormal in MND. In
sis. For example, a patient can be misdiagnosed contrast, a polyradiculopathy is commonly seen at
as having a demyelinating polyneuropathy, when the cervical and lumbar regions, and is much less
the underlying pathological entity is actually likely at the thoracic region. Moreover, some fas-
motor neuron disease. This can result from ciculations can be seen during the needle EMG of
improper testing of nerve conduction responses, patients with a polyradiculopathy (or any other
and the inability to acquire the SNAPs correctly, neurogenic process), and need careful interpreta-
and consequently documenting an abnormal or tion. Fasciculations alone cannot be considered as
absent response which should otherwise be pres- evidence of active/ongoing denervation. However,
ent. Therefore, proficiency in nerve conduction in conjunction with chronic motor axon loss
studies is of paramount importance. changes, they may have similar significance per
Again, at times it is recommended to repeat the Awaji criteria.
electrodiagnostic testing after several months to Other caveats in the interpretation of needle
confirm progression of disease over time and to electromyography include patient’s tolerance for
ascertain the diagnosis. Moreover, cervical and testing (intolerance usually manifested by subop-
lumbosacral polyradiculopathies can manifest timal activation of MUAPs), and their ability to
with the same nerve conduction features of a complete the full extensive protocol. Intolerance
lower motor neuron disease, mostly because the issues (e.g. from pain-related effects) could lead
SNAPs are normal (lesions are proximal to the to incomplete estimation of MUAP recruitment,
dorsal root ganglia). However, in these patients, because of suboptimal MUAP activation.
sensory symptoms and signs are typically pres- Additionally, incomplete muscle relaxation ham-
ent, contrasting with MND patients. pers reliable spontaneous activity assessment.
Late responses are not expected to be signifi- This is commonly seen during craniobulbar mus-
cantly abnormal in most cases of MND/ cle needle EMG, especially with impaired relax-
ALS. This finding can be seen in the late or end ation typically encountered when examining the
stages of the disease, as more motor neurons tongue muscle.
become affected and can’t contribute to the late Adequate discussion, including clarification
response. As more of the largest and fastest the of expectations should occur before requesting
constituent fibers are affected, the F-wave laten- electrodiagnostic study to ensure that the patient
cies are expected to be progressively prolonged. understands the testing procedure, especially as
Significant abnormalities of the late responses the MND protocol is very extensive.
are commonly seen in a polyradiculopathy, and Some chronic muscle diseases can be very
this feature could assist the electromyographer challenging to differentiate from a motor neuron
when making the distinction between this entity disorder, particularly if there are superimposed
and MND, but it is generally not considered suf- chronic denervation-type changes (as can be
ficient, especially as an isolated finding. commonly seen in inclusion body myositis). On
needle electromyography, they may exhibit On initial neurological examination: mental

chronic neurogenic changes with or without status, cranial nerves, and spine/straight leg raise
abundant spontaneous activity abnormalities test were normal/negative. Both upper extremi-
(fibrillation potentials/positive wave potentials) ties and the right lower extremity were normal in
which may be seen in both active/ongoing dener- motor and sensory examination. The left lower
vation and myopathy with inflammatory/necro- extremity had mild-to-moderate diffuse muscle
tizing features. Therefore, these disorders can atrophy, mostly distal to the knee with motor
sometimes mimic a motor neuron process. As strength graded at 3- to 4−/5 (MRC scale),
mentioned previously, the MUAP recruitment throughout the left L2-S1 myotomes. No fascicu-
pattern can be used to differentiate between the lations, no tongue atrophy, dystonic posturing,
two, as well as history and physical examination, tremors, dysmetria or spasticity were noted.
and other laboratory testing (e.g. creatine kinase Reflexes were 2+ throughout, even in the context
level). This is why electrodiagnostic testing alone of the weakness noted in the left lower limb.
cannot be used to diagnose a motor neuron dis- Plantar responses were mute bilaterally, and there
ease, and can only be a component (albeit an was no clonus. Sensory examination was normal
important one) of the comprehensive evaluation. to all modalities tested.
Amyotrophic lateral sclerosis remains a clinical Since the history, neurological examination,
diagnosis, supported by electrodiagnostic testing, and recent electrodiagnostic testing were rather
neuroimaging, laboratory studies, and history/ contradicting, we decided to order additional
physical examination findings. On this basis, it testing. A lumbar puncture was performed show-
may be prudent that the interpretation section of ing normal: cell count, protein, glucose, albumin,
the EDX study does not claim that the pertinent IgG index/synthesis rate, myelin basic protein,
results are “diagnostic” for MND/ALS, but rather culture, and smear. In addition, she tested nega-
are compatible/consistent with this diagnosis in tive for CSF Lyme antibodies, VDRL, and oligo-
the appropriate clinical context. clonal bands. On blood testing she had normal/
negative: 24-hour urine heavy metal panel,
comprehensive ganglioside panel, GAD anti-
Case Study body, vitamin B-12, comprehensive metabolic
panel, Lyme IgG/IgM, CBC, ESR, and CRP.
A 68 year-old right-handed Caucasian woman, with Neuroimaging showed multilevel degenerative
a past medical history of hypertension, was referred changes in the cervical spine, and very minimal
for progressive left foot drop for about 4 months. disc degeneration in the lumbar spine without
The weakness started very distally at the toes, then evidence of significant central canal or neurofo-
slowly progressed proximally to involve the ankle. raminal stenosis. There was evidence of wide-
There was no lower back pain, limb numbness or spread chronic ischemic white matter changes on
paresthesia, symptoms of bowel/bladder dysfunc- the brain MRI, but no acute findings were seen.
tion, or prior history of falls or trauma. She saw a On the follow-up appointment 6 months later,
neurosurgeon who advised her that there was no there was now more progressive leg weakness,
surgical intervention needed for the essentially involving the right lower extremity, and hands.
unremarkable lumbar spine findings on MRI. There She had subjective symptoms of mild swallowing
was no involvement of the right lower extremity, or dysfunction, without breathing difficulties.
the upper extremities. There were no symptoms of Sensory examination remained normal. Reflexes
craniobulbar or respiratory muscle weakness. At were now pathologically brisk, and mild spastic-
another facility, she was recently diagnosed with a ity was noted in the lower extremities. Considering
severe, subacute on chronic mixed axonal- normal testing, including CSF protein level, the
demyelinating peripheral polyneuropathy, based on patient agreed to have the electrodiagnostic test-
electrodiagnostic testing, and intravenous immuno- ing repeated. Please refer to Table 9.3 for EDX
globulin therapy had been commenced. There is no study results.
family history of neurodegenerative diseases.
224
Sensory Nerve Conduction

Nerve Stimulus Recording L R L R L R L R Amp Norm LatNPk Norm CV L R
Sural Lower Leg Lat Malleolus 12.17 3.88 n/a 140 >3 uV <4.6 ms >40 m/s 31.9
Lower Leg Ankle 6.25 3.64 n/a 100 >3 uV <4.6 ms >40 m/s 31.8
Median Wrist Index 13.49 3.70 n/a 130 >10 uV <3.8 ms >50 m/s 32.8
Ulnar Wrist 5th Dig 16.02 2.80 n/a 110 >5 uV <3.2 ms >50 m/s 32.6
Motor Nerve Conduction
B-P Amp (μV) LatOn (ms) CV (m/s) Dist (mm) Norm Norm Distal Norm Temp (°C)
Nerve Recording Stimulus L R L R L R L R B-P Amp LatOn CV L R
Peroneal (fibular)/ EDB Ankle 2.40 4.60 n/a 70 >2.5 <6 ms >40 32.3
EDB Pop 2.00 13.75 45.4 415 mV m/s 32.4
Tibial/AH AH Ankle 4.01 4.05 n/a 80 >4 mV <6 ms >40 32.7

Pop 3.77 13.20 44.8 410 m/s 32.8
Peroneal (fibular)/ TA Below Fib 2.67 2.45 n/a >3 mV <4.5 ms >40 33.1
TA Head m/s
Median APB Wrist 5.32 3.90 n/a 50 >5 mV <4 ms >50 33.0
Elbow 4.92 9.10 57.7 300 m/s 33.0
Ulnar/ADM ADM Wrist 9.26 2.96 n/a 50 >7 mV <3.1 ms >50 33.2
Below 8.68 6.90 58.2 230 m/s 32.9
Elbow
Above 8.41 8.90 55.5 330 33.1
Elbow
Ulnar/1stDI 1stDI Wrist 7.28 3.05 n/a >7 mV <4.5 ms >50 33.3
m/s
F-Wave Side-To-Side Comparison Table
F-Waves
Lat (ms)
Tibial/AH Ankle AH 56.70
M. M. Dompenciel
9
Table
M-Wave H-Wave
Pop Fossa Soleus Left 5.5ms 9.6mV 33.7ms 1.7
Needle EMG Summary
Side Muscle Ins Act Fib PW Fasc Other Number Recruit Dur Dur Amp Amp Poly Poly Descript Descript Descript
L 1st Dorsal Inter Norm 1+ 1+ 0+ 2- Rapid Many 1+ Many 1+ Norm NC NC NC
Abduc.Pol.Brevis Norm 1+ 0 1+ 3- Rapid All 1+ All 1+ Some 1+ ATR NC NC
Abduc.Digiti.Minimi Norm 0 0+ 0 2- Rapid Many 1+ Some 1+ Norm NC NC NC

Flex.Pollicis Longus Norm 0 0+ 0 1- Mod-R Some 1+ Some 1+ Norm NC NC NC
Extn. Indicis Pro Norm 0+ 0 0+ 1- Rapid Many 1+ Many 1+ Norm NC NC NC
Pronator Teres Norm 0 0+ 0+ 1- Mod-R Few 1+ Few 1+ Norm NC NC NC
Biceps Brachii Norm 0 0 0+ Norm Full Norm Norm Norm NC NC NC
Triceps-Lat Norm 0 0 0 1- Mod Norm Norm Norm NC NC NC
Deltoid, Middle Norm 1+ 0 0+ 1- Mod-R Few 1+ Norm Few 1+ NC NC NC
SCM Norm 0 0+ 0 1- Mod-R Few 1+ Few 1+ Norm NC NC NC
Cervical PSP (Low) Norm 1+ 0 1+ NE NC NC NC
Abductor Hallucis Norm 0 0 0+ MTP 3- Mod-R Many 1+ Many 1+ Norm NC NC NC
Extn. Digitorum Brv Norm 0+ 0 0+ 3- Mod-V Most 1+ Many 1+ Norm NC NC NC
Flex. Digitrum 2- Mod-R Many 1+ Some 1+ Norm NRLX NC NC
Longus
Tibialis Anterior Norm 2+ 1+ 1+ 3- Rapid Most 1+ Many 1+ Some 1+ MMAV NC NC
Gastroc. Medial H Norm 0 1+ 0+ 2- Mod-R Many 1+ Some 1+ Few 1+ NC NC NC
Rectus Famoris Norm 0+ 0 0 1- Mod-R Few 1+ Few 1+ Norm NC NC NC
Vastus Lateralis Norm 1+ 0 0 2- Mod-R Many 1+ Many 1+ Norm NC NC NC
Gluteus Medius Norm 0 1+ 0 2- Mod-R Many 1+ Many 1+ Few 1+ NC NC NC
Lumbar PSP (Low) Norm 0 0+ 0 NE NC NC NC
Tongue Norm 1+ 0 1+ 1- Mod-R Few 1+ Few 1+ Norm NC NC NC
Thoracic PSP (Mid) 2- Mod-R Many 1+ Few 1+ Some 1+ NRLX
Thoracic PSP (Low) Norm 1+ 0 1+ 1- Mod-R Few 1+ Few 1+ Norms NC NC NC
Abbreviations: ATR atrophy; MMAV moment-to-moment amplitude variation; MTP myotonic potential; NE recruitment pattern not examined (muscle examined at rest only)
NRLX not relaxed; SCM sternocleidomastoid; EDB Extensor digitorum brevis; AH Abductor hallucis; TA Tibialis anterior; APB Abductor pollicis brevis; ADM Abductor digiti
minimi; 1st DI First dorsal interosseous; PSP Paraspinals
225
Considering the nerve conduction findings, tubes, and referral to other medical specialists
especially the preserved SNAPs, we decided to (e.g. pulmonary, physical/occupational therapy,
perform a more extensive needle electromyogra- nutritionist), as well as referral to a medical social
phy evaluation, conforming to the lab’s MND worker. Although the diagnosis of ALS may be
protocol. Widespread chronic MUAP neurogenic initially difficult to elucidate, prompt diagnosis
changes (including increased duration and ampli- can allow the patient to have an opportunity to
tude, with or without polyphasic units), with evi- receive treatment/supportive care that could
dence of active/ongoing denervation (fibrillation increase quality of life, even if the improvement
and positive sharp wave potentials) in addition to in longevity is not very marked.
scattered fasciculation potentials were seen in the
muscles of the left upper and lower extremities,
as well as the thoracic and craniobulbar regions. References
No myopathic units were seen. The findings
spanned multiple nerve roots/myotomes (also 1. Xu J, et al. Abnormal cortical-basal ganglia net-
work in amyotrophic lateral sclerosis: a voxel-wise
implicating progression from the initial areas network efficiency analysis. Behav Brain Res.
described as involved), correlating with the most 2017;333:123–8.
recent worsening of clinical features disclosed at 2. Isaacs JD, Dean AF, Shaw CE, Al-Chalabi A, Mills
the follow-up office visit. Collectively, the results KR, Nigel LP. Amyotrophic lateral sclerosis with
sensory neuropathy: part of a multisystem disorder?
were consistent with a generalized active/ongo- J Neurol Neurosurg Psychiatry. 2007;78(7):750–3.
ing on chronic motor axon loss process (conspic- 3. Ling L, Xiaodong C, Jia L, Zhuang K, Jing L, Zizhen
uously sparing sensory responses) compatible H, Ruomi G, Zhengqi L. A madras motor neuron dis-
with an evolving widespread disorder of anterior ease patient with cerebellar atrophy: a new clinical
feature. Front Neurosci. 2018;12:722.
horn cells/motor neurons. 4. Volk AE, Weishaupt JH, Andersen PM, Ludolph AC,
These results cannot be explained by the neu- Kubisch C. Current knowledge and recent insights
roimaging, or laboratory/spinal tap results into the genetic basis of amyotrophic lateral sclerosis.
obtained. In this case, it became apparent that the Med Genet. 2018;30(2):252–8.
5. Pehar M, Harlan BA, Killoy KM, Vargas
diagnosis of MND/ALS was strongly supported MR. Role and therapeutic potential of astrocytes
by the latest EDX study, and that the initial study in amyotrophic lateral sclerosis. Curr Pharm Des.
produced erroneous results and interpretation. 2017;23(33):5010–21.
Electrodiagnostic testing should be repeated for 6. Tartaglia MC, Rowe A, Findlater K, et al.
Differentiation between primary lateral sclerosis and
cases in which the clinical presentation is not amyotrophic lateral sclerosis: examination of symp-
consistent the EDX results provided. A repeat toms and signs at disease onset and during follow-up.
EDX study may also serve to more objectively Arch Neurol. 2007;64(2):232–6.
demonstrate progression of disease. It is impera- 7. Zhai P, Pagan F, Statland J, Butman JA, Floeter
MK. Primary lateral sclerosis: a heterogeneous dis-
tive to have the appropriate expertise when per- order composed of different subtypes? Neurology.
forming these studies. In this case, pertinent 2003;60(8):1258–65.
alternative etiologies were excluded by compre- 8. Statland JM, Barohn RJ, Dimachkie MM, Floeter
hensive testing. MK, Mitsumoto H. Neurol Clin. 2015;33(4):749–60.
9. Vora M, Kumar S, Sharma S, et al. Advanced mag-
ALS patients should ideally be further evalu- netic resonance neuroimaging in bulbar and limb
ated and managed at ALS multidisciplinary clin- onset early amyotrophic lateral sclerosis. J Neurosci
ics, consistent with recommended best practice Rural Pract. 2016;7(1):102–8.
guidelines. Such specialized ALS clinics typi- 10. Tripathi P, Rodriguez-Muela N, Klim JR, et al. Stem
Cell Reports. 2017;9(2):667–80.
cally provide timely access to several services/ 11. Ghasemi M. Amyotrophic lateral sclerosis mimic
resources including assistive devices/adaptive syndromes. Iran J Neurol. 2016;15(2):85–91.
equipment, non-invasive ventilation, feeding
Disorders of the Neuromuscular
Junction 10
Raghav Govindarajan and Elanagan Nagarajan
natomy and Physiology of the

A The skeletal muscle fiber membrane at the end
Neuromuscular Junction plate is thickened and invaginates into the muscle
fiber forming a depression. This depression is
A synapse is a functional junction between two known as “synaptic trough or gutter.” This synap-
excitable cells. The neuron which transmits infor- tic gutter has both chemical and electrical charac-
mation is the presynaptic neuron, and the one teristic features that are different from the rest of
which receives the information is the postsynap- the muscle membrane and also serves as a loca-
tic neuron (in the case of the NMJ, this is the tion for most of the acetylcholine receptors in the
muscle fiber). Between the pre- and postsynaptic postsynaptic membrane.
membranes, there is a definite anatomical gap The synaptic cleft is filled with an electron-
called the synaptic cleft [2–5]. dense material called “ground substance”, made
The axon of the presynaptic neuron loses its up of acetylcholinesterase enzyme (AChE). This
myelin sheath as it reaches the muscle fiber and is AChE degrades the acetylcholine into acetate and
branched at the nerve terminal, where it ends as a choline [5]. The detailed anatomy of the NMJ is
knob-like structure. This structure contains mito- shown in Fig. 10.1a.
chondria which is rich in adenosine triphosphate The series of sequential events needed for the
(ATP) along with coated and uncoated vesicles. generation and propagation of the action poten-
The mitochondrial ATP is used as an energy tial leading to muscle contraction is summarized
source for the synthesis of acetylcholine which is in Fig. 10.1b [2–5].
stored in the vesicles. These vesicles are not uni- When an impulse reaches the presynaptic ter-
formly distributed along the synaptic cleft but are minal, a delay of 0.5 msec occurs before a
clustered at a region called the active zone response is elicited in the postsynaptic mem-
[2–5]. brane. This time delay is called synaptic delay,
and this occurs because the aforementioned
R. Govindarajan (*) · E. Nagarajan sequence of events has to occur [2–5].
Department of Neurology, Neurology Clinics, ALS The integrity of the AChR at the postsynaptic
and MDA Clinic, EMG/Neurophysiology Lab, membrane and its function are determined by the
Clinical Outcomes for Department of Neurology,
presence of several other proteins in the muscle end
University of Missouri, Columbia, MO, USA
plate region. Agrin is a large proteoglycan secreted
Department of Neurology, University of Missouri,
by the nerve terminal which interacts with muscle-
Columbia, MO, USA
e-mail: govindarajanr@health.missouri.edu; specific tyrosine kinase (MuSK) through its core-
nagarajane@health.missouri.edu ceptor, low-density lipoprotein receptor-related

https://doi.org/10.1007/978-3-030-74997-2_10
228 R. Govindarajan and E. Nagarajan
protein 4 (LRP4). The resultant MuSK-LRP4 com- Epidemiology

plex results in the activation and clustering of
AChRs at the neuromuscular junction. Based on the available epidemiological data,
there is a significant variation in the incidence
and prevalence of MG due to ethnic diversity and
Myasthenia Gravis geographic region. It was reported that the world-
wide prevalence is 40–180 per 100,000 people
Myasthenia gravis (MG) is a prototypical human with an annual incidence of 4–12 per 100,000
autoimmune disorder in which the neuromuscular people [6]. Recent studies have suggested that the
transmission is impaired due to the presence of incidence is higher, especially in the elderly and
antibodies against structural components in the this is due to the more widespread availability of
muscle membrane. This results in fluctuating antibody testing, and the better understanding of
muscle weakness and fatigability. The identifica- clinical presentation [7]. Juvenile myasthenia
tion of different antibodies and the relatively easy gravis is most commonly seen in patients of East
availability of antibody assays have shown a sig- Asian origin and half of the cases have an onset
nificant improvement in the diagnosis and tailored before the age of 15 and is restricted to ocular
therapy. The current management options include muscle only [8]. Women are more prone to have
symptomatic treatment, immunomodulation/ MG before the age of 50 years old, which is typi-
immunosuppression therapy, and thymectomy in cal of many autoimmune disorders when com-
a selected subgroup of the MG population. pared to male [9]. Late-onset MG which is above
Propagation of action potential

a Axon terminal
in the motor neuron
Voltage gated Calcium Channel

Calcium ions
Acetylcholine vesicles
Voltage gated Sodium Channel

Propagation of action potential
in the muscle fiber
Acetylcholinesterase
Acetylcholine gated
receptor-channel
Motor End Plate
Contractile Elements of the Muscle
Fig. 10.1 (a) The Neuromuscular Junction anatomy and schematic. (b) Sequential Physiological Changes During
Neuromuscular Junction Transmission
10 Disorders of the Neuromuscular Junction 229
Action potential reaches the

Activation of motor neuron nerve terminal and activates
b and propagation of Ca++ channels in the
action potential presynaptic terminal.
Entry of Calcium through Ca++

channel causes synaptic vesicles
containing Acetylcholine (ACh) to
be released into synaptic cleft.
ACh binds ACh receptors

(AChR) in the postsynaptic
nicotinic receptor.
Followed by rapid decline in

synaptic ACh levels via:
Once ACh binds to postsynaptic
membrane, it leads to influx of
Na+ and Ca++ ions
ACh ACh deactivation by

Diffusion acetylcholinesterase
(AChE) within the
synaptic cleft
Results in the formation of Endplate
Potentials (EPP) and change in the
configuration of ACh receptor
This prevents multiple

reactivations of ACh
receptors.
EPP ultimately triggers action

potential in the muscle fiber and
cause muscle contraction.
Fig. 10.1 (continued)
50 year old is more commonly seen in males elderly population usually presents with eye and
when compared to females [9]. bulbar symptoms [10–12]. The weakness is gen-
erally more common in ocular muscles, but it
can also be seen in the extraocular eye muscles,
Clinical Presentation other craniobulbar, limb and axial/truncal mus-
cles. Roughly around 60% of patient’s presents
Muscle weakness is the major hallmark in the with ptosis or diplopia or both and in 20% of
diagnosis of MG. Accentuation in weakness at patients, symptoms remain essentially restricted
the end of the day and exercise-induced weak- to the ocular distribution [10, 11, 13]. The weak-
ness is strongly suggestive of MG. Younger ness of extraocular muscle is almost always
individuals often present with unspecified asymmetrical whereas limb muscles are gener-
fatigue as a major complaint, whereas the ally symmetrically involved with proximal
uscles usually being involved more than the

m with antibodies against agrin and cortactin, but
distal muscles [13]. the pathological role in the disease process
remains unclear [17, 18]. The diagnosis becomes
more difficult when there is total absence of anti-
Subtypes of Myasthenia Gravis bodies. In such a population, the possibility of
other myasthenic syndromes, muscle, and non-
MG can be classified into different subtypes muscle disorders needs to be considered as well
based upon the presence of antibodies (and the [11].
specific antibody) against the structural proteins
in the postsynaptic membrane. There may be
some noteworthy differences in the clinical fea- Ocular Myasthenia Gravis
tures and treatment responses between the
subtypes. The weakness remains restricted to ocular mus-
cles in this population. This group of patients has
the highest risk of developing generalized MG
ysthenia Gravis (MG) Associated
M within 2 years of symptom onset. Approximately
with Acetylcholine Receptor half of the patients with ocular myasthenia gravis
Antibodies (ACHR) Antibodies have a positive acetylcholine receptor antibody,
and rarely the presence of MUSK antibodies are
Figure 10.2 below summarizes the key clinical also reported [19].
and pathological characteristics of AChR anti-
body positive MG.
CASE
ysthenia Gravis (MG) Associated

M A 23-year-old woman who has a known history
with Muscle-Specific Tyrosine of epilepsy presented with a complaint of exces-
Kinase (MuSK) and LDL Receptor sive daytime fatigue and tiredness. On further
Related Protein 4 (LRP4) Receptor questioning, she had a frequent head drop with-
Antibodies out any loss of awareness and was diagnosed
with psychogenic non-epileptic spells. Since then
Figure 10.3 below summarizes the key clinical she had a progressive worsening with an increase
and pathological characteristics of MuSK and in the frequency of head drop along with breath-
LRP4 antibody positive MG. ing difficulty at rest and worse with activity. Her
symptoms were worse in the evening and also
associated with dysphagia. Neurological exami-
Seronegative Myasthenia Gravis nation was significant for mild neck extensor
muscle weakness and fatigable weakness in the
In this subgroup, patients have clinical features upper extremities. EDX including repetitive
consistent with MG, however, their AChR, nerve stimulation (RNS) studies showed 40%
MuSK, or LRP4 antibodies levels are found to be decrement in the abductor digiti minimi, and
undetectable which is probably due to a low needle examination was notable for fibrillation
affinity or low concentration (below threshold) potentials and myopathic-appearing motor units
on routine assays [14, 15]. These antibodies may in the paraspinal and proximal upper limb mus-
be detectable through the cell-based assay which cles. Single fiber EMG of the orbicularis oculus
is not routinely done in most laboratories [16]. showed abnormal jitter. She was started on pyr-
Roughly around 20–50% of patients with sero- idostigmine for symptomatic control which para-
negative MG were found to have low-affinity doxically made her symptom worse. Anti-MuSK
antibodies [14, 15]. Cases have also been reported antibodies were found to be positive and the
Presence of
Acetylcholine receptor
(Ach R) antibody
Presence of Thymoma on Thymoma Associated

Imaging Myasthenia Gravis
Age of the Patient

• It is a paraneoplastic
disorder
• Prevalence is same in both
Less than 50 More than 50 male and female
years old years old
• Peak incidence around 50
year old.
• Ach R antibody positive
Late Onset • Thymoma and non
Early Onset Myasthenia Gravis -thymoma MG have similar
Myasthenia MG long-term prognosis
Gravis
• Common in male
• Common in female • Weakly
• Associated with HLA associated
DR3, HLA-B8 with HLA-DR3,
• May have presence of HLA-B8
Thymic Hyperplasia
• Rarely this patient
• Respond well to will have Thymic
thymectomy Hyperplasia
• Possibly not respond
HLA = human leukocyte antigen as well to
thymectomy
Fig. 10.2 Clinical and Pathological Features of Acetylcholine Receptor Antibody positive myasthenia gravis. HLA
human leukocyte antigen
AChR antibodies testing was found to be unre- Comment

markable. CT chest was unremarkable for thymic This case highlights myasthenia gravis at a
abnormalities. She was started with monthly young age, female predominance with promi-
IVIG treatment along with prednisone and ritux- nent bulbar, respiratory and proximal muscle
imab. Within a year, she was off of IVIG and has weakness. MuSK antibodies are found to be
been maintained on low-dose prednisone and positive with EDX findings consistent with a
rituximab. At 2 years follow-up, swallowing and postsynaptic disorder of neuromuscular junction
breathing difficulties, and head drop remain transmission. Typically, clinical symptoms in
improved significantly. MuSK MG get worse with acetylcholineesterase
Presynaptic membrane
releases AGRIN
Antibody
against MuSK
Interact with Muscle Specific MUSK Associated

Kinase (MuSK) on the Myasthenia Gravis
postsynaptic membrane
LRP4 Antibody MUSK interacts with Low

against LRP4
Associated density lipoprotein receptor • 1-4 % of patients with MG has
Myasthenia related peptide (LRP4) reported to have this antibody.
gravis • Female predominance
• Begins from childhood through
middle age, rarely in elderly and
kids
• Predominantly involve cranial
MUSK – LRP4 - Complex and bulbar muscles
• Female predominance • 1/3 of patients presents with
• 2-27% of patients with ptosis and diplopia
MG have this • Thymic changes are absent or
antibody positive. minimal.
• Often presents with • Many patients do not improve
ocular or mild Clustering and with cholinesterase inhibitors
generalized stabilization of • Most improve dramatically with
myasthenia gravis. acetylcholine receptor PLEX or corticosteroids.
• Respiratory
with help of trans-member
insufficiency is rare
proteins rapsyn
in this population.
• Thymus found to be and DOK-7
atrophic, hyper plastic
or normal for age.
Contraction followed
by relaxation of
skeletal muscle
PLEX: Plasma Exchange/Plasmapheresis
Fig. 10.3 Clinical and Pathological Features of Myasthenia Gravis with Muscle-Specific Tyrosine Kinase (MuSK) and
LDL Receptor Related Protein 4 (LRP4) Receptor Antibodies. PLEX Plasma Exchange/Plasmapheresis
inhibitors and they respond well to immunomod- formed by placing an instant cold ice pack on the
ulation therapy. affected eye for 2–5 min. If this test is positive,
the patient will no longer have characteristic pto-
sis of MG. The sensitivity of this test has been
Diagnostic Testing reported as 76.9% and the specificity of 98.3%.
The effect seen is attributable to inhibition of ace-
Ice Pack Test tylcholinesterase activity at a reduced tempera-
ture which results in transient improvement of
The ice pack test is a simple and practical bedside clinical symptoms [20]. However, the final diag-
test if the patient has ptosis on physical examina- nosis of MG needs to be confirmed by the pres-
tion and it can also help to rule out other causes ence of serum antibodies and electrophysiological
of ptosis or ophthalmoparesis. This test is per- findings.
Edrophonium Chloride Test spontaneously as a single quantum or multiple

quanta depending on the action potential. The
This is one of the classic tests for MG. It is fea- spontaneous release causes a small depolariza-
sible at the bedside and also in an office setting, tion in the postsynaptic membrane called
but it is infrequently used now due to potentially Miniature Endplate Potential (MEPP). A large
serious complications such as bradycardia and number of quanta released causes a depolariza-
syncope. The diagnostic yield is greater than tion of resting membrane at the endplate region
90% in patients with ptosis or diplopia, and this called endplate potential (EPP). When the EPP
can also be used as ancillary testing when anti- reach a threshold which leads to a generation of
body or electrodiagnostic test is unrevealing. The action potential in the postsynaptic muscle mem-
response is dose-dependent and usually begins brane, this ultimately leads to muscle contrac-
with intravenous administration of 2 mg edro- tion. When motor nerve fibers are stimulated, the
phonium, with dose up to 10 mg typically pre-synaptic region releases acetylcholine from
accepted. The clinical response needs to be moni- the immediately available primary stores, and
tored for 60 seconds after administration. If there when a continuous voluntary contraction is sus-
is any intermittent improvement in clinical symp- tained, the primary stores are depleted. To main-
toms within 60 seconds of administration, then tain the contraction, the secondary stores starts to
no further dose is necessary. Some people are replenish the depletion from the primary stores.
susceptible even to the low doses, and it is advised Thus initiation of the secondary stores use hap-
to have atropine available at the bedside to pre- pens with a slight lag of time. This secondary
vent any deleterious complications [21, 22]. stores activation when primary stores are depleted
is called the Safety Factor. At the time of second-
ary stores activation, the nerve releases three
Electrodiagnostic Studies times the effective dose of acetylcholine.
The depletion of the primary store competes
Electrophysiological studies such as RNS and with mobilization from the secondary store. This
Single Fiber Electromyography (SFEMG) indi- may become apparent as impulses fail to dis-
rectly measure the neuromuscular junction func- charge from the muscle fibers effectively (block-
tion and helps us to diagnose and characterize the ing), causing a decrease in the Compound muscle
specific defects i.e. presynaptic, synaptic and action potential (CMAP). CMAP is the sum of
post synaptic disorders. action potentials from muscle fibers, which are
activated by nerve stimulation [23, 24].
Repetitive Nerve Stimulation (RNS) Slow-Rate Stimulation

At the optimal frequency (2–5 Hz) slow rate
Definition stimulation, the primary store is maximally
It is a neurophysiological study to assess the depleted while the secondary store mobilization
integrity of neuromuscular transmission. is minimized. A dip in the CMAP waveform is
typically visualized, with the size of the CMAP
Physiology of RNS responses decreasing progressively by the fourth
As we discussed earlier in this chapter, acetyl- to sixth impulses (decline of ACh quanta from the
choline is stored in the presynaptic membrane immediately available pool), followed by a rever-
inside vesicles. The amount of acetylcholine sal. This is called as the decremental response
stored in vesicles is referred to as quantum. These [25].
quanta have been physiologically divided into
three compartments (primary, secondary and ter- Fast-Rate Stimulation
tiary), depending on their availability for use. A high frequency (15–30, or up to 50 Hz) RNS,
The release of acetylcholine can happen either causes cumulative facilitation of transmitter
release (due to increasing Ca+ entry into the A: Decrement of compound muscle action poten-
nerve terminal). This is the basic response to tial (CMAP) amplitude at rest.
rapid nerve stimulation, leading to post activa- B: Postexercise facilitation, Decrement of CMAP
tion potentiation. The CMAP responses gradu- immediately following 10 seconds of maximal
ally increases, this is called an incremental voluntary exercise has repaired toward
response [25]. normal.
lectrophysiological Findings in MG
E
The electrophysiological findings in MG is a
classical decremental response by slow rate
(2–5 Hz) RNS due to the defective postsynaptic
NMJ transmission. The decremental response in a 32%
slow rate occurs due to the failure of some mus-
cle fibers to reach the threshold. This is because,
even when a successive volley of acetylcholine is
released from the synaptic vesicles, there is much
less availability of ACh receptor in the post syn- b
8%
aptic muscle membrane. Thus, CMAP failure
occurs which corresponds to difficultly in initiat-
ing muscle contraction. This process is called
blocking. A train of stimuli (8–10) with the supra-
maximal intensity is given to elicit CMAP
responses from the muscle. The percentage of the c 44%
amplitude decrement from first to fifth CMAP
waveform should be 10% or more to be inter-
preted as abnormal. In some patients, the decre-
mental response can be observed during resting
and tends to worsen after activation (exercise).
This is called postexercise exhaustion which d 56%
occurs in 2–5 min after the exercise. In some
patients, after activation, the decrement response
tend to improve or repair, and this is called as
postexercise facilitation. Proper technical meth-
ods, immobilization of the limb during the proce-
dure, standard stimulus at the same point, proper e 69%
voluntary activation and patient cooperation
ensures a good result. RNS studies the integrity
of NMJ transmission, and the decrement response
is usually observed reliably in the clinically weak
muscles. The decremental responses in MG are
generally more prominent in the proximal mus- f 11%
cles compared to the distal muscles [26, 27].
Electrophysiological findings are illustrated in
Fig. 10.4. Fig. 10.4 The RNS findings in patients with myasthenia
Postexercise facilitation and exhaustion. gravis (used with permission- from Electromyography and
Neuromuscular Disorders: Clinical-Electrophysiologic
3-Hertz repetitive nerve stimulation in a patient Correlations, second Edition by Preston, David C.;
with myasthenia gravis. Shapiro, Barbara published by Butterworth-Heinemann)
C–E: Postexercise exhaustion. Decrements of to drop or reduce, the examiner should advise the
CMAP 1, 2, and 3 min after 1 min of maximal patient to continue with the activation exercise. If
voluntary exercise. Decrement becomes pro- the noise reduces or drops, the patient’s muscle
gressively more marked over the baseline contraction should be improved to maintain con-
decrement. traction for at least 1 minute.
F: Postexercise facilitation after a decrement. RNS should be performed at rest and also with
Immediately following another 10 seconds of regular intervals after activation as above. Greater
maximal voluntary exercise, the decrement, ≥10% decrement in at least one muscle is
which has worsened as a result of postexercise required to confirm the diagnosis.
exhaustion, repairs toward normal. Examination of another muscle group can be
considered depending upon the patient clinical
presentation.
Technical Aspect of RNS
The selection of a particular muscle depends on Pretest Instructions

several factors which include clinical symp-
toms, the simplicity of performing the test, reli- • Patients should be off anticholinesterase treat-
ability of the test, and the amount of potential ment for at least 12–24 h if the condition
discomfort for the patient. It is recommended to allows.
start testing with small distal muscles that can • Let the patient rest for 15 min before the
be easily immobilized and reliably tested, fol- recording (important for follow up studies). In
lowed by proximal muscles if they are needed LEMS 20 min rest is required.
[27, 28]. • The patient’s tested limb must be warm, if
The skin should be cleaned with alcohol wipes not—warm them up appropriately. (cooling
prior to the procedure, and temperature should be increases the safety factor at NMJ)
maintained as close to 35 °C as possible over the • Stimulus-related pain must be pre-discussed
recording site. The active electrode is placed over to help with anticipation and tolerance,, which
the motor endplate area (muscle belly) and the should reduce the liklihood of suboptimal
reference electrode over the distal tendon. The recordings/waveforms.
muscle should be at rest and immobilized [27].
The technical aspects of commonly tested mus-
cles, their nerve supply, their stimulation site, Recording Protocol
electrode placement and activation procedures
are summarized in Table 10.1. The standard recoding protocol is as follows
A pathological decremental response is typi- (adopted from the textbook of Electromyography
cal and shows a successive decrease in CMAP and Neuromuscular Disorders: Clinical-
amplitude from the first to the fourth stimulation, Electrophysiologic Correlations, second Edition
then a slight recovery (facilitation) towards the by Preston, David C.; Shapiro, Barbara published
tenth stimuli (“saddle shaped” response). If the by Butterworth-Heinemann, utilized with
amplitudes fluctuate up and down during the permission).
recording, this indicates a technical problem sec-
ondary to movements or electrode artifacts 1. Keep the body temperature close to 35
[25–29]. degrees.
During activation, the examiner should inform 2. Immobilize the muscle as best as possible.
the patient that he/she should exert full effort in 3. Recommended to do routine nerve conduction
activating the muscle against his/her resistance studies before to make sure nerves are suffi-
(when applied). When the contraction noise tends ciently normal;
Table 10.1 Technical Aspects of Nerve Conduction Study with Repetitive Nerve Stimulation (RNS) For Myasthenia Gravis
236
Nerve supply Stimulation site Ground placement Electrode placement

Muscle Active Reference Activation procedure
Abductor Median nerve At the wrist, between the At forearm, nearerthe At the belly Tendon of Ask the subject to abduct the thumb
Pollicis Brevis tendons of palmaris longus site of stimulation of abductor abductor and sustain abduction for 1 min and
and flexor carpi radialis at pollicis pollicis immediately give 8–10 stimuli at 3 Hz.
the level of second crease. brevis. brevis.
Deltoid muscle Axillary nerve Supramaximal stimulus Shoulder Deltoid Shoulder Ask the subject to move his elbow in a
over the upper plexus, Erb’s muscle belly bone lateral direction and the examiner
point, proximal to the resists against this movement for 1 min
clavicular bone. and immediately give 8–10 stimuli at
3 Hz.
Biceps Musculocutaneous Supramaximal stimulus Shoulder Belly of Biceps Ask the patient to flex the arm against
nerve over the upper plexus, Erb’s biceps tendon resistance for 1 min and immediately
point, proximal to the muscle give 8–10 stimuli at 3 Hz.
clavicular bone.
Abductor digiti Ulnar nerve At the wrist, medial or Extensor surface of the Belly of Distal Ask the patient to spread his/her fingers
minimi lateral to the flexor carpi hand abductor phalanx of maximally for 1 min and immediately
ulnaris tendon at the second digiti fifth digit give 8–10 stimuli at 3 Hz.
crease with the hand resting minimi
and with the dorsal side muscle
facing upwards.
Orbicularis Facial nerve Beneath the ear lobe at the Forehead Over the Over the Ask the subject to do continuous
oculus grove of stylomastoid orbicularis canthus closure of the eyes tightly for 1 min and
foramen oculus immediately give 8–10 stimuli at 3 Hz.
muscle.
Nasalis Facial nerve Beneath the ear lobe at the Forehead Over the Over the Ask the subject to shrink his nose for
grove of stylomastoid nasalis nasal bone 1 min and immediately give 8–10
foramen muscle or nasal stimuli at 3 Hz
bridge.
Trapezius Accessory nerve Behind the Placed between the Placed over Placed over Ask the subject to lift the shoulder up
sternocleidomastoid recording and the belly of the clavicle for 1 min and immediately give 8–10
muscle, at the midpoint of a stimulating electrode trapezius or acromion stimuli at 3 Hz
line connecting the mastoid muscle. process.
and the clavicle
R. Govindarajan and E. Nagarajan
4. Perform RNS at rest. After making sure that generation of motor potentials (“afterpotentials”)
the stimulus is supramaximal, perform 3 Hz after single stimulation shocks [27, 29].
RNS at rest for 5–10 impulses, repeated three False-positive result happens when an obvious
times, 1 min apart. Normally, there is <10% decrement is found when there is none.
decrement between the first and fourth The factors that would cause false positive test
responses. include poor electrode placement, inadequate
5. If >10% decrement occurs and is consistently mobilization of the limb, and inappropriate stim-
reproducible: ulus delivery. Patient cooperation and discomfort
• Have the patient perform maximal volun- tolerance are important technical parameters that
tary exercise for 10 seconds. affect the RNS outcome. Patients with severe dis-
• Immediately repeat 3 Hz RNS post exer- ease may have difficulty in performing the activa-
cise to demonstrate post exercise facilitation procedure. Intubated patients (who often
tion and repair of the decrement. have mental status effects from critical illness
6. If <10% Decrement or no Decrement Occurs comorbidity and/or medications) may also have
• Have the patient perform maximal volun- poor cooperation, and artifacts from the ventila-
tary exercise for 1 min, then perform 3 Hz tor and other intensive care unit electronics which
RNS immediately and 1,2,3 and 4 min can affect the RNS tests results [25, 27, 29].
after exercise to demonstrate post exercise
exhaustion.
• If a significant decrement occurs, have the ingle Fiber Electromyography
S
patient perform maximal voluntary exer- (SFEMG)
cise again for 10 seconds and immediately
repeat 3 Hz RNS to demonstrate repair of SFEMG is a selective recording of a small num-
the decrement. ber of single muscle fiber action potentials
belonging to the same motor unit [27, 30].
SFEMG technique is a confirmatory test for
Pitfalls and Limitations screening the NMJ disorders even when the RNS
results are negative [31].
Technical factors play a major role in the mea- Further details regarding SFEMG are found in
suring accuracy of RNS, and they are broadly the dedicated chapter (Chap. 11) covering this
classified into false positive and false negative topic.
results.
The false negative results occur when there is
no decrement observed when, in fact, under the Treatment of MG
appropriate conditions, it would be seen. Two
major factors that can cause false negative report Patients with mild symptoms due to MG often do
include low temperature and presence of acetyl- well with symptomatic treatment alone [6].
cholinesterase inhibitors. Drugs, which causes the reduction of acetylcho-
Studies show that cold temperate slows down line breakdown through the inhibition of enzyme
the activity of acetylcholinesterase in the synap- acetylcholinesterase results in an increase of ace-
tic cleft and results in an increase in the availabil- tylcholine concentration in the synaptic cleft.
ity of acetylcholine and this would account for Pyridostigmine is the most commonly used drug,.
the difference (masking of the pathological NMJ
transmission deficit). The presence of acetylcho-
linesterase inhibitors during the procedure may Immunosuppressive Therapy
similarly obscure the underlying NMJ patho-
physiology, but may be suspected as the con- Immunosuppressive therapy is warranted in
founding factor present when there is repetitive patients who do not achieve complete remission
with symptomatic therapy alone. The commonly patients with small cell lung carcinoma (SCLC)
used immunosuppressive drugs include azathio- but may occur with other malignancies including
prine, and mycophenolate mofetil (among other non-small cell lung cancer, prostate cancer, thy-
similar medications). These drugs often are com- moma and lymphoproliferative disorder [36–38].
bined with corticosteroids such as prednisone It History of smoking, male gender, and weight loss
is recommended to start with steroids to curb the are found to have a strong association in patients
initial clinical deterioration. Once the symptoms with LEMS [39]. The clinical symptoms of
are stable along with the addition of immunosup- LEMS precede approximately 5 years before the
pressive agents over the duration of time for their evidence of malignancy. Sixty-five percent of
onset of action (typically over several months), patient with NT-LEMS were found to have an
the steroid needs to be reduced slowly to the low- association with HLA -B8-DR 3 genetic poly-
est effective level [6, 8, 32, 33]. morphism [39].
Myasthenic crises are considered a neuromus-
cular emergency and should be managed in the
critical care unit setting (details beyond the scope Epidemiology
of this textbook).
LEMS is a rare disorder with a predicted inci-
dence of 0.5 out of 1,00,000 and a prevalence of
Lambert-Eaton Myasthenic 2.3 out of 1,00,000 patients. The prevalence of
Syndrome (LEMS) LEMS is 46 times less than that of myasthenia
gravis which is suggestive of an overall poor out-
Lambert-Eaton myasthenic syndrome (LEMS) is come, especially when it is associated with
a neuromuscular disorder with core clinical fea- malignancy [40, 41]. The median age at the onset
tures consistent with proximal muscle weakness, of diagnosis in patients with malignancy was
areflexia, and autonomic dysfunction [8, 34]. It 60 years, and up to 75% of them are men.
may present either as a primary autoimmune dis- Interestingly in NT-LEMS patients, age and sex
order or could be due to a paraneoplastic etiol- distribution are similar to patients with MG with
ogy. This is most commonly seen in patients with a peak age of onset of 35 years and a second
small cell lung cancer. The weakness is putatively larger peak at 60 years [40, 42]. NT -LEMS is
due to the generation of auto-antibody against the most commonly seen in young females with a
voltage-gated calcium channels in the presynap- genetic association of HLA-B8-DR3 polymor-
tic membrane which causes selective destruction phism which is suggestive of autoimmune etiol-
and reduction of acetylcholine which in turn ogy [39].
leads to subsequent weakness [8]. The diagnosis
is based on clinical examination and is confirmed
by serologic testing and electrodiagnostic Pathophysiology
studies.
The voltage-gated calcium channel (VGCC) is a
transmembrane protein in the presynaptic mem-
Etiology brane, and it has approximately 4–5 subunits with
a central pore. As the cell membrane depolarizes,
LEMS is classified based upon the presence or it causes an influx of calcium through the central
absence of malignancy. Patients without any evi- pore resulting in the release of acetylcholine into
dence of malignancy are classified as Non-Tumor the synapse [8]. The tumor cells express VGCC,
Lambert-Eaton Myasthenic Syndrome an antigen which induces autoantibody produc-
(NT-LEMS) [8, 35]. Approximately 60% of the tion. The antibody-mediated immune response in
patients with the LEMS have an association with patients with LEMS is thought to be due to the
malignancy. This is most commonly seen in crosslinking and selective destruction of VGCC
which is often associated with the antibodies rest and increased to 4+ out of 5 after 30 seconds
against the P/Q subtype of VGCC and rarely in contraction. Deep tendon reflexes were dimin-
association with N-type antibody as well [41, 43]. ished (though somewhat improved after sus-
tained contraction as well), and rest of the
neurological examination was found to be unre-
Clinical Features markable. Routine chest X ray showed 4.3 cm
left upper lobe lung mass. CT chest confirmed
The clinical symptoms are usually gradual in the mass along with the presence of central
onset, but it can also progress rapidly in patients necrosis. These findings were concerning for
with SCLC. The classic triad consists of proxi- possible LEMS. On nerve conduction studies of
mal weakness, areflexia, and autonomic dysfunc- his right ulnar nerve, a reduced compound mus-
tion. The weakness is generally symmetrical and cle action potential (CMAP) of 2.5 mV (normal
usually begins in the proximal lower extremities, >5) was noted recording the abductor digiti min-
causing gait disturbances [8, 34–36]. Patients imi muscle. The response improved to 5 mV
often present with complaints of difficulty in after sustained contraction of the muscle for
raising from a seated position, dull aching, and 10 seconds. There was no conduction block
stiffness in the affected limbs. The symptoms across the elbow on other ulnar motor studies.
usually progress from proximal to distal, caudal Sensory nerve conduction responses were found
to cranial distribution and then it finally reaches to be normal as well. Slow repetitive nerve stim-
the oculobulbar region [44]. Deep tendon reflexes ulation (at 2 Hz) of right ulnar nerve showed
are either diminished or absent with no signifi- more than 20% decrement in the amplitude.
cant muscle atrophy. Up to 95% of patient with Paraneoplastic antibodies testing revealed the
LEMS are reported to have autonomic dysfunc- presence of P/Q voltage-gated calcium channel
tion. Most commonly reported symptoms antibodies. The diagnosis of paraneoplastic
includes dry mouth, constipation, erectile dys- LEMS was confirmed. She underwent surgical
function in male, orthostatic hypotension, pupil- resection of the lung lesion and is undergoing
lary abnormalities and loss of sweating [44, 45]. chemotherapy along with 3,4 DAP for symptom-
Involvement of respiratory muscles, and cranial atic therapy (seeing improvement in muscle
nerves is uncommon during initial stages but it weakness on this).
can happen as the disease progresses. In those
cases, ptosis and diplopia comprise the most
common presentation, followed by dysarthria Diagnostic Evaluation
and dysphagia. [8, 34]
The diagnosis of LEMS is confirmed by the pres-
ence of antibodies against the P/Q type voltage
CASE gated calcium channels (VGCC). Up to 90% of
patients with LEMS are found to have high titers
A 62-year-old Caucasian male with a history of of these antibodies. Almost all the patients with
hypertension and smoking presented with SCLC are positive for this antibody and up to
3 months history of bilateral lower extremity 90% of patients with NT-LEMS also have detect-
weakness and fatigue. The weakness was associ- able levels of P/Q type VGCC antibodies. Patients
ated with 5-pound weight loss. The patient with undetectable levels of antibodies probably
denied any numbness, paresthesia, and/or change have lower concentration of antibodies or could
in bowel and bladder habits. There has been possess antibodies to different epitopes or other
some excessive mouth dryness and reduction in structural proteins. However, the presence of
sweating. Neurological examination was signifi- these antibodies is not only reported in LEMS but
cant for 3 out 5 power (Medical Research are also seen in other neurological/autoimmune
Council scale) in bilateral lower extremities at conditions as well [43, 46].
The N-type VGCCs antibody is more com- SFEMG in LEMS

monly seen in patients with primary lung cancer,
and the presence of this antibody may increase • Increased Jitter and Blocking occurs
the possibility of having an underlying malig- • Contrary to what is typically seen in MG,
nancy as well [46]. SCLC also express an immu- blocking tends to improve with the surface
nogenic tumor antigen called SOX1and 64% of stimulation rates.
patients with LEMS are found to have antibodies
against this antigen. Reports suggest that SOX1
may play a role in predicting future predisposi- Treatment and Management
tion to LEMS/SCLC. Accordingly, the presence
of SOX1 antibodies with no underlying evidence Treatment of patients with LEMS includes resect-
of malignancy should be followed up very closely ing the underlying tumor (to the extent possible)
[47, 48]. and symptomatic management. The aim of the
symptomatic treatment is to improve the
acetylcholine concentration in the synaptic cleft
Electrophysiological Findings with drugs like pyridostigmine, neostigmine,
in LEMS guanidine and 4-aminopyridine, and 3,4-diami-
nopyridine (3,4-DAP) [49, 50]. Only 3,4-DAP is
The classical electrophysiological finding in studied extensively in clinical trials, and all other
LEMS is a low amplitude CMAP at rest. This is drugs are reported from case series.
due to the decreased acetylcholine in the presyn- 3,4-DAP blocks potassium channels, which in
aptic terminal and blocking in many of the neuro- turn keeps calcium channels open for longer peri-
muscular junctions. A decremental response is ods, ultimately allowing more acetylcholine to be
observed at supramaximal slow rate stimulus and released into the synaptic cleft.
is due to the depletion of the available acetylcho- In patients with rapidly progressive symp-
line. After brief, sustained isometric contraction toms, intravenous immunoglobulin (IVIG) or
(of at least 10 seconds), the amplitude of CMAP plasmapheresis are found to be effective in both
tends to increase by 100% or more. The facilita- paraneoplastic LEMS and NT LEMS [51].
tion (CMAP amplitude increment) is due to the Long term oral immunosuppressive medica-
presynaptic calcium influx which ultimately tions such as prednisone, azathioprine, and ritux-
increases the available acetylcholine. Apart from imab have also been tried with a variable degree
demonstrating the increment with this methodol- of success in patients when the symptoms are not
ogy, fast-rate RNS (15–50 Hz) may be employed. adequately controlled with symptomatic treat-
In LEMS patients undergoing fast-rate RNS, ment alone [34].
there are successive CMAP amplitude increases,
with the first and fifth waveforms typically dem-
onstrating an increase by 100% or more. The ongenital Myasthenic Syndrome
C
limitation with fast-rate RNS is that it is painful (CMS) (CMS)
and the associated limb movement may interfere
with a technically adequate CMAP capture. The Congenital myasthenic syndrome is a rare group
RNS and short exercise testing findings in LEMS of genetic conditions that are due to functionally
are shown in Fig. 10.5. abnormal proteins that affect NM transmission.
For RNS in LEMS cases, 2 Proximal muscles This results in fluctuating or fatigable weakness.
(e.g. orbicularis oculus, nasalis, trapezius) and 1 The onset of symptoms varies and are commonly
distal muscle (e.g. abductor pollicis brevis and seen at birth but may go unrecognized until ado-
abductor digiti minimi) are usually selected. The lescence or adulthood [52–54]. Congenital myas-
details of the procedure, limitations, and pitfalls thenia gravis is classified based upon the location
have been discussed under the MG section of this of the defect. Presynaptic defects accounts for
chapter. approximately 7–8%, synaptic defect are
Fig. 10.5 Electrophysiological findings of RNS in a patient with LEMS

e stimated at 14–15% and the remaining 75–80% the formation of acetylcholine. Mutations within
are due to postsynaptic defects [52–56]. the gene coding for this enzyme results in deple-
tion of acetylcholine-containing vesicles. The
clinical spectrum of patients with this enzyme
Clinical Features deficiency varies significantly [59, 60]. Patients
with severe disease often have severe apnea, bul-
Males are more commonly affected than females bar weakness and may also require ventilation
with an estimated ratio of 2:1. Almost all CMS sub- support at birth. While others may present with
types follow an autosomal recessive pattern except mild weakness and episodic apneic spells. There
for the slow channel syndrome which is an autoso- is also another group of patients who have milder
mal dominant disease. The classification and diag- symptoms but with proximal weakness and no
nosis of this syndrome is based on clinical features, respiratory involvement [59–62].
response to acetyl cholinesterase inhibitors Patients with ChAT enzyme deficiency do
(AChEi) and electrophysiological study findings. respond well to acetylcholinesterase inhibitors,
Incomplete ophthalmoparesis, ptosis, and mild and the symptoms are triggered by or become
facial paresis are often seen during infancy. worse with exposure to cold temperature which
Ophthalmoparesis and facial paresis progress dur- has not been similarly reported in other myas-
ing childhood and infancy along with generalized thenic syndromes [61].
weakness and fatigue. Often these are associated
with other features such as high arched palate,
facial dysmorphism, arthrogryposis, and scoliosis. Synaptic
Episodic respiratory crises can occur with any form
of CMS but is most commonly seen in patients Acetylcholinesterase Deficiency
with acetylcholinesterase deficiency [54, 57, 58]. Acetylcholinesterase is an enzyme present in the
synaptic cleft and is responsible for hydrolysis of
acetylcholine after its action. The enzyme serves
Diagnosis and Testing as a rate limiting factor which controls the num-
ber of collisions between acetylcholine and its
The diagnosis is based on the presence of clinical receptor, thereby determining the duration of the
symptoms suggestive of myasthenia gravis like synaptic transmission [54]. This enzyme com-
fluctuating or fatigable weakness, absence of per- prises catalytic subunits that bind to collagenic
tinent antibodies (against the acetylcholine recep- tails (COQL) which helps to anchor this enzyme
tor, MuSK, or LRP4), and a decremental response in the synaptic cleft. The deficiency of acetylcho-
on RNS. [57] Genetic testing is also available for linesterase is caused by a recessive mutation in
the diagnosis of the specific subtype.. the gene COLQ resulting in the blockade of its
Interestingly, roughly around 30–50% of patients binding in the basal layer. This leads to prolonged
with CMS do not carry the mutations that have exposure of acetylcholine to the postsynaptic
been already described in the literature [52–54]. membrane [63, 64].
The clinical manifestations of this enzyme
deficiency often present during early childhood
Specific Genetic Syndromes and are rare during infancy. It is characterized by
severe axial weakness, muscle atrophy and slow
Pre-Synaptic pupillary response to light stimulation. As the
disease progresses skeletal deformities (e.g.,
holine Acetyltransferase (ChAT)
C lordosis or scoliosis), ptosis, ophthalmoplegia,
Deficiency dysphagia, and difficulty breathing are noted
Choline acetyltransferase is an enzyme present in [52, 53]. NCS usually discloses repetitive
the presynaptic region which is responsible for CMAPs (“afterdischarges”) in these patients.
Cholinesterase inhibitors such as pyridostigmine rimary Kinetic Abnormalities

P
is contraindicated with COLQ deficiency. of the Acetylcholine Receptor
Ephedrine or albuterol seem to show improve-
ment of symptoms via an unknown mechanism in The clinical characteristics, physiology, and
some patients [62, 65]. pathophysiology in patients with a kinetic abnor-
mality is summarized in the flowchart (Fig. 10.6)
below.
ongenital Myasthenic Syndrome
C Electrophysiologically, patients with slow
with Postsynaptic Defects channel syndrome have a decremental response
to slow-rate RNS(2–3 Hz). They tend to exhibit a
Anatomy of the Acetylcholine smaller amplitude and substantial decrement on
Receptor the second peak compared to previous compound
muscle action potential. NCS also often discloses
The nicotinic acetylcholine (AChR) is a pen- repetitive CMAPs (“afterdischarges”) in these
tamer that exists in two different forms—fetal patients. Needle electromyography often shows a
and adult form. The fetal form (α2 β δ γ) consists myopathic pattern which is most likely second-
of two alpha subunits (α2), one beta subunit (β), ary to an increase in the intracellular calcium due
one delta subunit (δ) and one gamma submit (γ) to prolonged opening time which eventually
whereas in adult form (α2 β δ €) gamma (γ) is leads to “endplate myopathy” as the disease pro-
replaced with an epsilon (€) subunit [66]. gresses [57]. Similarly, patients with fast channel
syndrome often have a decremental response
with 2–3 Hz stimulation [57]. Acetlycholines
Acetylcholine Receptor Deficiency terase inhibitors need to be avoided in the patients
with slow channel syndrome because they cause
Patients with this deficiency are noted to have a a prolonged exposure of acetylcholine in the
relative reduction in the expression of acetylcho- postsynaptic muscle membrane which can lead to
line receptors in the postsynaptic membrane. accelerated endplate myopathy. Quinidine and
Often this follows an autosomal recessive inheri- fluoxetine are among agents found to be effective
tance pattern due to a mutation in the CHRNE in patients with slow channel syndrome as they
gene. The mutation most commonly is seen is in appear to block the opening of the acetylcholine
€ subunit, however, patients continue to have an receptor [69]. Medications that increase opening
expression of fetal form of the receptor which time of the acetylcholine receptor such as AChE
compensates for the deficiency [59].Patients with inhibitors (e.g. pyridostigmine) and 3,4-DAP are
mutation of other subunits generally have more found to be effective in patients with fast channel
severe clinical manifestations. The clinical symp- syndrome [54, 57, 70].
toms usually begin in childhood, adolescence or
adulthood. The common clinical presentations
include hypotonia, ptosis, ophthalmoplegia, efects of the Acetylcholine Receptor
D
weakness, skeletal deformities (e.g., arthrogry- Complex
posis, lordosis, or scoliosis), muscular atrophy,
dysphagia, and respiratory difficulty [67]. The The acetylcholine receptor in the postsynaptic
diagnosis is confirmed by 2–3 Hz RNS, which membrane is organized and clustered with the
shows decremental response and molecular anal- help of other structural proteins such as rapsyn,
ysis of acetylcholine receptor [57]. They often Dok-7, and MuSK. The pathophysiology of some
respond to treatments that increase acetylcholine CMS subtypes is related to reduced expression or
receptor activation including AChE inhibitors functionality in the acetylcholine receptor sec-
(e.g., pyridostigmine) and 3,4-Diaminopyridine ondary to an alternation in these related structural
(3,4-DAP) [68]. proteins.
Kinetic abnormality of the ACh

receptor
PHYSIOLOGY:-
€ β δ γ subunit of ACh receptor is responsible for
opening and closing of ion channels
α, €- subunits responsible for affinity to acetylcholine in
ACh receptor.
Mutation to any of this subunits lead to following
disease manifestation
Slow channel Fast channel

syndrome syndrome
Autosomal dominant Autosomal recessive

inheritance pattern inheritance pattern
Pathophysiology: Pathophysiology:
Difficulty in closing ion Ion channels remain close
channels in the in the ACh receptor
ACh receptor
Clinical Features:
Clinical Features:
- Seen in neonates and infants
- Seen in adulthood
- Presents with ptosis,
- Involvement of neck and distal
ophthalmoplegia, dysphagia
limb (Upper more than
and weakness.
lower limbs)
- Easy fatigue
- As disease progresses, patient
may have muscle atrophy,
ptosis, and ophthalmoplegia.
ACh= Acetylcholine
Fig. 10.6 Pathophysiology and clinical features in patients with kinetic abnormality of the Acetylcholine receptor
DOK-7 Deficiency zation of acetylcholine receptors in the postsyn-

aptic membrane. Mutation of this gene results in
Patients with DOK-7 deficiency are noted to have the production of abnormal agrin which causes a
weakness that predominately affects the proxi- failure of neuromuscular transmission. The clini-
mal muscles compared to distal muscle groups. cal features include ophthalmoplegia, ptosis, and
They are also regarded as limb-girdle myasthenic proximal muscle weakness [75]. Slow-rate RNS
syndromes for this reason [71]. Slow-rate recordings typically demonstrate a decremental
(2–3 Hz) RNS results in a decremental response [ response and post-exercise increment is com-
57]. This subgroup often does not respond to monly observed in the distal limb muscles.
drugs that would increase acetylcholine concen- Confirmation is based on muscle biopsy and
tration in the synaptic cleft, but they tend to AGRN gene mutation [57]. Albuterol is usually
respond to alpha adrenergic agonists such as beneficial in patients with this mutation [54].
ephedrine or albuterol [71, 72].
ther Gene Defects Associated
O
with Congenital Myasthenic Syndrome
Rapsyn Deficiency The centronuclear myopathies are a rare group of
muscle disorders due to mutations including
The mutation of Rapsyn protein leads to a defi- those in the BIN1, MTM1, and DNM2 genes
ciency in the acetylcholine receptor channels. Most [76–78]. These are reported to be associated with
often the clinical symptoms are associated with congenital myasthenic syndrome as well.
arthrogryposis and are typically neonatal in onset Electrophysiological features are also usually
[73]. RNS typically shows a decremental response consistent with impaired neuromuscular trans-
pattern at slow-ratestimulation (2–3 Hz) [57]. This mission manifested by decrement as in the other
subgroup of patients benefits from a combination subtypes aforementioned, and some of these
of acetylcholinesterase inhibitors (e.g. pyridostig- patients are reported to have some response to
mine), 3,4-Diaminopyridine and alpha adrenergic pyridostigmine [78].
agonists such as ephedrine or albuterol [71–73].
References
oltage-Gated Sodium Channel-
V
SCN4A Deficiency 1. Lang B, Vincent A. Autoimmune disorders of the
neuromuscular junction. Curr Opin Pharmacol. 2009
Jun;9(3):336–40.
This is a rare form of congenital myasthenic syn- 2. Nishimune H, Shigemoto K. Practical Anatomy of
drome associated with mutation of the SCN4A the Neuromuscular Junction in Health and Disease.
gene. This gene encodes for voltage gated sodium Neurol Clin. 2018 May;36(2):231–40.
3. Ganong WF. Review of medical physiology. 23rd ed.
channel in the muscle membrane. Patients with New York: McGraw-Hill Medical; 2010.
this mutation present with episodic weakness 4. Guyton AC. Textbook of medical physiology. 11th ed.
which involves axial, limb and respiratory mus- Philadelphia: Elsevier Saunders; 2006.
cles [74]. Treatment involves the combination of 5. Khurana I Textbook of medical physiology. Elsevier,
India; 2015.
acetylcholinesterase inhibitors and acetazol- 6. Gilhus NE, Verschuuren JJ. Myasthenia gravis: sub-
amide [54, 55]. group classification andtherapeutic strategies. Lancet
Neurol. 2015 Oct;14(10):1023–36.
7. Pakzad Z, Aziz T, Oger J. Increasing incidence of
myasthenia gravis amongelderly in British Columbia.
ther Rare Congenital Myasthenic
O Canada Neurol. 2011 Apr 26;76(17):1526–8.
Syndrome 8. Nicolle MW. Myasthenia gravis and lambert-eaton
myasthenic syndrome. Continuum (Minneap Minn).
Agrin Deficiency 2016 Dec;22 (6, Muscle and Neuromuscular Junction
Disorders):1978–2005. Review.
Agrin is secreted from the presynaptic membrane 9. Renton AE, Pliner HA, Provenzano C, Evoli
and is responsible for the clustering and organi- A, Ricciardi R, Nalls MA, et al. A genome-
wideassociation study of myasthenia gravis. JAMA gle fiber EMG evaluation of adults with suspected
Neurol. 2015 Apr;72(4):396–404. myasthenia gravis or Lambert-Eaton myasthenic
10. Meriggioli MN, Sanders DB. Autoimmune myasthe- syndrome: summary statement. Muscle Nerve. 2001
nia gravis: emerging clinical and biological heteroge- Sep;24(9):1236–8.
neity. Lancet Neurol. 2009;8:475–99. 27. Litchy WJ, Albers JW Repetitive stimulation an
11. Querol L, Illa I. Myasthenia and the neuromuscular AANEM workshop. https://www.aanem.org/mxon-
junction. Curr Opin Neurol. 2013;26:459–65. line/resources/downloads/products/REPS.pdf Used
12. Skeie GO, Apostolski S, Evoli A, et al. Guidelines for on 07/25/2018
treatment of autoimmune neuromuscular transmission 28. Costa J, Evangelista T, Conceição I, de Carvalho
disorders. Eur J Neurol. 2010;17:893–902. M. Repetitive nerve stimulation in myasthenia
13. Rodolico C, Toscano A, Autunno M, et al. Limb- gravis--relative sensitivity of different muscles.
girdle myasthenia; clinical, electrophysiological and ClinNeurophysiol. 2004 Dec;115(12):2776–82.
morphological features in familial and autoimmune 29. Chiou-Tan FY. Electromyographic approach to
cases. Neuromuscul Disord. 2002;12:964–9. neuromuscular junction disordersrepetitive nerve
14. Zhang B, Tzartos JS, Viegas S, et al. Autoantibodies stimulation and single-fiber electromyography. Phys
to lipoprotein-related protein 4 in patients with Med Rehabil Clin N Am. 2003 May;14(2):387–401.
double-negative myasthenia gravis. Arch Neurol. Review
2012;69:445–51. 30. Sarrigiannis PG, Kennett RP, Read S, Farrugia
15. Leite MI, Jacob S, Viegas S, et al. IgG1 antibodies to ME. Single-fiber EMG with aconcentric needle elec-
acetylcholine receptors in “seronegative” myasthenia trode: validation in myasthenia gravis. Muscle Nerve.
gravis. Brain. 2008;131:1940–52. 2006 Jan;33(1):61–5.
16. Jacob S, Viega S, Leite MI. Presence and pathogenic 31. Srivastava A, Kalita J, Misra UK. A comparative
relevance of antibodies to clustered acetylcholine study of single fiberelectromyography and repetitive
receptor in ocular and generalized myasthenia gravis. nerve stimulation in consecutive patients withmyas-
Arch Neurol. 2012;69:994–1001. thenia gravis. Electromyogr Clin Neurophysiol. 2007
17. Gasperi C, Melms A, Schoser B, et al. Anti-agrin Mar-Apr;47(2):93–6.
autoantibodies in myasthenia gravis. Neurology. 32. Gilhus NE. Myasthenia gravis. N Engl J Med. 2016
2014;82:1976–83. Dec 29;375(26):2570–81.
18. Gallardo E, Martinez-Hernandez E, Titulaer MJ, 33. Kim JY, Park KD, Richman DP. Treatment of myas-
et al. Cortactin autoantibodies in myasthenia gravis. thenia gravis based on its immunopathogenesis. J Clin
Autoimmun Rev. 2014;13:1003–7. Neurol. 2011 Dec 1;7(4):173–83.
19. Kerty E, Elsais A, Argov Z, Evoli A, Gilhus 34. Kesner VG, Oh SJ, Dimachkie MM, Barohn
NE. EFNS/ENS guidelines for the treatment of ocular RJ. Lambert-eaton myasthenic syndrome. Neurol
myasthenia gravis. Eur J Neurol. 2014;21:687–93. Clin. 2018 May 31;36(2):379–94.
20. Chatzistefanou KI, Kouris T, Iliakis E, Piaditis G, 35. Tarr TB, Wipf P, Meriney SD. Synaptic patho-
Tagaris G, Katsikeris N, Kaltsas G, Apostolopoulos physiology and treatment of Lambert-Eaton
M. The ice pack test in the differential diagno- myasthenic syndrome. Mol Neurobiol. 2015 Aug
sis ofmyasthenic diplopia. Ophthalmology. 2009 1;52(1):456–63.
Nov;116(11):2236–43. 36. Nicole S, Azuma Y, Bauché S, Eymard B, Lochmüller
21. Oh SJ, Cho HK. Edrophonium responsiveness not H, Slater C. Congenital Myasthenic syndromes or
necessarily diagnostic of myasthenia gravis. Muscle inherited disorders of neuromuscular transmission:
Nerve. 1990 Mar;13(3):187–91. recent discoveries and open questions. J Neuromuscul
22. Pasnoor M, Dimachkie M, Farmakidis’s C, Barohn Dis. 2017;(Preprint):1–6.
R. Diagnosis of myasthenia gravis. Neurol Clin. 37. Titulaer MJ, Verschuuren JJ. Lambert–Eaton myas-
2018;36(2):261–74. thenic syndrome. Ann N Y Acad Sci. 2008 Jun
23. Albuquerque EX, Rash JE, Mayer RF, Satterfield 1;1132(1):129–34.
JR. An electrophysiological andmorphological study 38. O’Neill JH, Murray NM, Newsom-Davis J. The
of the neuromuscular junction in patients with myas- Lambert-Eaton myasthenic syndrome. A review of 50
theniagravis. Exp Neurol. 1976 Jun;51(3):536–63. cases. Brain. 1988 Jun;111(Pt 3):577–96.
24. Albuquerque EX, Warnick JE, Mayer RF, Eldefrawi 39. Wirtz PW, Willcox N, van der Slik AR, Lang B,
AT, Eldefrawi ME. Recentadvances in the molecular Maddison P, Koeleman BP, Giphart MJ, Wintzen
mechanisms of human and animal models of myas- AR, Roep BO, Verschuuren JJ. HLA and smoking in
theniagravis. Ann N Y Acad Sci. 1981;377:496–518. prediction and prognosis of small cell lung cancer in
25. Preston DC, Shapiro BE. Electromyography autoimmune Lambert-Eaton myasthenic syndrome. J
and neuromuscular disorders e-book: clinical- Neuroimmunol. 2005 Feb;159(1–2):230–7.
electrophysiologic correlations (expert consult- 40. Wirtz PW, Smallegange TM, Wintzen AR, Verschuuren
online). Elsevier Health Sciences; 2012 Nov 1. JJ. Differences in clinical features between the
26. AAEM Quality Assurance Committee. American Lambert-Eaton myasthenic syndrome with and with-
Association of Electrodiagnostic Medicine. Practice out cancer: an analysis of 227 published cases. Clin
parameter for repetitive nerve stimulation and sin- Neurol Neurosurg. 2002 Sep;104(4):359–63.
41. Payne M, Bradbury P, Lang B, Vincent A, Han C, the neuromuscular junction. Ann N Y Acad Sci.
Newsom-Davis J, Talbot D. Prospective study into 2008;1132:99e103.
the incidence of Lambert Eaton myasthenic syndrome 55. Engel AG. The therapy of congenital myasthenic syn-
in small cell lung cancer. J Thorac Oncol. 2010 Jan dromes. Neurotherapeutics. 2007 Apr 1;4(2):252–7.
1;5(1):34–8. 56. Barišić N, Chaouch A, Müller JS, Lochmüller
42. Titulaer MJ, Maddison P, Sont JK, et al. Clinical H. Genetic heterogeneity and pathophysiological
dutch-english lambert-eaton myasthenic syndrome mechanisms in congenital myasthenic syndromes.
(LEMS) tumor association prediction score accu- Eur J Paediatr Neurol. 2011 May 1;15(3):189–96.
rately predicts small-cell lung cancer in the LEMS. J 57. Shieh PB, Oh SJ. Congenital Myasthenic Syndromes.
Clin Oncol. 2011 Mar 1;29(7):902–8. Neurol Clin. 2018 May 31;36(2):367–78.
43. Lennon VA, Kryzer TJ, Griesmann GE, O’Suille 58. Lorenzoni PJ, Scola RH, Kay CS, Werneck
abhain PE, Windebank AJ, Woppmann A, Miljanich LC. Congenital myasthenic syndrome: a brief review.
GP, Lambert EH. Calcium-channel antibodies in the Pediatr Neurol. 2012 Mar 1;46(3):141–8.
Lambert-Eatonsyndrome and other paraneoplastic 59. Ohno K, Tsujino A, Brengman JM, Harper CM,
syndromes. N Engl J Med. 1995;332(22):1467–74. Bajzer Z, Udd B, Beyring R, Robb S, Kirkham FJ,
44. Titulaer MJ, Lang B, Verschuuren JJ. Lambert-Eaton Engel AG. Choline acetyltransferase mutations
myasthenic syndrome: fromclinical characteris- cause myasthenic syndrome associated with epi-
tics to therapeutic strategies. Lancet Neurol. 2011 sodic apnea in humans. Proc Natl Acad Sci. 2001 Feb
Dec;10(12):1098–107. 13;98(4):2017–22.
45. Titulaer MJ, Lang B, Verschuuren JJ. Lambert-Eaton 60. Byring RF, Pihko H, Tsujino A, Shen XM, Gustafsson
myasthenic syndrome: fromclinical characteris- B, Hackman P, Ohno K, Engel AG, Udd B. Congenital
tics to therapeutic strategies. Lancet Neurol. 2011 myasthenic syndrome associated with episodic apnea
Dec;10(12):1098–107. and sudden infant death. Neuromuscul Disord. 2002
46. Oh SJ, Kurokawa K, Claussen GC, Ryan HF Jr. Aug 1;12(6):548–53.
Electrophysiological diagnosticcriteria of Lambert- 61. Maselli RA, Chen D, Mo D, Bowe C, Fenton G,
Eaton myasthenic syndrome. Muscle Nerve. 2005 Wollmann RL. Choline acetyltransferase mutations
Oct;32(4):515–20. in myasthenic syndrome due to deficient acetylcho-
47. Motomura M, Lang B, Johnston I, Palace J, Vincent line resynthesis. Muscle Nerve: Official Journal of the
A, Newsom-Davis J. Incidence of serum anti-P/O-- American Association of Electrodiagnostic Medicine.
type and anti-N-type calcium channel autoantibodies 2003 Feb;27(2):180–7.
in theLambert-Eaton myasthenic syndrome. J Neurol 62. Schara U, Christen HJ, Durmus H, Hietala M,
Sci. 1997 Mar 20;147(1):35–42. Krabetz K, Rodolico C, Schreiber G, Topaloglu H,
48. Sabater L, Titulaer M, Saiz A, Verschuuren J, Güre Talim B, Voss W, Pihko H. Long-term follow-up in
AO, Graus F. SOX1 antibodies are markers of para- patients with congenital myasthenic syndrome due
neoplastic Lambert-Eaton myasthenic syndrome. to CHAT mutations. Eur J Paediatr Neurol. 2010 Jul
Neurology. 2008 Mar 18;70(12):924–8. 1;14(4):326–33.
49. Tim RW, Massey JM, Sanders DB. Lambert-Eaton 63. Donger C, Krejci E, Serradell AP, Eymard B, et al.
myasthenic syndrome: electrodiagnostic findings Mutation in the humanacetylcholinesterase-asso-
and response to treatment. Neurology. 2000 Jun ciated collagen gene, COLQ, is responsible for-
13;54(11):2176–8. congenital myasthenic syndrome with end-plate
50. Oh SJ, Kim DS, Head TC, Claussen GC. Low-dose acetylcholinesterase deficiency(Type Ic). Am J Hum
guanidine and pyridostigmine: relatively safe and Genet. 1998 Oct;63(4):967–75.
effective long-term symptomatic therapy in Lambert- 64. Aldunate R, Casar JC, Brandan E, Inestrosa
Eaton myasthenic syndrome. Muscle Nerve. 1997 NC. Structural and functional organization of syn-
Sep;20(9):1146–52. aptic acetylcholinesterase. Brain Res Rev. 2004 Dec
51. Bain PG, Motomura M, Newsom-Davis J, Misbah 1;47(1–3):96–104.
SA, et al. Effects of intravenous immunoglobulin 65. Bestue-Cardiel M, de Cabezón-Alvarez AS,
on muscle weakness andcalcium-channel autoanti- Capablo-Liesa JL, López-Pisón J, Peña-Segura JL,
bodies in the Lambert-Eaton myasthenic syndrome. Martin-Martinez J, Engel AG. Congenital endplate
Neurology. 1996 Sep;47(3):678–83. acetylcholinesterase deficiency responsive to ephed-
52. Beeson D, Hantai D, Lochmuller H, Engel AG. rine. Neurology. 2005 Jul 12;65(1):144–6.
126th international workshop: congenital myasthenic 66. Unwin N. Structure and action of the nicotinic ace-
syndromes, 24–26 September 2004, Naarden, The tylcholine receptor explored by electron microscopy.
Netherlands. Neuromuscul Disord. 2005;15:498–512. FEBS Lett. 2003 Nov 27;555(1):91–5.
53. Kinali M, Beeson D, Pitt MC, et al. 67. Palace J, Beeson D. The congenital myasthenic syn-
Congenitalmyasthenic syndromesin child- dromes. J Neuroimmunol. 2008 Sep 15;201–202:2–5.
hood: Diagnostic and management challenges. J 68. Liewluck T, Selcen D, Engel AG. Beneficial effects
Neuroimmunol. 2008;201:6e12. of albuterol in congenital endplate acetylcholinester-
54. Beeson D, Webster R, Cossins J, et al. Congenital ase deficiency and Dok-7 myasthenia. Muscle Nerve.
myasthenic syndromes and the formation of 2011 Nov;44(5):789–94.
69. Harper CM, Engel AG. Quinidine sulfate therapy for Stojkovic T. Phenotype of a patient with recessive
the slow-channel congenitalmyasthenic syndrome. centronuclear myopathy and a novel BIN1 mutation.
Ann Neurol. 1998 Apr;43(4):480–4. Neurology. 2010 Feb 9;74(6):519–21.
70. Engel AG, Shen XM, Selcen D, Sine SM. What 78. Robb SA, Sewry CA, Dowling JJ, Feng L, Cullup T,
have we learned from the congenital myasthenic Lillis S, Abbs S, Lees MM, Laporte J, Manzur AY,
syndromes. J Mol Neurosci. 2010 Jan;40(1–2): Knight RK. Impaired neuromuscular transmission
143–53. and response to acetylcholinesterase inhibitors in cen-
71. Selcen D, Milone M, Shen XM, Harper CM, Stans tronuclear myopathies. Neuromuscul Disord. 2011
AA, Wieben ED, Engel AG. Dok-7 myasthenia: phe- Jun 1;21(6):379–86.
notypic and molecular genetic studies in 16 patients.
Ann Neurol: Official Journal of the American Suggested Reading
Neurological Association and the Child Neurology
Society. 2008 Jul;64(1):71–87.
Selvan VA. Single-fiber EMG: a review. Ann Indian Acad
72. Schara U, Barisic N, Deschauer M, Lindberg C,
Neurol. 2011 Jan;14(1):64.
Straub V, Strigl-Pill N, Wendt M, Abicht A, Müller
Liveson JA, Ma DM. Laboratory reference for clinical
JS, Lochmüller H. Ephedrine therapy in eight
neurophysiology. Oxford: Oxford University Press;
patients with congenital myasthenic syndrome due
1992.
to DOK7 mutations. Neuromuscul Disord. 2009 Dec
Jabre JF, Chirico-Post J, Weiner M. Stimulation SFEMG
1;19(12):828–32.
in myasthenia gravis. Muscle Nerve: Official Journal
73. Ohno K, Engel AG, Shen XM, Selcen D, Brengman J,
of the American Association of Electrodiagnostic
Harper CM, Tsujino A, Milone M. Rapsyn mutations
Medicine. 1989 Jan;12(1):38–42.
in humans cause endplate acetylcholine-receptor defi-
Sanders DB. Clinical impact of single-fiber electromyog-
ciency and myasthenic syndrome. Am J Hum Genet.
raphy. Muscle Nerve. 2002;Suppl 11:S15–20.
2002 Apr 1;70(4):875–85.
Bromberg MB, Scott DM. Single fiber EMG refer-
74. Habbout K, Poulin H, Rivier F, Giuliano S, Sternberg
ence values: reformatted in tabular form. AD HOC
D, Fontaine B, Eymard B, Morales RJ, Echenne B,
Committee of the AAEM Single Fiber Special Interest
King L, Hanna MG. A recessive Nav1. 4 mutation
Group. Muscle Nerve. 1994;17(7):820–1.
underlies congenital myasthenic syndrome with peri-
Howard JF Jr. Electrodiagnosis of disorders of neuromus-
odic paralysis. Neurology. 2016 Jan 12;86(2):161–9.
cular transmission. Phys Med Rehabil Clin N Am.
75. Huzé C, Bauché S, Richard P, Chevessier F, Goillot
2013 Feb;24(1):169–92.
E, Gaudon K, Ammar AB, Chaboud A, Grosjean I,
Titulaer MJ, Klooster R, Potman M, et al. SOX antibodies
Lecuyer HA, Bernard V. Identification of an agrin
in small-cell lung cancer and Lambert-Eaton myas-
mutation that causes congenital myasthenia and
thenic syndrome: frequency and relation with sur-
affects synapse function. Am J Hum Genet. 2009 Aug
vival. J Clin Oncol. 2009 Sep 10;27(26):4260–7.
14;85(2):155–67.
Molgó J, Lundh H, Thesleff S. Potency of
76. Gibbs EM, Clarke NF, Rose K, Oates EC, Webster
3,4-
diaminopyridine and4-aminopyridine on
R, Feldman EL, Dowling JJ. Neuromuscular junction
mammalian neuromuscular transmission and the
abnormalities in DNM2-related centronuclear myopa-
effect of pHchanges. Eur J Pharmacol. 1980 Jan
thy. J Mol Med. 2013 Jun 1;91(6):727–37.
11;61(1):25–34.
77. Claeys KG, Maisonobe T, Böhm J, Laporte J, Hezode
M, Romero NB, Brochier G, Bitoun M, Carlier RY,
Single Fiber EMG
11
Alexandra Soriano
Single Fiber Electromyography done correctly by an experienced electromyogra-

pher. These needles have a lower cost and are dis-
The technique of single fiber EMG (SFEMG) is posable, eliminating the need to sterilize the
mainly used to help with the diagnosis of myas- needle electrode or to maintain the needle. It is
thenia gravis when there is high clinical suspi- recommended to increase the high pass filter to
cion and other tests, including acetylcholine ~1000 Hz for better results. To the extent possi-
receptor, muscle specific tyrosine kinase (MuSK) ble, a needle electrode with the smallest electrode
antibodies and EMG with repetitive nerve stimu- surface should be used (e.g. 0.019 mm2).
lation have been negative. The single fiber EMG results can be obtained
This electrodiagnostic technique was developed by using one of two techniques:
in the 1960s by Erik Stalbërg and Jan Ekstedt with
the purpose of obtaining action potentials from a 1. Stimulation SFEMG, in where individual
single muscle fiber [1]. For optimal results with the motor fibers or motor axons can be activated
single fiber EMG technique, a special concentric using an intramuscular axonal stimulator in
needle electrode with a small recording surface of the form of a monopolar needle electrode
25 microns in diameter located 3 mm from the tip is positioned near the motor end-plate zone [1],
used, so that it can selectively capture these action which serves as the cathode, and a small sur-
potentials from individual muscle fibers (see face electrode which serves as the anode.
Figs. 11.1 and 11.2). The use of a high pass filter of 2. Voluntary activation SFEMG, in which the
500 Hz when recording the action potentials further patient voluntarily activates the muscle to be
helps with the selectivity [3]. studied.
Acceptable action potentials are those with an
amplitude of 200 microvolts or more. In either technique, the goal is to measure two
More recently, monopolar and concentric nee- parameters, the neuromuscular jitter and the pres-
dles have also been used to perform SFEMG, and ence of neuromuscular blocking.
reliable results can be obtained if the technique is
A. Soriano (*) Voluntary Activity SFEMG

Florida, Weston, FL, USA As the patient does minimal contraction of the
Cleveland Clinic Lerner College of Medicine of Case muscle to be examined, the SFEMG electrode is
Western Reserve University, Cleveland, OH, USA inserted into the muscle, preferably in the middle
e-mail: soriana@ccf.org
third of its length. The electrode is then placed in a
https://doi.org/10.1007/978-3-030-74997-2_11
250 A. Soriano
Fig. 11.1 Picture of a

concentric single fiber
electromyography
(SFEMG) needle. Note
the small recording
surface (see inset)
located 3 mm from the
tip
one from a single motor unit (Fig. 11.3). One action

Concentric potential triggers the display sweep, and the second
G1 (paired) action potential appears with slightly vari-
G2 able position for each discharge [4]. This variabil-
ity exists due to the changes in the transmission
time across the synaptic gap, or the time it takes for
end-plate potentials at the neuromuscular junction
Monopolar
to reach the action potentials threshold [1]. Once
G1 the SFEMG electrode is in position where these
action potentials are present, with adequate ampli-
tude, then a minimum of 50 discharges need to be
recorded. This technique is repeated several times,
Single Fiber
until a total of 20 action potential pairs, from differ-
G1 ent areas of the muscle, can be obtained. This may
G2
require a total of 3–4 skin insertions (aiming to
minimize the number of these).
Fig. 11.2 Illustration showing the relatively small
recording area (shaded) of the concentric single fiber elec- The jitter is then expressed as the mean value
tromyography (SFEMG) needle, compared to regular of consecutive differences (MCD) of successive
concentric and monopolar needle electrodes. G1, active interpotential intervals (IPIs), represented in
recording site; G2, reference site. [Used with permission Fig. 11.4 below.
from [2]]
Many of the modern electromyography
machines have the capability of calculating the
position where two (or at times more than two) MCD directly, though it is always advisable that
time-locked action potentials from the same motor the operator visually analyzes the signals that are
unit appear. Neuromuscular jitter is then recorded. being acquired to asses for poor triggering or dis-
The best recording is usually obtained from the turbing activity from other motor units and qual-
superficial layer of the muscle and with minimal ity of the signal.
muscle contraction (firing frequency of the motor When obtaining jitter measurement and action
unit between 8 and 15 discharges per second) [4]. potentials, errors can occur if the electrode is
The most used muscles for SFEMG include moved in relation to the fiber and the amplitude
the orbicularis oculi, extensor digitorum commu- of the action potential decreases, affecting the
nis and frontalis muscle. calculated jitter.
The voluntary activation SFEMG technique
requires more patient cooperation but is subject
to less technical errors or misinterpretations. ormal Jitters Findings and Values
N
With the needle in position, the neuromuscular with Voluntary Activation
jitter is obtained, which is described as the minimal
variability in latencies that exist between the The measurement of jitter varies from muscle to
appearance of one action potential and a second muscle and with age. As age increases, so does
11 Single Fiber EMG 251
Motor
Neuron
Muscle SFEMG
2
electrode
1 IPI 2
EPP2 Threshold
Fig. 11.3 Method of single fiber electromyography the neuromuscular junction, there may be marked vari-
(SFEMG) with voluntary activation. The SFEMG needle ability of the IPI (abnormal jitter). If severe, neuromuscu-
is inserted into voluntarily activated muscle and is posi- lar transmission failure may occur in which the EPP
tioned so that recordings are obtained from two or more amplitude fails to reach the threshold for action potential
single muscle fibers belonging to the same motor unit. generation. This is demonstrated here by the absence of
One single muscle fiber action potential serves as a time the second recorded fiber pair when the EPP in the second
reference and the interpotential interval (IPI) is measured muscle fiber (EPP2) is subthreshold (dotted lines and
after consecutive discharges between the reference poten- arrows). [Used with permission from [5]]
tial and subsequent time-locked potentials. In disorders of
IPI1 IPI2 IPI3
MCD= [IPI1-IP12]+[IPI2-IPI3]+...[IPIn-1-IPIn]
n-1
IPI= interpotential interval

MCD=mean value of consecutive differences
Fig. 11.4 Representation of interpotential interval (IPI) and calculation of mean value of consecutive differences
(MCD) in the determination of jitter
252 A. Soriano
jitter in normal subjects. Normal jitter values sis. Most patients cooperate well with this study
range from 5 to 65 microseconds, and is different and report relatively little discomfort. The
to each muscle. There exist predetermined refer- SFEMG needle must be in good condition, with a
ence jitter values for the most common muscles sharp tip that prevents more than minimal muscle
studied with the SFEMG technique. fiber damage.
The value of the interpotential interval (IPI) is Conditions that could limit SFEMG study
important and it is recommended that this stays include patients with limb tremor, in which the
below 4 milliseconds, as erroneous high jitter use of a facial muscle is generally preferred.
values can be obtained from recordings with long Another challenging patient subgroup includes
IPI [1]. sedated patients, uncooperative patients or chil-
The results of jitter measurements in each dren under 8 or infants, in which the use stimu-
muscle is presented as the mean or median value lated SFEMG is then preferred. A decrease in
of the MCD values in all the pairs or endplates intramuscular temperature below 35 °C can
measured; the percentage of paired potentials in increase the jitter in normal muscle, so such low
which blocking was present (given as percentage temperatures such be avoided.
of blocking); and the percentage of pairs in which
jitter exceeds the limit of normal for that particu-
lar muscle [3]. SFEMG in Myasthenia Gravis
For a study to be considered abnormal the fol-
lowing must be present: In patients with myasthenia gravis, the following
in a tested muscle may be found:
1. The mean (or median) jitter exceeds the upper
limit of normal for the muscle; or 1. Endplates with normal jitter values.
2. More than 10% of pairs have increased jitter 2. Endplates with jitter values above the normal
(including blocking). range without impulse blocking.
3. Endplates with increase jitter and intermittent
In general, when blocking is present jitter val- impulse blocking.
ues should already be abnormally increased. In
MG gravis for example, blocking occurs during Jitter is found to be increased in most patients
voluntary activation once jitter values exceed with myasthenia gravis, and this finding is more
80–100 μs. pronounced when weak muscles are tested but
A jitter value of 5 μS or less can be seen in can also be found in muscles that do not show
some cases of myopathies and rarely with volun- clinical weakness. A SFEMG tracing showing
tary activation in normal muscle, this probably marked jitter in a clinically affected muscle is
representing recording from split muscle fibers shown in Fig. 11.5 below. Jitter is abnormal in
activated by a single NMJ. These values should
not be counted for assessment of the neuromus-
cular transmission [3].
It is best to calculate mean MCD from indi-
vidual muscles with data of jitter values less than
150 μs, as this can significantly increase mean jit-
ter value, even if all other endplate potentials of
this muscle show normal jitter values. Increase
jitter, with or without blocking, can occasionally
occur in one of 20 pairs in normal muscle [4].
Overall for reliable results, SFEMG must be Fig. 11.5 Single fiber electromyography (SFEMG) with
performed by an electromyographer knowledge- high jitter in the frontalis muscle, 1 kHz high-pass filter-
able in the technique of data collection and analy- ing, 200 μV/D, 0.5 ms/D. [Used with permission from [6]]
11 Single Fiber EMG 253
Fig. 11.6 Single fiber electromyography (SFEMG) tech-

nique recording from the extensor digitorum (communis)
muscle. This is done by having the patient extend the
middle finger only while the needle is inserted parallel to
the muscle fibers
nique recording from the orbicularis oculus muscle. This
is done by having the patient close the eyes, minimally
squeezing the eyelids shut. The needle is inserted parallel
to the muscle fibers, directed away from the eyelid
margin
muscle weakness. This muscle can be abnormal in

about 85% of patients with MG during their initial
electrodiagnostic assessment. This muscle is pre-
ferred due to being relatively easy to activate, with
minimal patient discomfort and ease of finding
pairs of action potentials. With the patient elevat-
ing the middle finger minimally, the needle is
inserted parallel to the axis of the muscle fibers.
With respect to firing rate and jitter values, the
jitter is most likely to be increased when the fir-
nique recording from the frontalis muscle. This is done by
having the patient gently elevate eyebrows while the nee- ing rate is rapid in an endplate pair, compared to
dle is inserted parallel to the muscle fibers when it is firing slowly.
We prefer patients discontinue the use of cho-
~85% of patients with ocular MG, and in up to linesterase inhibitors, when clinically safe, at
~95–99% of patients with generalized MG. least 12 hours before the study. This may be more
The muscle to be tested should be selected useful in patients with ocular MG or in those with
depending on patients’ symptoms, and it is pref- minimal limb weakness as jitter can become
erable to select an unequivocally symptomatic abnormal only when these medications are dis-
muscle in order to increase the probability of continued. In other patients, jitter values can still
finding abnormal jitter. We prefer the extensor be increase even when they continue to take the
digitorum (communis) (Fig. 11.6) in patients cholinesterase inhibitors.
with generalized myasthenia gravis, and the fron- If SFEMG is done in a clinically weak muscle,
talis (Fig. 11.7) or orbicularis oculus (Fig. 11.8) and jitter is normal, then the diagnosis is almost
in patients with primarily ocular symptoms with surely not MG.
or without generalized weakness. In MG, jitter may not be present during initial
The extensor digitorum is mostly tested first, recording of muscle activation, and measurement
and preferable in patients with limb or with bulbar may need to be made for several minutes for jitter
254 A. Soriano
to become abnormal. This does not occur in 8. Kouyoumdjian JA, Stalberg EV. Reference jitter val-
ues for concentric needle electrodes in voluntarily
healthy muscle, as jitter remains stable even with activated extensor digitorum communis and orbicu-
prolonged activation. laris oculi muscles. Muscle Nerve. 2008;37:694–9.
Jitter values do not generally correlate well 9. Sanders DB, Howard JF. Jr AAEE Minimonograph
with disease severity, but these values could #25: single-fiber electromyography in myasthenia
gravis. Muscle Nerve. 1986;9:809–19.
potentially be used to monitor patients with MG, 10. Stålberg E, Sanders DB, Kouyoumdjian JA. Pitfalls
in which jitter becomes abnormal over time indi- and errors in measuring jitter. Clin Neurophysiol.
cating a possible clinical exacerbation. 2017;128(11):2233–41. https://doi.org/10.1016/j.
Abnormally increased jitter does not only clinph.2017.09.001.
11. Stålberg E, Sanders DB, Ali S, Cooray G, Leonardis
occur in MG and can also be found in Lambert L, Löseth S, Machado F, Maldonado A, Martinez-
Eaton myasthenic syndrome (LEMS), polyneu- Aparicio C, Sandberg A, Smith B, Widenfalk J,
ropathies or motor neuron disorders. Therefore, it Kouyoumdjian JA. Reference values for jitter recorded
is very important to do nerve conduction studies by concentric needle electrodes in healthy controls: a
multicenter study. Muscle Nerve. 2015;53(3):351–62.
(including repetitive nerve stimulation) and rou- 12. Chiou-Tan FY, Gilchrist JM. Repetitive nerve stimu-
tine EMG in patients prior to SFEM, in order to lation and single-fiber electromyography in the evalu-
exclude these diagnoses. In cases of LEMS, the ation of patients with suspected myasthenia gravis
jitter will be increased out of proportion to the or Lambert–Eaton myasthenic syndrome: review of
recent literature. Muscle Nerve. 2015;52(3):455–62.
severity of muscle weakness, and impulse block- 13. Kouyoumdjian JA, Stålberg EV. Concentric needle jit-
ing is commonly found. In polyneuropathies, the ter on voluntary activated frontalis in 20 healthy sub-
jitter can be increased during reinnervation, and jects. Muscle Nerve. 2013;47(3):440–2.
later normalizes or reduces. 14. Juel VC. Evaluation of neuromuscular junction disor-
ders in the electromyography laboratory. Neurol Clin.
2012;30(2):621.
15. Sanders DB, Howard JF, Johns TR. Single-fiber elec-
References/Suggested Reading tromyography in myasthenia gravis. Neurology. 1979
Jan;29(1):68.
1. Sanders DB, Stålberg EV. AAEM minimonograph 16. Machado FCN, Kouyoumdjian JA, Marchiori
#25: single-fiber electromyography. Muscle Nerve. PE. Diagnostic accuracy of concentric needle jit-
1996;19:1069–83. ter in myasthenia: prospective study. Muscle Nerve.
2. Rubin DI. Needle electromyography: basic concepts 2016;55(2):190–4.
and patterns of abnormalities. Neurol Clin. 2012 17. Baruca M, Leonardis L, Podnar S, Hojs-Fabjan T,
May;30(2):429–56. Grad A, Jerin A, Blagus R, Šega-Jazbec S. Single
3. Stalberg E. Clinical electrophysiology in myasthenia fiber EMG as a prognostic tool in myasthenia gravis.
gravis. J Neurol Neurosurg Psychiatry. 1980;43:622–33. Muscle Nerve. 2016;54(6):1034–40.
4. Selvan VA. Single-fiber EMG: a review. Ann Indian 18. Kouyoumdjian JA, Stålberg EV. Concentric needle jit-
Acad Neurol. 2011;14(1):64–7. ter on voluntary activated frontalis in 20 healthy sub-
5. Meriggioli MN, Sanders DB. Myasthenia gravis: jects. Muscle Nerve. 2013;47(3):440–2.
diagnosis. Semin Neurol. 2004 Mar;24(1):31–9. 19. Stålberg E. Jitter analysis with concentric needle elec-
6. Sirin NG, Kocasoy Orhan E, Durmus H, Deymeer trodes. Ann N Y Acad Sci. 2012;1274(1):77–85.
F, Baslo MB. Repetitive nerve stimulation and jitter 20. Chenevier F, Gervais-Bernard H, Bouhour F, Vial
measurement with disposable concentric needle elec- C. Myasthénies et syndromes myasthéniques. EMC
trode in newly diagnosed myasthenia gravis patients. Neurol. 2011;8(2):1.
Neurophysiol Clin. 2018 Oct;48(5):261–7. 21. Tidswell T, Pitt MC. A new analytical method to
7. Cherian A, Baheti NN, Iype T. Electrophysiological diagnose congenital myasthenia with stimulated
study in neuromuscular junction disorders. Ann single-fiber electromyography. Muscle Nerve.
Indian Acad Neurol. 2013;16:134–41. 2006;35(1):107–10.
Myopathies
12
Payam Soltanzadeh
Etiology and Clinical Features presentation. Very high serum levels of creatine
kinase (CK) can be a good clue to the diagnosis
Myopathies or muscle diseases are classified into of a muscle disease; however, depending on the
acquired or genetic disease processes, which pri- type and the stage of myopathy, CK can be either
marily affect the function and/or structure of the normal or only slightly elevated. Neurogenic pro-
muscle tissue ultimately leading to various mani- cesses like motor neuron disease, motor radicu-
festations such as muscle weakness, neuromus- lopathies and some neuropathies can also be
cular hyperexcitability (including myotonia, associated with slightly elevated CK levels. There
cramps and myalgia), and physical fatigability. are a few myopathies that can cause very high
Due to recent advances in molecular genetics and CK, which can help with the diagnosis
immunology and significant clinical and patho- (Table 12.2).
logical overlap among various muscle diseases,
classification of myopathies is evolving and
genetic and immunologic biomarkers are being Differential Diagnosis
used to further refine previous classifications that
were purely based on clinical and pathologic cri- Subacute or chronic development of symmetric
teria (Table 12.1). Most myopathies primarily weakness in proximal muscles should raise
cause symmetrical proximal muscle weakness concern for a myopathic process; however, in
but there are muscle diseases, like sporadic inclu- the neuromuscular category of neurologic dis-
sion body myositis (sIBM) or myotonic dystro- eases, some forms of motor neuron disease like
phy type 1, that can primarily or initially affect late-onset spinal muscular atrophy (SMA) or
distal muscle groups and, later, involve the proxi- atypical amyotrophic lateral sclerosis (ALS),
mal muscles. Symmetry is also not a universal bilateral polyradiculopathies or polyradiculo-
characteristic of myopathies as some myopathies neuropathies like Chronic inflammatory demy-
like facioscapulohumeral muscular dystrophy elinating polyradiculoneuropathy (CIDP),
(FSHD) or sIBM can have a very asymmetric motor predominant neuropathies, disorders of
the neuromuscular junction like myasthenic
syndromes, botulism or Lambert-Eaton myas-
thenic syndrome (LEMS) may also present
P. Soltanzadeh (*) with prominent proximal weakness in a rela-
Neuromuscular Program, Department of Neurology,
UCLA, Los Angeles, CA, USA tively symmetrical fashion. If a neuromuscular
e-mail: psoltanzadeh@mednet.ucla.edu process presents asymmetrically, differential

https://doi.org/10.1007/978-3-030-74997-2_12
256 P. Soltanzadeh
Table 12.1 General classification of myopathies

Acquired myopathies:
– Inflammatory or non-inflammatory necrotizing autoimmune myopathies: myositis associated with a specific
antibody (anti-SRP, anti-HMGCR, anti-Jo, etc.), dermatomyositis, polymyositis
– Sporadic inclusion body myositis (sIBM)
– Granulomatous (sarcoid) myopathy
– Amyloid myopathy
– Drug-induced or toxic myopathies: self-limited statin myopathy, myopathy caused by steroids, colchicine, or alcohol
– Endocrine myopathies: thyroid or parathyroid disorders, Cushing’s syndrome, vitamin D deficiency/
osteomalacia, hypokalemia
– Infectious myopathies: trichinosis, cysticercosis, toxoplasmosis, HIV, coxsackie, influenza, SARS-CoV-2,
Lyme disease, staphylococcus aureus (pyomyositis)
– Critical illness (myosin loss) myopathy
Genetic myopathies:
– Muscular Dystrophies:
– Dystrophinopathies (X-linked): DMD
– Emery-Dreifuss muscular dystrophy: EMD, FHL1, LMNA
– Facioscapulohumeral muscular dystrophy (FSHD) types 1 and 2: DUX4, SMCHD1
– Limb-girdle muscular dystrophies (LGMDs): many genes
– Congenital muscular dystrophies:
– Congenital muscular dystrophy with merosin (LAMA2) deficiency
– Collagen VI diseases (Bethlem and Ullrich)
– Rigid spine syndrome: SEPN1, FHL1, LMNA, DNM2 (dynamin 2)
– Congenital muscular dystrophies due to defective glycosylation (Fukuyama, Walker-Warburg, muscle-eye-
brain disease)
– Congenital myopathies:
– Nemaline myopathies: TPM3, TPM2, NEB, ACTA1 (actin alpha 1)
– Congenital myopathy with fiber-type disproportion (CFTD): ACTA1, SEPN1, TPM3, RYR1, MYH7
– Myotubular myopathy: MTM1 (myotubularin 1)
– Centronuclear myopathies: DNM2 (dynamin 2), BIN1 (amphiphysin), RYR1, TTN
– Central core disease: RYR1
– Multiminicore disease (classic form): SEPN1
– Cap myopathy: TPM2, TPM3, ACTA1
– Distal Myopathies
– Miyoshi: DYSF
– Tibial muscular dystrophy (Udd): TTN
– Hereditary IBM and Nonaka (now called GNE myopathy): GNE (glucosamine (UDP-N-acetyl)-2-epimerase/
N-acetylmannosamine kinase)
– Distal myopathy with VCP defect (IBMPFD): VCP
– Laing Distal myopathy: MYH7
– Welander distal myopathy: TIA1 (cytotoxic granule-associated RNA binding protein)
– Myopathies due to mutant Z-disk associated proteins or Myofibrillar myopathies: CRYAB (alpha B crystallin),
DES (desmin), LDB3 (ZASP), MYOT (myotilin)
– Oculopharyngeal muscular dystrophy (OPMD)
– Myotonic dystrophies
– Myotonic dystrophy type 1 (DM-1) or Steinert’s disease: DMPK
– Myotonic dystrophy type 2 (DM-2) or PROMM: ZNF9
– Other Myotonic syndromes (Schwartz-Jampel syndrome, etc)
– Channelopathies (include nondystrophic myotonic syndromes/periodic paralyses)
– Chloride channel: autosomal dominant myotonia congenita (Thomsen); autosomal recessive (Becker): CLCN1
– Sodium channel: hyperKPP, hypoKPP, paramyotonia congenita: SCN4A
– Calcium channel: hypoKPP1: CACNA1S
– Potassium channel: hypoKPP3: KCNE3; thyrotoxic hypokalemic: KCNJ18
– Metabolic myopathies
– glycogen storage diseases: type II (Pompe disease): GAA (acid alpha glucosidase/acid maltase), type V
(McArdle): PYGM (myophosphorylase)
– glycolytic pathway
– disorders of lipid metabolism: carnitine palmitoyltransferase deficiency: CPT2
– Mitochondrial myopathies
12 Myopathies 257
Table 12.2 Myopathies with very high CK levels (typi- Table 12.3 Causes of neuromyopathy (presenting with
cally, in the thousands U/L) both myopathy and neuropathy)
– Some inflammatory or necrotizing autoimmune – HIV
myopathies – Sarcoidosis
– Dystrophinopathies (Duchenne and Becker – Amyloidosis
muscular dystrophies) – Paraneoplastic neuromyopathy
– LGMD 2A (calpainopathy) – Mitochondrial neuromyopathy
– LGMD 2B (dysferlinopathy) – Toxic neuromyopathies (alcohol abuse, colchicine,
– LGMD 2C-2F (sarcoglycanopathies) hydroxychloroquine etc)
– LGMD 2I (Fukutin-related proteinopathy) – Critical illness neuromyopathy
– LGMD 2L (anoctaminopathy) – Graft-versus-host disease (GVHD)
diagnosis extends to multifocal motor neuropa- Electrodiagnostic Evaluation

thy with conduction block (MMN), u nilateral of Myopathies
motor radiculopathies, plexopathies, and mono-
neuropathies. Other clinical and laboratory fea- EDX work up is an important component of the
tures especially the electrodiagnostic (EDX) assessment of a patient with suspected myopathy.
findings can usually identify the cause of weak- Despite recent advances in molecular biology and
ness. In addition to the data from needle EMG genetics, EDX data can be helpful to differentiate
examination, nerve conduction study is an myopathic processes from the mimickers dis-
important component of EDX work up in a cussed above as well as to better characterize the
patient presenting with weakness. Although myopathic process. In some genetic myopathies
patients with myopathies may have a pre-exist- like Duchenne muscular dystrophy or myotonic
ing length-dependent neuropathy, presence of dystrophy type 1, if clinical presentation is classic
demyelinating features like slow motor conduc- or there is known family history, one could skip
tion, conduction block, or temporal dispersion EDX data and muscle biopsy and directly move
can indicate diseases like CIDP or MMN. on to genetic testing. If genetic test results are not
Significant loss of sensory amplitudes indicate consistent with the initial clinical hypothesis, a
pathology in the peripheral nerve or plexus. classic approach then needs to be pursued.
Presence of decrement or increment in com- Needle EMG findings can help diagnose and
pound muscle action potential amplitudes favors characterize a myopathy; however, it should be
a disease process involving the neuromuscular emphasized that a normal needle EMG study
junction. In patients with a neuromuscular junc- does not exclude a myopathic process. It is worth
tion transmission defect, fluctuation of muscle reminding that the EDX data may be considered
strength and involvement of the cranio-bulbar an extension to the clinical data and should be
segments help with the diagnosis; however, dis- interpreted within the patient’s clinical context.
eases like LEMS or limb-girdle congenital Needle EMG examination should follow the
myasthenic syndromes (including DOK7) may nerve conduction studies component of the EDX
not show prominent ocular or bulbar deficits and assessment and guided by the clinical presenta-
fluctuations may not be as notable as in typical tion, adequate nerve conduction studies should
myasthenia gravis. Some disease processes can be performed. To differentiate myopathy from a
damage both nerves and muscles (neuromyopa- polyradiculoneuropathy like CIDP or from
thies), hence creating a mixed neurogenic and MMN, adequate sensory and motor responses
myopathic picture (Table 12.3). In a pure myo- from both upper and lower extremities should be
pathic process, nerve conduction studies are assessed. These include one or two motor with
expected to be normal; however, myopathies corresponding F response(s) plus one sensory
that involve distal muscles can cause reduction conduction study from the lower extremity; and
of distal motor amplitudes, with preserved laten- one or two motor with corresponding F
cies and conduction velocities. response(s) plus one sensory conduction study
258 P. Soltanzadeh
from the upper extremity. In the lower extremity, comprehensive one “insertional/spontaneous
peroneal (fibular) and tibial motor and sural sen- activity”. In this chapter, we will refer to both as
sory responses are routinely checked and in the “spontaneous activity”. Increased spontaneous
upper extremity, median and ulnar motor and activity can develop in denervation or terminal
sensory responses are usually assessed. Radial motor axon injury, muscle membrane hyperexcit-
nerve studies can be useful in MMN affecting ability or muscle membrane injury, abnormal sig-
upper extremities. nal transmission between the nerve endings and
If a neuromuscular junction disorder is sus- the motor end plate, myofiber necrosis or split-
pected, repetitive nerve stimulation studies ting, inflammatory changes in the muscle tissue,
should be planned. Electrodiagnosis of LEMS and vacuolar myopathologies. Based on the pres-
can be difficult in the co-existence of a polyneu- ence or absence of increased spontaneous activity
ropathy. In the presence of low motor amplitudes, in needle EMG examinations, myopathies are
post-exercise measurements should be included electrodiagnostically categorized into two broad
in both upper and lower extremities. categories of “irritable myopathies” (Table 12.4)
Patients with myotonic dystrophies type 1 or and “non-irritable myopathies” (Table 12.5).
2, which are both systemic diseases, may have Irritable myopathies show positive sharp wave or
diabetes with a co-existing diabetic polyneuropa- fibrillation potentials on needle examination.
thy. The presence of polyneuropathy in these Positive sharp wave or fibrillation potentials are
cases may complicate the EDX findings but the not specific to myopathies and can be seen in
electromyographer should note myotonic dis- various neurogenic diseases as well as in botu-
charges and the myopathic EMG changes (dis- lism or following botulinum toxin injections. In
cussed below). non-irritable myopathies, spontaneous activity is
Short and long exercise tests have been normal. As noted in Tables 12.4 and 12.5, many
described in myotonic myopathies/periodic paral- myopathies can cause both irritable and non-irri-
yses. Availability of genetic testing for myotonic
dystrophies, non-dystrophic myotonic myopa- Table 12.4 Irritable myopathies (myopathic-type MUPs
thies and other skeletal muscle channelopathies, with abnormal spontaneous activity, including positive
sharp waves and fibrillation potentials)
as well as the uncomfortable technique and ques-
tionable diagnostic accuracy have made these Acquired muscle diseases
– Inflammatory or non-inflammatory necrotizing
tests of limited practical utility. Accordingly, we autoimmune myopathies
will defer discussing these tests in this chapter. – Sporadic inclusion body myositis (sIBM)
– Sarcoid myopathy
– Amyloid myopathy
– Toxic myopathies
Needle EMG Findings in Myopathies – Infectious myopathies
– Hypothyroid myopathy
Needle EMG findings in patients with myopathy – Critical illness (myosin loss) myopathy
fall into two major categories: the ones that are Genetic muscle diseases
– Dystrophinopathies
associated with abnormal insertional and sponta- – Other muscular dystrophies (MD): Emery-Dreifuss
neous activities, and those that are related to MD, Facioscapulohumeral muscular dystrophy (FSHD),
changes in motor unit potentials (MUPs). some Limb-girdle muscular dystrophies (LGMDs),
“Insertional activity” is referred to the activity Oculopharyngeal muscular dystrophy (OPMD)
– Collagen VI diseases (Bethlem and Ullrich
that immediately follows the mechanical stimu- congenital muscular dystrophies/myopathies)
lus by the needle and may continue after cessa- – Centronuclear, myotubular, and nemaline rod
tion of needle movement. “Spontaneous activity” congenital myopathies
does not require any mechanical trigger. – Distal myopathies
– Myofibrillar myopathies
Distinction between these two types of activities – Metabolic myopathies: acid maltase deficiency
is often considered arbitrary and clinically irrel- (Pompe disease), debrancher enzyme deficiency,
evant and one could use either term or the more carnitine deficiency, mitochondrial myopathies.
12 Myopathies 259
Table 12.5 Non-irritable myopathies (myopathic-type Table 12.7 Myopathies with prominent myotonic
MUPs with often normal spontaneous activity) discharges
Acquired muscle diseases Myotonic syndromes
– Treated Inflammatory or non-inflammatory – Myotonic dystrophy type 1 (Steinert’s disease)
necrotizing autoimmune myopathies – Myotonic dystrophy type 2 (proximal myotonic
– Steroid myopathy and myopathy from Cushing myopathy or PROMM)
syndrome – Myotonia congenita (chloride channelopathy)
– Myopathy due to hyper- or hypothyroidism – Hyperkalemic periodic paralysis (sodium
– Critical illness myopathy channelopathy)
Genetic muscle diseases – Paramyotonia congenita (sodium channelopathy)
– Facioscapulohumeral muscular dystrophy (FSHD) Non-myotonic syndromes
– Oculopharyngeal muscular dystrophy (OPMD) – Acid maltase deficiency (Pompe disease)
– Limb-girdle muscular dystrophies (LGMDs) – Some inflammatory myopathies
– Collagen VI diseases (Bethlem and Ullrich – Statin-induced myopathies
congenital muscular dystrophies/myopathies) – Toxic myopathies
– Congenital myopathies
– Distal myopathies
– Myofibrillar myopathies fibrillation potentials. CRDs can be seen in both
– Metabolic myopathies: some glycogen storage
diseases, lipid storage myopathies, carnitine
neurogenic and myopathic conditions and may
deficiency, carnitine palmitoyltransferase II (CPT II) or may not suggest chronicity of the underlying
deficiency, some mitochondrial myopathies pathology.
Myotonia is the spontaneous firing of a mus-
Table 12.6 Myopathies with potentially normal EMG cle fiber in which both amplitude and frequency
wax and wane. In contrast to myotonia, fibrilla-
Acquired muscle diseases
– Steroid myopathy tion potentials fire at a regular rate. A myotonic
Genetic muscle diseases discharge is comprised of positive sharp wave or
– Congenital myopathies brief spike potentials and its sound resembles that
– Metabolic myopathies: some mitochondrial of an accelerating and decelerating motorcycle or
myopathies, glycogen storage diseases between acute
attacks, carnitine palmitoyltransferase II (CPT II) chain saw. Although myotonia is a form of
deficiency increased/abnormal spontaneous activity, muscle
disorders showing prominent myotonia are elec-
trodiagnostically categorized under a separate
table patterns depending on the stage of the dis- group, called “myotonic syndromes” (Table 12.7).
ease, muscles involved and individual variability. Myotonic syndromes are divided into myotonic
In these myopathies, myopathic motor unit dystrophies and non-dystrophic myotonic syn-
potentials (discussed below) can be associated dromes. Myotonic dystrophies are either type 1
with or without abnormal spontaneous activity. or type 2. Myotonic discharges are typically more
Sometimes spontaneous activity and motor prominent in type 1 than in type 2. Electrodiagnosis
unit potentials are both normal and needle EMG of myotonic dystrophy type 2 can be very diffi-
examination does not show any abnormality cult, as many muscles might not reveal myotonic
(Table 12.6). discharges. Non-dystrophic myotonic syndromes
Complex repetitive discharges (CRDs) are include paramyotonia congenita (sodium chan-
created by a group of adjacent hyperexcitable nelopathy), myotonia congenita (chloride chan-
myofibers with one serving as a pacemaker fiber nelopathy), and hyperkalemic periodic paralysis
and the others becoming activated via ephaptic (sodium channelopathy). Myotonia is more
transmission. A loop of activation is created and severe and consistent in chloride channelopathies
the spikes remain uniform from one to another. than in patients with sodium channel mutations.
CRDs start suddenly and stop suddenly and they Hypokalemic periodic paralysis and Andersen-
mimic the sound of a machine gun. CRDs tend Tawil syndrome are two skeletal muscle chan-
to have higher amplitude spikes compared to nelopathies that are not typically associated with
260 P. Soltanzadeh
a b
Fig. 12.1 Myopathic motor unit potentials (MUPs) compared to normal MUPs. Panel A shows normal MUPs in biceps
brachii; Panel B demonstrates myopathic MUPs with early recruitment in biceps brachii (scale: 200 μV, 20 ms)
myotonia and therefore are not considered non- injured myofibers. These MUP changes may also
dystrophic myotonic syndromes. Prominent be seen in diseases of the neuromuscular junction
myotonic discharges are also seen in some other and during the early reinnervation phase after a
myopathies that are not known to primarily affect severe nerve injury, when “nascent” units result.
the skeletal muscle ion channels and are not con- Another EDX feature of myopathies is “early”
sidered myotonic syndromes per se; these include (or “increased”) recruitment of MUPs. This is
acid maltase deficiency (Pompe disease), some caused by activation of more motor units in order
inflammatory myopathies, statin-induced myop- to generate the required muscle force.
athies, and toxic myopathies. Examination of early recruitment requires atten-
Absent EMG activity (electrical silence) while tion to the degree of patient’s effort during mus-
the muscle is in the contraction phase is known as cle contraction. In order to assess recruitment,
“contracture” and can be seen in McArdle dis- the patient is asked to slightly activate the mus-
ease. “Cramps” are clinically similar to contrac- cle. In typical early recruitment, many MUPs
tures but during a muscle cramp, which is usually appear almost simultaneously as the patient starts
neurogenic, there is involuntary firing of normal to contract. Only the electromyographer can
motor unit potentials (MUPs) at a high frequency assess early recruitment as he or she is aware of
in the range of 200–300 Hz. Diminished sponta- how much force is being generated. If MUP
neous activity can also be seen during a periodic assessment starts with a rather forceful contrac-
paralysis attack. In muscular dystrophies and tion, early recruitment and smaller myopathic
progressive acquired myopathies, as the disease MUPs may miss detection.
progresses, muscle tissue is replaced by fatty or Rarely, in very chronic or advanced myopa-
fibrous tissue leading to end stage muscle. thies, entire motor units are lost and needle exam-
Spontaneous activity noted on insertion of the ination shows either mixed myopathic and
needle is typically decreased in such muscles. neurogenic features or sometimes predominantly
Examination of the MUPs may provide the neurogenic changes with reduced recruitment of
most specific data in support of a myopathic pro- large MUPs. This pattern makes EDX difficult
cess. Typical myopathic MUPs demonstrate short and is characteristically seen in sIBM, but also
duration, low amplitude, and polyphasia described in other chronic myopathies like mus-
(Fig. 12.1). Short duration and low amplitude cular dystrophies (Fig. 12.2).
MUPs are due to destruction or dysfunction of Disease processes involving both nerves and
the muscle fibers. Polyphasia results from loss of muscles can also cause a mixed neurogenic and
synchrony (asynchrony) in depolarization of the myopathic pattern (Table 12.3).
12 Myopathies 261
reduced. Needle EMG data can help increase the

yield of the muscle biopsy. Usually, muscles with
more abnormal spontaneous activity and myo-
pathic motor units show more pathologic find-
ings than muscles with normal EMG findings.
Muscles that feel gritty or like passing a knife
through butter during needle examination should
be avoided because they are likely to be end-
stage. Decreased insertional activity and reduced
recruitment of mixed neurogenic and myopathic
MUPs also suggest end-stage muscle tissue and
such muscles are less preferred for biopsy. It is
important to select muscles only on one side of
Fig. 12.2 Mixed myopathic and neurogenic motor unit the body and reserve the contralateral side for tis-
potentials (MUPs) in the rectus femoris muscle of a
patient with sporadic inclusion body myositis (sIBM)
sue diagnosis to avoid needle EMG
(scale: 500 μV, 20 ms) (inflammatory-like) artifacts created during nee-
dle examinations. In the appendicular segments,
both proximal and distal muscles should be sam-
EMG can help clinicians differentiate steroid pled. Sampling distal muscles is more important
myopathy from a recurrent or undertreated in myopathies that sometimes start from distal
inflammatory or autoimmune myopathy. In such segments; such myopathies include sIBM and
patients, an irritable myopathic pattern with myotonic dystrophy type 1. A comprehensive
abundant fibrillation or positive sharp wave needle examination typically includes sampling
potentials suggests recurrence or inadequately- at least three proximal and two distal appendicu-
treated inflammatory/autoimmune myopathy, lar muscles in each upper and lower extremity
whereas steroid myopathy typically shows nor- (total of 10 muscles) plus one or two paraspinal
mal or decreased spontaneous activity. muscles.
It is important to be mindful that in a patient Deltoid, biceps and triceps muscles in the
with myopathy, needle examination may be nor- upper extremity, and iliacus, rectus femoris, and
mal (Table 12.6) or only a limited number of vastus lateralis in the lower extremity are usually
these myopathic features may be observed. included in the EMG examination of a patient
with proximal myopathy. To check the distal
upper extremity segment, one should consider
Needle EMG Protocol in Myopathies one of the finger flexors like flexor pollicis longus
muscle as well as the first dorsal interosseous
As discussed above, nerve conduction studies are muscle. Brachioradialis, extensor digitorum or
performed after a brief history and manual mus- pronator teres muscles may also be sampled
cle testing. The data obtained from these studies depending on the phenotype. Irritable myopathic
lead to a higher yield needle EMG examination. changes in the forearm flexor muscles are com-
Muscles that are weak on clinical exam are monly seen in patients with sIBM and may pre-
expected to show more myopathic motor units cede clinical weakness or atrophy. Distal lower
with or without increased spontaneous activity extremity muscles that help the most include the
(fibrillation or positive sharp wave potentials), tibialis anterior and gastrocnemius. EMG
depending on whether the myopathy is irritable changes can be seen in distal myopathies that
or non-irritable. It is necessary to keep in mind affect anterior or posterior compartments of the
that if the muscle involvement is advanced or lower extremities. If posterior thigh compartment
end-stage and there is significant fatty or fibrous is involved, sampling the hamstring muscles or
tissue replacement, spontaneous activity is the gluteus maximus may also be added.
262 P. Soltanzadeh
Involvement of the posterior leg compartments is exertion intolerance. She was originally diag-
seen in limb-girdle muscular dystrophies 2A nosed with “fibromyalgia”. Her manual muscle
(calpainopathy) and 2B (dysferlinopathy). testing was normal despite prominent “give-way”
Sampling paraspinal muscles (the most proxi- weakness. CK was in the 1700–5000 U/L range
mal muscles) can be helpful in axial myopathies but her nerve conduction studies and needle
(including isolated neck extensor myopathy), EMG examination of proximal and distal mus-
adult-onset Pompe disease, and some inflamma- cles in both upper and lower extremities were
tory myopathies. Selection of paraspinal muscles normal. She also underwent a muscle biopsy of
depends on the clinical phenotype. The thoracic her vastus lateralis, which was unrevealing. An
paraspinal muscles are more protected from LGMD genetic panel revealed two mutations in
degenerative changes that lead to radiculopa- ANO5 (c.191dupA and c.692G > T(p.G231V)).
thies, and can generally be more helpful; how-
ever, in a patient with dropped head syndrome,
cervical paraspinal muscles (and possibly sple- Discussion These two unrelated sporadic
nius capitis) need to be assessed. patients with LGMD 2L (anoctaminopathy)
show very different clinical and EDX presenta-
tions. In patient 1, needle EMG shows neuro-
Case Studies genic MUPs with increased spontaneous activity.
Symmetrical involvement and very high CK are
Patient 1 A 23 year-old man presented with clues to a myopathic process. As discussed
6-month history of lower extremity weakness and above, neurogenic MUPs can be seen in advanced
muscle cramps leading to imbalance and near- or chronic myopathies. Although the patient’s
falls. His examination showed severe bilateral symptoms started about 6 months prior to the
calf atrophy and mild weakness in plantar flex- neuromuscular visit, the muscle tissue injury
ors. Upper extremity strength examination was probably started earlier in his life. The second
normal. Nerve conduction studies were normal. patient demonstrates that genetic muscle dis-
Needle EMG examination of the lower extremity eases can sometimes present with nonspecific
showed increased spontaneous activity (fibrilla- neuromuscular complaints, normal EMG, and
tion and positive sharp wave potentials) with neu- normal muscle biopsy. Very high CK is a good
rogenic motor units (long duration, high diagnostic clue.
amplitude, polyphasic MUPs). The EMG abnor-
malities were more severe in the posterior com-
partment muscles both below and above the knee
(gastrocnemius, short head of the biceps femoris, Suggested Reading
and semitendinosus). MRI of the lumbar spine
1. Bonne G, Bonne G, Rivier F, Hamroun D. The 2018
was normal. CK level was also checked showing
version of the gene table of monogenic neuromuscu-
a very high level of 11,322 (normal range: lar disorders (nuclear genome). Neuromuscul Disord.
30–220 U/L). Because of the very high CK level, 2017;27(12):1152–83.
a limb-girdle muscular dystrophy (LGMD) 2. Fournier E, Arzel M, Sternberg D, Vicart S,
Laforet P, Eymard B, Willer JC, Tabti N, Fontaine
genetic panel was requested showing two muta-
B. Electromyography guides toward subgroups of
tions in ANO5 (c.191dupA and c.2272C > T(p. mutations in muscle channelopathies. Ann Neurol.
R758C)). Based on the genetic results, the final 2004;56(5):650–61.
diagnosis was LGMD 2L (anoctaminopathy). 3. Jackson CE, Barohn RJ. A pattern recognition
approach to myopathy. Continuum (Minneap Minn).
2013;19(6 Muscle Disease):1674–97.
4. Joy JL, Oh SJ, Baysal AI. Electrophysiological spec-
Patient 2 A 33 year-old woman presented with trum of inclusion body myositis. Muscle Nerve.
7 years of severe myalgia, chronic fatigue and 1990;13(10):949–51.
12 Myopathies 263
5. Kimura J. Electrodiagnosis in diseases of nerve and 10. Petajan JH. AAEM minimonograph #3: motor unit
muscle: principles and practice. 4th ed. Oxford: recruitment. Muscle Nerve. 1991;14(6):489–502.
Oxford University Press; 2013. 11. Preston D, Shapiro B. Electromyography and neuro-
6. Lacomis D. Electrodiagnostic approach to the muscular disorders: clinical-electrophysiologic corre-
patient with suspected myopathy. Neurol Clin. lations. 3rd ed. London: Elsevier Saunders; 2013.
2012;30(2):641–60. 12. Robinson LR. AAEM case report #22: polymyositis.
7. Logigian EL, Ciafaloni E, Quinn LC, Dilek N, Pandya Muscle Nerve. 1991;14(4):310–5.
S, Moxley RT 3rd, Thornton CA. Severity, type, and 13. Shouman K, Morren J, Soltanzadeh P. Phenotypic
distribution of myotonic discharges are different in heterogeneity in limb-girdle muscular dystrophy type
type 1 and type 2 myotonic dystrophy. Muscle Nerve. 2 L (Anoctaminopathy) (P5.446). Neurology. 2018
2007;35(4):479–85. Apr;90(15 Supplement):P5.446.
8. Meriggioli MN, Barboi AC, Rowin J, Cochran 14. Young NP, Daube JR, Sorenson EJ, Milone
EJ. HMG-CoA reductase inhibitor myopathy: clini- M. Absent, unrecognized, and minimal myotonic dis-
cal, electrophysiological, and pathologic data in five charges in myotonic dystrophy type 2. Muscle Nerve.
patients. J Clin Neuromuscul Dis. 2001;2(3):129–34. 2010;41(6):758–62.
9. Paganoni S, Amato A. Electrodiagnostic evalua- 15. Zhou C, Wu L, Ni F, Ji W, Wu J, Zhang H. Critical
tion of myopathies. Phys Med Rehabil Clin N Am. illness polyneuropathy and myopathy: a systematic
2013;24(1):193–207. review. Neural Regen Res. 2014;9(1):101–10.
Recommendations for Writing
an Electrodiagnostic Study Report 13
Alexandra Soriano and John A. Morren
After performance of nerve conduction and elec- 2. An EDX report should include the date of the
tromyography studies, a written report with thor- study, patient demographic data (name, medical
ough description of the findings should be record number, and age or birth date, also more
produced, with the goal of interpreting the results optionally- height, weight, gender, handedness
in a clear way that can be understood by the refer- and relevant medical diagnoses), a brief descrip-
ring physician, and that explains the diagnosis, tion of the indication for study, including clini-
correlated with the findings- including the nature/ cal findings (pertinent history and exam
character (e.g. axon loss or demyelinating), dis- components), the type of EDX test that was per-
tribution, and severity of nerve, muscle or neuro- formed (especially if a particular protocol was
muscular junction transmission disorder. followed e.g. for myopathy), all nerve conduc-
tion study and needle electrode examination
1. The performing physician must have not only data collected during the study (preferably tabu-
the appropriate training and education to do lated, in conjunction with normal values) and a
this task, but also needs to know the patient’s narrative interpretation of these data with final
history and exam findings (repeating as neces- electrodiagnosis. It is important to include these
sary) that lead to ordering the study in the first components on the report in order to allow the
place. With this information, the performing ordering physician, or others reviewing the
physician can tailor the study to answer the results, to corroborate the conclusion to the spe-
specific questions posed and create a report cific findings, and/or to compare to any prior
that helps correlate the final findings on the studies. Most of the data from the nerve conduc-
EDX study to the clinical features. tion study can be given in tables that specify the
side (right or left) tested, which limbs were
tested, limb temperature at the time of testing,
A. Soriano (*)
Braathen Neurological Center, Cleveland Clinic specific nerve +/− muscle tested, site of stimu-
Florida, Weston, FL, USA lation and recording, distance measured, and
Cleveland Clinic Lerner College of Medicine of Case results of latency, amplitude and conduction
Western Reserve University, Cleveland, OH, USA velocity. It is best if the results that are outside
e-mail: soriana@ccf.org normal range values are highlighted.
J. A. Morren 3. Findings of needle EMG are best described in
Neuromuscular Center, Neurological Institute, addition to tabulated data, as these results can
only be fully interpreted right at the time of

https://doi.org/10.1007/978-3-030-74997-2_13
266 A. Soriano and J. A. Morren
the study i.e. in “real time”. In this part of the studies. It may be optional to provide a clini-
report, one should include muscle and side cal diagnosis that correlates with the electro-
tested. The insertional activity, presence of diagnostic test findings (e.g. median
any abnormal spontaneous activity, and the neuropathy at or distal to the wrist, consis-
description of the motor unit action potential tent with a clinical diagnosis of carpal tunnel
configuration/morphology (including ampli- syndrome), possible differential diagnosis,
tude, duration, and number of phases) and and if any further testing could be of value.
recruitment pattern appreciated when the The further testing recommended may
muscle is activated should be described. include other modalities within the scope of
4. If the study featured any limitations that could neuromuscular diagnostics, including neuro-
have affected the results, this must be described muscular ultrasound.
(e.g. limb edema, suboptimal patient tolerance
or inability to adequately relax during evalua- The name of the physician performing the
tion of spontaneous activity). EDX study should be clearly identified on the
5. The study interpretation should specify if report with an appropriate signature (which may
results are normal or abnormal, and if abnor- be electronic) and date/time stamp. In cases for
mal, which abnormalities exist. An electro- which appropriately trained NCS technologists
physiological diagnosis should be given, perform NCSs under the direction and supervi-
with specific location of the pathology, sion of the EDX physician, the name of the tech-
nature/character (e.g. axon loss versus demy- nologist and the physician should both be
elinating for polyneuropathies; with or with- included on the report, along with the signature
out necrotizing/inflammatory features for of the physician. In cases involving a resident or
myopathies), chronicity, severity, and if pos- fellow, the name of the trainee should also be
sible, comparison with any available prior included on the report.
13
Sample Report
Institution/Office Address: Solveit Clinic, 1000 Madeup Ave, Fictionville, XY. 77077. USA.
Telephone: (111) 222-3333.
[Accreditation status: E.g. AANEM Electrodiagnostic Laboratory Accreditation status (optional but recommended)]
Patient Name: Random, John DOB: 06/20/1991 Age: 28 Height/Weight: 5′7″, 160 lbs
Medical Record Number: 99887766 Gender: Male Handedness: Right
Pertinent medications-Anticoagulant medication: No Acetylcholinesterase inhibitor: N/A
Implanted electrical devices: None.
Referring provider: John Livingston, M.D.
EMG staff: Peter Needleman, M.D. Technician (credentials): Susie Elektra (CNCT)
Study date: 06/05/2020 EMG Room#: 5
Symptoms and signs: Patient with a history of well-controlled asthma, noting right more than left hand pain/paresthesia and numbness primarily
affecting digits 1–3 on both sides, with some proximal spread to the mid forearm at times. There is intermittent neck ache, sometimes spreading into
the shoulders bilaterally. Hand symptoms started about 3 months ago, worse at night, causing sleep disturbance. Symptoms onset noted after about 1
month into a new job working at an assembly line for automotive parts. No significant motor symptoms apart from some loss of dexterity on the right.
Exam most notable for marked reduction of pinprick and temperature perception mostly over the palmar aspect of the thumb, index and middle finger,
right more than left. There appears to be mild weakness of the right abductor pollicis brevis muscle. Tinel’s sign is positive over the median nerve at the
right wrist, equivocal on the left.
NCS/EMG exam type or protocol ordered (optional/depends on lab): General upper extremity screen.
Recommendations for Writing an Electrodiagnostic Study Report

Nerve Stimulus Recording L R L R L R L R Norm B-P Amp Norm LatNPk Norm CV L R
Median Wrist Index 31.74 No 3.94 No n/a n/a 130 130 >20 uV <3.3 ms >51 m/s 32.8 33.4
Response Response
Ulnar Wrist 5th Digit 35.77 2.18 n/a 110 >18 uV <3 ms >51 m/s 33.9
Radial Thumb Forearm 45.33 1.74 n/a 100 >18 uV <2.7 ms 33.6
267
268

B-P Amp (mV) LatOn (ms) CV (m/s) Dist (mm) Norm Norm Temp (°C)
R L R L R L R B-P Distal L
Nerve Recording Stimulus L Amp LatON Norm CV R
Median Abductor Wrist 6.99 4.91 4.25 5.60 n/a n/a 50 50 >6 mV <3.9 ms >51 m/s 32.5 34.0
pollicis Elbow 4.81 9.60 56.3 225 33.8
brevis Post exercise 4.96 5.50 n/a 34.1
Ulnar Abductor Wrist 11.93 1.45 n/a 50 >8 mV <3 ms >51 m/s 33.4
digiti Below elbow 11.36 4.90 62.3 215 33.4
minimi Above elbow 11.12 6.55 61.8 315 33.4

F-waves
Lat (ms)
Ulnar Wrist Abductor digiti minimi 23.15
Needle EMG summary

L Abductor Pollicis Brevis Norm 0 0 0 Norm Full Norm Norm Norm NC
R 1st Dorsal Interosseous Norm 0 0 0 Norm Full Norm Norm Norm NC
Abductor Pollicis Brevis Norm 0 0 0 1− Mod-R Few 1+ Few 1+ Norm NC
Flexor Pollicis Longus Norm 0 0 0 Norm Full Norm Norm Norm NC
Extensor Indicis Proprius Norm 0 0 0 Norm Full Norm Norm Norm NC
Pronator Teres Norm 0 0 0 Norm Full Norm Norm Norm NC
Biceps Brachii Norm 0 0 0 Norm Full Norm Norm Norm NC
Triceps—lateral head Norm 0 0 0 Norm Full Norm Norm Norm NC
Deltoid—middle head Norm 0 0 0 Norm Full Norm Norm Norm NC
A. Soriano and J. A. Morren
13
Technician’s comments: Patient somewhat anxious, with slightly sweaty palms.
Study Interpretation:
Extensive electrodiagnostic examination of the right upper limb and additional studies of the left upper limb reveal the following:
1. Evidence of bilateral median neuropathies at or distal to the wrist (consistent with a clinical diagnosis of carpal tunnel syndrome) which is severe
in degree electrically on the right and mild in degree electrically on the left.
Pertinent features include absence of the median nerve sensory response on the right (that on the left is normal in amplitude, with mild prolonga-
tion of its peak latency), in addition to significant prolongation of median motor distal latencies (right> left), with reduction of the median motor
amplitude recording the abductor pollicis brevis (APB) on the right. There are also mild chronic (no active/ongoing) motor axon loss changes in the
right APB.
2. There is no definite evidence of a superimposed right cervical (C5–C8) motor radiculopathy.
3. There is also no definite evidence of a right ulnar or radial mononeuropathy, based on screening electrodiagnostic studies in those respective nerve
distributions.
EMG Staff’s Signature (may be electronic, if allowed):

This examination was performed, interpreted and electronically signed by: Peter Needleman, M.D. on June 05, 2020 at 9:31 AM.
------------------------------------------------------------------------------------------------------------------------.
Suggested Reading
Recommendations for Writing an Electrodiagnostic Study Report
1. Jablecki CK, Busis NA, Brandstater MA, Krivickas LS, Miller RG, Robinton JE, American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM).
Reporting the results of needle EMG and nerve conduction studies: an educational report. Muscle Nerve. 2005;32(5):682–5.
269
Correction to: Radiculopathies
orrections to: Chapter 8 in N. Galvez-Jimenez et al. (eds.),

C
Electrodiagnostic Medicine,
https://doi.org/10.1007/978-3-030-74997-2_8
This book was inadvertently published with a spelling error in the author’s name of chapter 8.
The correct name is Karen A. Karwa, this has been updated with this erratum.
The updated version of the book can be found at

https://doi.org/10.1007/978-3-030-74997-2_8
© Springer Nature Switzerland AG 2022 C1

https://doi.org/10.1007/978-3-030-74997-2_14
Index
A electrodiagnostic assessment, 164, 165, 167–170

Abductor digiti minimi (ADM), 7, 47–48 elemental anatomy
Abductor Digiti Quinti Pedis (ADQP), 39–40 cords, 162
Abductor hallucis (AH), 8 divisions, 161
Abductor pollicis brevis (APB), 7, 43, 85 roots, 159–161
Acetylcholine receptor deficiency, 243 terminal nerves, 162
Acetylcholinesterase, 242 trunks, 161
Acetylcholinesterase enzyme (AChE), 227 general anatomy, 158–160
Acute inflammatory demyelinating incidence, 157
polyradiculoneuropathy (AIDP), 122, 134–136 NCS assessment, 157, 165–166
Acute motor sensory axonal neuropathy (AMSAN), 136 lateral cord, 172, 173
Agrin, 245 lower plexus, 172
Alcohol, 129 medial cord, 173
Amyotrophic Lateral Sclerosis (ALS), 142, 213 middle plexus, 172
Anterior interosseous nerve (AIN) syndrome, 86 posterior cord, 173
Anterior primary ramus (APR), 160 preterminal and terminal nerves, 173
Axillary neuropathy, 96, 104, 107–109 upper plexus, 171, 172
Axon (A) reflex, 145 regional anatomy, 162–164
Axonal polyneuropathy, 129–131, 137, 146, 147 site-specific disorders, 157, 158
Axonotmesis, 6 Brachioradialis, 50
B C
B12 deficiency, 129 Carpal tunnel syndrome (CTS), 17, 43, 85
Behcet’s disease, 19 Cervical radiculopathy, 198–200
Bell’s palsy, 94 Choline acetyltransferase (ChAT) deficiency, 242
Brachial plexus Chronic inflammatory demyelinating
clinical assessment, 164 polyradiculoneuropathy (CIDP), 123–128,
EDX assessment 132–135, 137–144, 146–148, 151
distal upper extremity sensory and motor CMT-IA, 143
abnormalities, 178–180 CMT-X, 143
left hand weakness and atrophy, 190, 191 Complex MUAP, 73
left upper extremity numbness, 180–183, Complex repetitive discharges (CRDs), 10, 71, 259
186–188, 192–194 Compound muscle action potential (CMAP) amplitude,
weakness, 175–178, 186–188, 192–194 7, 80, 129
left upper extremity pain, 175–178, 180–183 Concentric and monopolar needle electrodes, 75
motor NCS, 171 Concentric EMG needle, 76
needle NCS, 171 Conduction block, 140, 142
postoperative left ulnar neuropathy, 183–186 Conduction velocity (CV), 129
progressive left upper extremity weakness, Congenital myasthenia gravis (CMS), 240, 242
188–190 Congenital myasthenic syndrome, postsynaptic defects,
sensory NCS, 170 243, 245
SNAP domains, 170 Cramp-fasciculation syndrome, 67
EDX exercises, 173–175 Cramps, 260

https://doi.org/10.1007/978-3-030-74997-2
272 Index
D Flexor digitorum profundus (FDP), 48

Deltoid, biceps and triceps muscles, 261 Frontotemporal dementia (FTD), 213
Demyelinating polyneuropathies, 133, 134
Diabetes mellitus (DM), 121, 123, 124, 128,
131–132, 135 G
Diabetic lumbosacral radiculoplexus neuropathy Glycogen metabolism, 65
(DLRPN), 21, 132 Guillain-Barré syndrome, 94, 126
Distal acquired demyelinating sensory neuropathy Guyon’s canal, 88
(DADS), 127
Distal acquired demyelinating symmetric neuropathy
(DADS), 125 H
Distal symmetric polyneuropathy Hematoxylin, 215
(DSP), 124, 130–133 Henneman size principle, 68
DOK-7 deficiency, 245 Hereditary neuropathy with liability to pressure palsies
Dorsal root ganglion (DRG), 159 (HNPP), 144
Hereditary spastic paraparesis, 219
H-reflex, 200
E
Edaravone, 216
Edrophonium chloride test, 233 I
Electrodiagnostic (EDX) studies Ice pack test, 232
A-wave/axon reflex, 13–14 Immune-mediated processes, 219
biases, advantages and disadvantages, 2–5 Inclusion body myositis (IBM), 218
blink reflexes, 14–15 Inherited polyneuropathies, 124, 125, 133, 143, 144
concentric needle Innervation ratio, 159
electromyography, 16 Interpotential interval (IPI), 252
facial nerve motor studies, 15–17 Intraspinal canal, 196
foot drop/weakness, 22–23 Irritable myopathies, 258
foot pain/numbness, 18–19
F-wave response, 11–13
hand pain/numbness/sensory K
disturbance, 17–18 Kennedy’s disease, 219
needle electrode examination, 9–10, 16
nerve conduction studies, 7–9
peripheral nerve fibers, 2 L
proximal lower limb/anterior Lambert-Eaton myasthenic syndrome (LEMS), 7
thigh weakness, 21 clinical features, 239
radiculopathies, 19–21 definition, 238
report, 265, 266, 268 diagnostic evaluation, 239, 240
routine NCS, 11 electrophysiological finding, 240, 241
tibial H-reflex assesses, 13 epidemiology, 238
Electromyography (EMG), 1 etiology, 238
El Escorial criteria, 214, 216, 218 pathophysiology, 238
Endoneurium, 6 SFEMG, 240
Endplate myopathy, 243 treatment and management, 240
Endplate potential (EPP), 233 Lateral antebrachial cutaneous (LABC) nerve, 167
Epineurium, 6 Lead toxicity, 133
Extensor carpi ulnaris (ECU), 49 Lower motor neurons (LMN), 213
Extensor digitorum, 253 Lumbosacral radiculopathy, 199, 200
Extensor digitorum brevis (EDB) muscle, 8
Extensor digitorum communis (EDC) muscle, 8
M
McArdle disease, 260
F Microtubules, 121
Femoral neuropathy, 93, 117, 118 Miller Fisher syndrome, 136
Fibrillation potentials, 69 Miniature Endplate Potential (MEPP), 233
First dorsal interosseous (FDI), 8, 47 Moment-to-moment amplitude variation (MMAV), 78
Flexor carpi radialis (FCR), 46–47 Monoclonal gammopathy with undetermined
Flexor carpi ulnaris (FCU), 48 significance (MGUS), 127, 143
Index 273
Mononeuritis multiplex, 143 needle EMG protocol, 261, 262

Mononeuropathies patient history, 262
anterior interosseous neuropathy, 109, 110 Myotonia, 259
axillary neuropathy, 96, 107–109, 1104 Myotonic potentials, 70
facial nerve, 94, 95 Myotonic syndromes, 259
femoral nerve, 93, 94
femoral neuropathy, 93, 117, 118
focal demyelination, 83 N
long thoracic neuropathy, 96–97 Needle electrode examination (NEE)
median nerve, 83–86 abductor hallucis, 58–59
median neuropathy, 83, 97–98 adductor longus, 63
musculocutaneous neuropathy, 96 ADM, 47–48
peroneal (fibular) nerve, 90–92 anconeus, 50
peroneal (fibular) neuropathy, 111–113 APB, 43
radial nerve, 89–90 biceps brachii, 51
radial neuropathy, 101 brachioradialis, 50
sciatic nerve, 93 cervical paraspinal muscles, 56
sciatic neuropathy, 111 deltoid, 52
spinal accessory neuropathy, 97 ECU, 49
superficial radial sensory neuropath, 83 extensor digitorum, 49–50
suprascapular neuropathy, 95, 101–104 extensor digitorum brevis, 56–57
tibial nerve, 92 extensor hallucis longus, 57
tibial neuropathy, 113–117 extensor indicis, 49
ulnar nerve, 86–89 FCR, 46–47
ulnar neuropathy, 83, 87–89, 98–101 FCU, 48
Monopolar EMG needle, 76 FDI, 47
Motor F-wave, 200 FDP, 48
Motor neuron diseases (MND), 2 flexor digitorum longus, 59
anatomy, 215, 216 flexor digitorum profundus, 45–46
clinical features, 216–218 flexor digitorum superficialis, 46
differential diagnosis, 218, 219 flexor hallucis brevis, 59
electrodiagnostic evaluation, 219–221 flexor pollicis brevis, 44
electrodiagnostic limitations, 221–223 flexor pollicis longus, 45
etiology gluteus maximus, 62
ALS, 213, 214 gluteus medius, 61–62
PBP, 214, 215 iliopsoas/iliacus, 59
PLS, 214 infraspinatus, 54
PMA, 214 latissimus dorsi, 55
UMN symptoms, 213 long head of biceps femoris, 60
patient history, 223, 224, 226 lumbar paraspinal muscles, 64
Motor unit action potentials (MUAPs), 65, 131, 136, 221 opponens pollicis, 44
Multifocal acquired demyelinating sensory and motor pectoralis major, 51–52
neuropathy (MADSAM), 142 peroneus longus, 58
Multifocal axonal motor neuropathy (MAMN), 142 peroneus tertius, 57
Multifocal motor neuropathy (MMN), 142 pronator quadratus (PQ), 44–45
Multifocal motor neuropathy with conduction block pronator teres (PT), 47
(MMNCB), 219 rectus femoris, 63
Muscle-specific tyrosine kinase (MuSK), 227 rhomboids, 54–55
Muscle stretch reflexes (MSRs), 164 semimembranosus, 60–61
Musculocutaneous neuropathy, 96 semitendinosus, 61
Myasthenia gravis (MG), 228, 230–232, 252–254 serratus anterior, 55–56
Myelin sheath, 122 short head of biceps femoris, 60
Myokymia, 10 sternocleidomastoid, 53
Myokymic potentials, 70 supraspinatus, 54
Myopathy surface localization, 43
differential diagnosis, 255, 257 tensor fascia lata, 61
electrodiagnostic evaluation, 257, 258 teres minor, 52
etiology and clinical features, 255–257 tibialis anterior, 58
MUAPs, 78 triceps brachii, 50–51
needle EMG findings, 258–261 upper trapezius, 53
274 Index
vastus lateralis, 62 medial antebrachial cutaneous sensory

Needle Electrode examination (NEE), 201, 203, 204 recording, 29
Needle electromyography (EMG) median motor recording, 30–31
abnormal spontaneous activity, 67 median palmar mixed nerve, 28–29
acute discontinuity lesion, 75 median sensory recording, 26–27
advantages and limitations of NCS, 66 musculocutaneous recording, 33–34
CMAP amplitude, 81 radial motor recording, 32–33
complex fasciculation potential, 67 radial sensory recording, 28
complex MUAP, 73 ulnar motor recording, 31, 32
concentric needles, 72, 76 ulnar palmar mixed nerve, 29
cooling of muscle, 68 ulnar sensory recording, 27
cramp potentials, 71 Neural foramina, 196
CRDs, 71 Neurapraxia, 6
decreased insertional activity, 65 Neuromuscular jitter, 250, 252
early recruitment, 72 Neuromuscular junction (NMJ)
end-plate noise, 65, 66 anatomy and physiology, 227, 228
end-plate spikes, 66 clinical presentation, 229
fasciculation potentials, 67 CMS, 240, 242
fibrillation potentials, 69 congenital myasthenic syndrome, postsynaptic
focal demyelination, 80 defects, 243, 245
Henneman size principle, 68 diagnostic testing
“inferred” block, 81 edrophonium chloride test, 233
insertional activity, 65 ice pack test, 232
monopolar EMG needle, 76 RNS, 233–235, 237
MUAP abnormalities, 78 SFEMG, 237, 239
MUAP appearance and recruitment, 65 epidemiology, 228, 229
myokymic potentials, 70 genetic syndromes, 242, 243
myopathy, 80 immunosuppressive therapy, 237
“myopathic” MUAPs, 79 LEMS, 238–241
myotonic potentials, 70 myasthenia gravis, 228, 230–232
neurogenic-appearing and myopathic-appearing, 80 transmission, 78
neuromyotonia, 70 Neuromyotonia, 70
normal insertional activity, 66 Neurotmesis, 6
normal recruitment pattern, 74 Nodopathies, 122
optimal needle EMG, 76, 77 Non-irritable myopathies, 259
positive sharp wave potentials, 69 Normal motor unit action potential (MUAP), 73
reduced recruitment, 69 Nutritional deficiency, 132
spontaneous activity, 65
tremor tracing- normal MUAPs, 71
Needle EMG, 77, 78 O
Nerve conduction studies (NCS), 2, 83, 129 Oculopharyngeal muscular dystrophy, 218
filter settings, 25 Opponens pollicis, 85
lower extremity
lateral femoral cutaneous nerve recording, 36
medial and lateral plantar mixed nerve response P
recording, 36–37 Paraneoplastic processes, 219
saphenous nerve recording, 35–36 Paraproteinemic polyneuropathy, 127
superficial peroneal (fibular) sensory recording, 35 Paraspinal muscle NEE abnormalities, 20
sural (sensory) recording, 34–35 Perineurium, 6
motor NCS Plasma cell dyscrasias, 127
facial motor recording, 41–42 Polyneuropathy
femoral motor recording, 40, 41 definition, 121
peroneal (fibular) motor recording, 37–38 electrodiagnosis
spinal accessory motor recording, 41 AIDP, 134–136
tibial motor recording, 38–40 AMAN/AMSAN, 136
upper extremities asymmetric, 129–132, 134
axillary recording, 34 axonal polyneuropathy, 129–131
dorsal ulnar cutaneous sensory recording, 27–28 CIDP, 133, 134, 137–140, 142, 143
lateral antebrachial cutaneous sensory, 29–30 demyelinating polyneuropathies, 133, 134, 143
diabetes mellitus, 131, 132
Index 275
distal symmetric polyneuropathy, 133 diagnosis, 205, 206

electromyography, 146, 147 electrodiagnostic evaluation
examination techniques and protocol for NEE, 201, 203, 204
evaluation, 144, 145 nerve conduction studies, 200, 201
GBS, 136 etiology, 195, 196
inherited polyneuropathies, 143, 144 patient history, 206–210
nerve conduction, 145–151 Rapsyn protein, 245
nutritional deficiency, 132 Repetitive nerve stimulation (RNS), 2
symmetric, 129, 134, 141 definition, 233
toxic polyneuropathies, 133 electrophysiological findings in MG, 234, 235
length dependent/non–length dependent, 125 fast-rate stimulation, 233
pathogenesis, 121, 122 physiology, 233
patient history, 143, 148, 151 slow-rate stimulation, 233
symmetry/asymmetry technical aspects, 235–237
CIDP, 126, 127 Reversible conduction failure (RCF), 136
DADS, 125 Riluzole, 216
DSP, 124
etiology, 128, 129
GBS, 126 S
inherited polyneuropathies, 124, 125 Sensory nerve action potentials (SNAPs), 7, 130, 139
length dependent polyneuropathy, 124 Single fiber electromyography (SFEMG), 237
paraproteinemic polyneuropathy, 127 development, 249
predominantly proximal weakness, 125 monopolar and concentric needles, 249, 250
weakness, 128 myasthenia gravis, 252–254
temporal evolution, 122 stimulation, 249
type of modality, 123, 124 voluntary activation, 249–252
type of nerve fiber, 123, 124 Single MUAP (SMU), 72
upper limb predominant weakness, 128 Slow firing MUAPS, 72
Polyphasic MUAP, 10 Somatosensory evoked potentials (SEPs), 200
Postexercise exhaustion, 234 Spinal accessory neuropathy, 97
Postexercise facilitation, 234 Spontaneous activity, 65, 68
Predominantly proximal weakness, 125 Spurling’s test, 198
Preganglionic sympathetic axons, 161 S-Radial response, 188
Primary lateral sclerosis (PLS), 214 Superficial part of flexor pollicis brevis, 85
Progressive bulbar palsy (PBP), 214 Suprascapular neuropathy, 95
Progressive muscular atrophy (PMA), 214
Pronator teres syndrome, 86
Proximal median neuropathies, 47 T
Terminal latency index (TLI), 143
Thoracic radiculopathy, 198
Q Tibial neuropathy, 113–117
Quadrilateral space, 96 Tibialis anterior (TA) muscle, 8
Toxic polyneuropathies, 133
R
Radiculopathy U
anatomy, 196, 197 Upper limb predominant weakness, 128
clinical features Upper motor neurons (UMN), 213
cervical radiculopathy, 198–200
lumbosacral radiculopathies, 199, 200
sensory loss and weakness, 197 V
thoracic radiculopathies, 198 Voltage-gated calcium channel (VGCC), 238, 245

Electrodiagnostic Medicine: A Practical Approach Nestor Galvez-Jimenez Alexandra Soriano John A. Morren

Uploaded by

Copyright:

Available Formats

Electrodiagnostic Medicine: A Practical Approach Nestor Galvez-Jimenez Alexandra Soriano John A. Morren

Uploaded by

Document Information

Original Title

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

Electrodiagnostic Medicine: A Practical Approach Nestor Galvez-Jimenez Alexandra Soriano John A. Morren

Uploaded by

Copyright:

Available Formats

Electrodiagnostic

ISBN 978-3-030-74996-5 ISBN 978-3-030-74997-2 (eBook)

© Springer Nature Switzerland AG 2021, corrected publication 2022

Few Antecedent Words of Gratitude from NGJ

When one of us (NGJ) was approached by Springer to work on a book proj-

Using the Book

One of the critical challenges facing trainees and experienced electrodiagnos-

Weston, FL Nestor Galvez-Jimenez

1 Principles of Electrodiagnosis: Introduction�������������������������������� 1

Karin Armstrong, C.N.C.T. Neurophysiology Lab, Cleveland Clinic

Elanagan Nagarajan, MD Department of Neurology, Neurology Clinics,

Introduction myopathy, neuropathy or radiculopathy all in one

© Springer Nature Switzerland AG 2021 1

Fig. 1.1 Illustration of

Fig. 1.2 Outline of a

responses include median sensory recording

Table 1.5 Nerve conduction studies Table 1.5 (continued)

tear drop appearance [13, 14]. When the electri-

Fig. 1.4 Illustration of

The A-wave or Axon Reflex

Fig. 1.5 Illustration of

Fig. 1.7 Blink reflex-normal responses and measurements

Table 1.6 Suggested standard assessment of the upper

Table 1.6 (continued) disturbance may be distributed variably in the

peroneal (fibular) or tibial nerve pathway. be absent if an S1 root involvement is present).

is intraspinal or at the intervertebral foramen, Again, it is important to keep in mind that

patient with prolonged lithotomy position). It is 1. Partial or complete demyelinating block

Generalized Weakness References

Introduction General Concepts

© Springer Nature Switzerland AG 2021 25

Recording electrode location:

Ground: Between stimulation and recording

Fig. 2.6 Radial sensory response—stimulating at distal

Ground: Between stimulation and recording Ground: dorsum of the hand.

 adial Sensory Recording at Base

of the Thumb (See Fig. 2.6) G

Anatomy: brachial plexus (posterior cord ←

Fig. 2.9 Medial Antebrachial Cutaneous sensory

 lnar Palmar Mixed Nerve (See Fig. 2.8)

Fig. 2.11 Median motor response recording the abductor

Fig. 2.10 Lateral Antebrachial Cutaneous sensory C

response—stimulating anterolateral elbow, recording lat-

Ground: between the stimulation and record-

(MGA) must be considered (this type of MGA

 lnar Motor Recording at Abductor

E1: Motor point, belly of the ADM.

Distal site: At the wrist medial to the tendon of

(See Fig. 2.14).

Fig. 2.16 Ulnar motor recording first dorsal interosse-

E1: Motor point, belly of the EDC.

Ground: on the forearm between the recording

Distal site: At the elbow, at the groove between

E1: Motor point, belly of the biceps.

Distal: At the axilla beneath the tendon of the

Caveat: Supramaximal stimulations may be

Fig. 2.20 Musculocutaneous motor recording biceps

Fig. 2.22 Axillary motor recording deltoid, stimulation

Stimulation: At Erbs point, in the supracla-

Fig. 2.23 Sural (sensory) recording at ankle/lateral mal-

Few Antecedent Words of Gratitude from NGJ

Using the Book

Weston, FL Nestor Galvez-Jimenez

1 Principles of Electrodiagnosis: Introduction�� 1

Introduction myopathy, neuropathy or radiculopathy all in one

The A-wave or Axon Reflex

Generalized Weakness References

Introduction General Concepts

adial Sensory Recording at Base

lnar Palmar Mixed Nerve (See Fig. 2.8)

lnar Motor Recording at Abductor

eroneal (Fibular) Motor Recording

Flexor Pollicis Brevis

lexor Digitorum Profundus (FDP)

Extensor Carpi Ulnaris (ECU)

Needle placement: with the forearm pronated, Anconeus

Upper Trapezius Activation: Turning head and neck to the con-

Flexor Hallucis Brevis

Long Head of Biceps Femoris

Tensor Fascia Lata

Lumbar Paraspinal Muscles

What Do We Measure resolve with 10 s of onset, is termed “snap,

seasoned EDX medicine consultant. However, it How Do We Perform These

hat Do the Measurements Mean?

dle EMG for diagnosing muscle disorders is vari- ow Do These Measurements