95 Cranial Nerve Disorders

Ernest E. Wang

diabetes and hypertension are common comorbid conditions.

KEY POINTS Cranial nerves I, VI, and VII are the most frequently affected
after minor head trauma.1 Trigeminal neuralgia is a common
cause of facial pain that affects approximately 4.5 per 100,000
• The 12 cranial nerves supply motor and sensory
individuals; women are affected twice as often as men, and it
innervation to the head and neck.
is more common in those older than 60 years.2 Trigeminal
• Cranial nerve disorders generally cause visual
neuralgia can be severely debilitating and has been termed the
disturbances, facial weakness, or facial pain or
“suicide disease.”3 Bell palsy is the most common cause of
paresthesias, depending on the nerve or nerves
acute facial paralysis worldwide. The peak age at incidence
involved.
has been reported to be between 15 and 45 years,4 but other
• Trigeminal neuralgia and Bell palsy are common cranial
investigators have noted an increased incidence in individuals
nerve disorders.
older than 70.5,6 Pregnant women and patients with diabetes
• A thorough history and physical examination should
have an associated increased incidence of the disease. A
focus on assessing the potential for trauma (skull
familial association of Bell palsy is noted in 4% of cases,4
fracture), tumor, cerebrovascular accidents, vascular
and it can cause both significant psychologic and physical
derangements (aneurysm, dissection, thrombosis), and
morbidity.
infection (meningitis, abscess).
• The presence of concomitant focal neurologic or
systemic signs should heighten suspicion for a central
PATHOPHYSIOLOGY
rather than a peripheral cause of the neurologic
dysfunction.
CRANIAL NERVE I (OLFACTORY NERVE)
Anatomy
Cranial nerve I is a special sensory nerve that provides the
sense of smell. Inhaled scents are detected by the olfactory
epithelium lining the nasal cavity and transmitted to the olfac-
tory bulb, which lies adjacent to the cribriform plate of the
PERSPECTIVE ethmoid bone. Olfactory sensations are relayed from the
olfactory bulb to the brain via the olfactory tract.
The 12 cranial nerves provide motor and sensory innervation
to the head and neck. Some nerves serve purely motor func- Presenting Signs and Symptoms
tions (cranial nerves III, IV, VI, XI, and XII), some serve The patient should be questioned about a history of head
purely sensory functions (cranial nerves I, II, and VIII), and trauma. An anteroposterior skull fracture parallel to the sagit-
the remainder serve mixed motor and sensory functions tal suture or an anteroposterior shearing injury can tear the
(cranial nerves V, VII, IX, and X). olfactory fibers traversing the cribriform plate and lead to
In addition to somatic and visceral sensory components, the disruption of the synapses from the olfactory epithelium to
cranial nerves provide the special sensory functions of sight, the olfactory bulb.
smell, hearing, taste, and balance. Understanding the func- A frontal lobe mass such as a tumor, meningioma, or
tions of individual cranial nerves aids in recognition of pat- abscess can compress the olfactory bulb as well, but the signs
terns of the clinical syndromes classically associated with and symptoms associated with such masses tend to be more
disorders of specific cranial nerves. subacute.

Treatment
EPIDEMIOLOGY Treatment depends on the presence of concomitant injury.
Basilar skull fracture and cerebrospinal fluid rhinorrhea asso-
Cranial neuropathies are a heterogeneous group of disorders ciated with trauma require immediate neurosurgical consulta-
with a variety of causes. Trauma is a common cause, and tion. A subacute mass or abscess should be managed in

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 CHAPTER 95 Cranial Nerve Disorders

consultation with neurosurgery, depending on the acuity of

the findings. Patients with anosmia secondary to trauma and BOX 95.1  Differential Patterns of Visual Loss
normal findings on head computed tomography (CT) can
referred to neurology or neurosurgery for outpatient follow-up. A central retinal etiology of the fovea or optic disk compro-
mises visual acuity or causes central loss of vision in the
CRANIAL NERVE II (OPTIC NERVE) affected eye only.
Anatomy Unilateral blindness is usually associated with an optic nerve
Visual stimuli are transmitted from the retina to the optic lesion, and only the affected eye has complete visual field
nerve through the optic chiasm to the lateral geniculate loss.
nucleus in the thalamus, where they synapse. From there, Unilateral nasal visual field loss can be caused by an internal
impulses are transmitted along the optic radiations (geniculo- carotid artery aneurysm compressing the lateral optic
calcarine tracts, including the Meyer loop) to the primary chiasm.
visual cortex in the occipital lobes. Bitemporal hemianopia can be caused by a midchiasmatic
lesion.
Presenting Signs and Symptoms Homonymous hemianopia from an optic tract lesion causes
Unilateral loss of vision is most common with injuries to the full contralateral visual field loss in both eyes.
optic nerve. Patients with bilateral visual loss may not be Homonymous quadrantanopia secondary to a Meyer loop
aware of any such injury until an examination is performed. lesion causes contralateral one-quarter visual field loss in
Acute visual loss is often of vascular origin, including central both eyes.
retinal arterial or venous occlusion and cerebrovascular
disease. Neurologic causes, such as multiple sclerosis, may be
suggested by progression of the visual loss over a period of
hours or days, pain, and a history of additional neurologic
complaints with a recurrent waxing and waning pattern.
Inflammatory processes such as optic neuritis may be the
initial symptom of multiple sclerosis.
Neuropathy from temporal arteritis usually occurs in elderly
patients and is associated with progressive loss of vision (uni-
laterally or bilaterally), constitutional symptoms, jaw claudi-
cation, and headache.
Idiopathic intracranial hypertension should be considered
in patients with a history of headache, visual scotomata, and
visual changes. The typical patient is a young, heavy-set Fig. 95.1  This 60-year-old man had diabetes mellitus,
woman who is taking oral contraceptives. The headache and hypertension, coronary artery disease, chronic renal failure, and
visual changes are typically worsened by coughing, bend­ multiple myeloma. He sought medical care because of double
vision (he described the images as “a little side by side but mostly
ing over, or performing techniques such as the Valsalva
up and down”), diplopia, ptosis, and papillary sparing. Findings
maneuver. on laboratory tests, magnetic resonance imaging, and magnetic
Orbital compressive tumors or aneurysms cause mass resonance angiography were negative. The patient was evaluated
effects that compromise optic nerve function. by a neurologist and an ophthalmologist, and diabetic cranial nerve
palsy was ultimately diagnosed. He was given an eye patch and
Differential Diagnosis scheduled for ophthalmologic follow-up.
The differential patterns of visual loss are described in
Box 95.1.

Treatment superioris muscle (upper eyelid elevator) and the intrinsic

Treatment depends on the cause. Emergency ophthalmologic visceral motor function of the sphincter pupillae muscles and
consultation is essential for vascular causes. Treatment of the ciliary muscles, which perform pupillary constriction and
central retinal artery occlusion should focus on lowering intra- accommodation, respectively.
ocular pressure. Inpatient evaluation for neurologic causes is
warranted depending on the clinical findings. Temporal arte- Presenting Signs and Symptoms
ritis requires high-dose steroid therapy. Idiopathic intracranial The patient typically complains of double vision or difficulty
hypertension requires urgent diagnostic and therapeutic seeing out of the affected eye. There may be mild photophobia
lumbar puncture. in bright light. The patient may also complain of an inability
to raise the eyelid (ptosis).
CRANIAL NERVE III (OCULOMOTOR NERVE) Cranial nerve III palsy is more common in patients older
Anatomy than 60 years and in those with diabetes or hypertension
The oculomotor nerve is a pure motor nerve that works in (Fig. 95.1).
conjunction with cranial nerves IV and VI to coordinate extra- Patients with herniation syndromes will have a history of
ocular movements. The oculomotor nerve controls the supe- trauma (Fig. 95.2), tumor, or other neurologic findings.7
rior rectus (globe elevator), medial rectus (globe adductor), Pain associated with unilateral mydriasis should alert the
inferior rectus (globe depressor), and inferior oblique (globe emergency physician (EP) to look for an aneurysm involving
elevator) muscles. It also controls the levator palpebrae the terminal internal carotid artery. Computed tomographic

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SECTION IX NERVOUS SYSTEM DISORDERS

angiography is more reliable than magnetic resonance Presenting Signs and Symptoms
angiography.8 Patients with a fourth cranial nerve palsy have double vision
Patients with an abscess or cavernous sinus thrombosis may exacerbated by looking downward. The classic complaint is
have headaches, altered mental status, and seizures. This diag- difficulty going down stairs. Most commonly, a history of
nosis should be considered in patients with signs and symp- trauma is reported. On physical examination the patient may
toms in the contralateral eye, previous sinus or midface unconsciously tilt the head away from the affected side (Fig.
infection, fever, chemosis, eyelid or periorbital edema, and 95.3). Etiologic mechanisms are similar to those for the third
exophthalmos. Extension of internal carotid artery dissection cranial nerve and include inflammatory processes, trauma,
intracranially into the cavernous sinus can result in third, and vascular causes.10
fourth, and sixth cranial nerve palsies.9
Treatment
Treatment Treatment of isolated fourth nerve palsy is generally conser-
Treatment is dependent on the cause. CT should be performed vative, and the patient should be referred to neurology or
to exclude a herniating mass. Admission for magnetic reso- neurosurgery as appropriate.10 CT, MRI, and neurology con-
nance imaging (MRI) and neurology or neurosurgical consul- sultation are warranted if multiple cranial nerves are involved.
tation is indicated for acute-onset deficits.
CRANIAL NERVE V (TRIGEMINAL NERVE)
CRANIAL NERVE IV (TROCHLEAR NERVE) Anatomy
Anatomy The trigeminal nerve is a mixed motor and sensory nerve. It
The trochlear nerve innervates the superior oblique muscle of provides motor innervation to the muscles of mastication, as
the eye and causes inward rotation and downward and lateral well as sensation from the face, scalp, conjunctiva, globe,
movement of the globe. It is the smallest cranial nerve but has mucous membranes of the sinuses, tongue, teeth, and part of
the longest intracranial course. the external tympanic membrane.
The trigeminal sensory ganglion is located in the middle
cranial fossa and branches into three divisions: the ophthalmic
nerve (V1), the maxillary nerve (V2), and the mandibular
nerve (V3).

Presenting Signs and Symptoms

Patients with trigeminal nerve dysfunction have either sensory
or motor deficits. Sensory dysfunctions include paroxysmal
pain, paresthesias (abnormal sensations such as burning,
pricking, tickling, or tingling), dysesthesias (disagreeable,
unpleasant, or painful sensations produced by ordinary
stimuli), and anesthesia (loss of sensation). The motor dys-
function is usually described as difficulty chewing and diffi-
Fig. 95.2  Ptosis and mydriasis suggest a cranial nerve III
culty swallowing.
palsy. The appearance of these signs after a crush injury indicates Peripheral lesions cause loss of sensation or pain in only
that a skull fracture is impinging on the nerve canal. (Reproduced one division. Positive findings in two or more divisions (e.g.,
with permission from Baker C, Cannon J. Images in clinical loss of light touch in one division and loss of sensitivity to
medicine. Traumatic cranial nerve palsy. N Engl J Med pain, temperature, or pinprick in another division) should
2005;353:1955.) raise suspicion for a central cause.

A B
Normal eye rotation Cranial nerve IV palsy (right eye)
When the head tilts to the left, both eyes rotate Right eye extorted and slightly elevated, causing
in the opposite direction (right eye extorts, left double vision. To compensate, the patient tilts her
eye intorts) head to the left
Fig. 95.3  A, Normal eye rotation. When the head tilts, both eyes rotate in the opposite direction. B, Cranial nerve IV palsy (right eye). The
right eye is extorted and slightly elevated, which is causing double vision. The patient compensates by tilting the head to the left.

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 CHAPTER 95 Cranial Nerve Disorders

The presence of associated cranial nerve deficits (III, IV, onset of symptoms should raise suspicion for a vascular, trau-
IV, or any combination of these nerves) suggests cavernous matic, or demyelinating cause, whereas a more indolent course
sinus involvement. In the setting of trauma, if a bruit over the suggests tumor or inflammation (Table 95.2).
orbit can be detected, a carotid–cavernous sinus fistula may
be present. Associated involvement of cranial nerve VII or TRIGEMINAL NEUROPATHY  Causes include compression
VIII or gait ataxia should raise suspicion for a cerebellopon- by an extrinsic mass, trauma, and vascular, inflammatory, or
tine angle or lateral pontine tumor (Table 95.1). demyelinating disorders.
Associated Horner syndrome may indicate a cervical or Symptoms include neuralgia or paresthesia (or both) involv-
lateral brainstem lesion. ing half of the face. Unlike trigeminal neuralgia, the pain with
The main categories of trigeminal nerve dysfunction are trigeminal neuropathy is more constant. Loss of the corneal
trigeminal neuralgia and trigeminal neuropathy. A sudden reflex is evident. The patient’s mouth may become more oval

Table 95.1  Clinicoanatomic Correlation of Localization of Lesions of Cranial Nerve V

ANATOMIC SITE OTHER NEUROLOGIC

OF DAMAGE CLINICAL FINDINGS AND MEDICAL FINDINGS COMMON CAUSES

Supranuclear

Sensory cortex Facial numbness, paresthesias Neglect, apraxia, aphasia Stroke, tumor, hemorrhage

Internal capsule Hemifacial sensory loss Hemiparesis of the arm Stroke, tumor, hemorrhage, MS

Corona radiata Central seventh cranial nerve paresis

VPM thalamus Facial numbness, paresthesias, Anosmia, hemisensory deficit Stroke, tumor, hemorrhage
pain; cheirooral syndrome

Midbrain Facial numbness, paresthesias, pain Ophthalmoparesis Stroke, MS, tumor, aneurysm

Nuclear

Pons Facial numbness and weakness, Ophthalmoparesis; CN VI, CN VII, CN Stroke, tumor, hemorrhage;
paresthesias, pain; trigeminal VIII palsies; Horner syndrome MS, syringobulbia, abscess,
neuralgia trauma

Medulla Facial numbness, paresthesias, Ataxia, CN X palsy, ophthalmoparesis, Stroke, MS, tumor, aneurysm,
pain; trigeminal neuralgia nystagmus, Horner syndrome, abscess, vasculopathy
Wallenberg syndrome

Preganglionic

Cerebellopontine Facial numbness CN VII, CN VIII palsies; headache, Neuroma, meningioma,

angle cerebellar dysergia meningitis (bacterial, TB,
cancer), aneurysm, trauma

Middle cranial fossa

Gasserian Facial numbness and weakness Gradenigo syndrome; CN VI, CN VII Tumor, infection, trauma
ganglion palsies
Skull base Facial numbness and weakness Headache, meningismus Meningitis (bacterial, TB,
cancer, sarcoid)

Trigeminal Nerve Branches

V1: Cavernous sinus Facial numbness, pain Headache, ophthalmoparesis; Horner Tumor, thrombosis, infection,
syndrome trauma

V1: Carotid- Facial numbness Proptosis, bruit, ophthalmoparesis Trauma

cavernous fistula

V2: Maxillary region Facial numbness; numb cheek Tumor, infarct, vasculopathy,
syndrome trauma

V3: Mandibular Weakness of mastication; numb Tumor, trauma, infarct

region chin syndrome

CN, Cranial nerve; MS, multiple sclerosis; TB, tuberculosis; VPM, ventroposteromedial.

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Table 95.2  Selected Specific Causes Associated with Trigeminal Nerve Disorders

ETIOLOGIC CATEGORY SELECTED SPECIFIC CAUSES

Structural Disorders

Developmental Brainstem vascular loop, syringobulbia

Degenerative and compressive Paget disease

Hereditary and Degenerative Disorders

Chromosomal abnormalities, neurocutaneous disorders Hereditary sensorimotor neuropathy type I, neurofibromatosis

(schwannoma)

Degenerative motor, sensory, and autonomic disorders Amyotrophic lateral sclerosis

Acquired Metabolic and Nutritional Disorders

Endogenous metabolic disorders Diabetes

Exogenous disorders (toxins, illicit drugs) Trichloroethylene, trichloroacetic acid

Nutritional deficiencies, syndromes associated with alcoholism Thiamine, folate, vitamin B12, pyridoxine, pantothenic acid, vitamin A
deficiencies

Infectious Disorders

Viral infections Herpes zoster, unknown

Nonviral infections Bacteria, tuberculous meningitis, brain abscess, Gradenigo

syndrome, leprosy, cavernous sinus thrombosis

HIV infection, AIDS Opportunistic infection; abscess, herpes zoster

Stroke, hemorrhage, aneurysm

Neurovascular Disorders

Neoplastic Disorders

Primary neurologic tumors Glial tumors, meningioma, schwannoma

Metastatic neoplasms, paraneoplastic syndromes Lung, breast; lymphoma, carcinomatous meningitis

Demyelinating Disorders

Central nervous system disorders Multiple sclerosis, acute demyelinating encephalomyelitis

Peripheral nervous system disorders Guillain-Barré syndrome, chronic inflammatory demyelinating

polyneuropathy
Tolosa-Hunt syndrome, sarcoidosis, lupus, orbital pseudotumor

Autoimmune and Inflammatory Disorders

Traumatic Disorders Carotid-cavernous fistula, cavernous sinus thrombosis, maxillary/

mandibular injury

Epilepsy Focal seizures

Headache and Facial Pain Raeder neuralgia, cluster headache

Drug-Induced and Iatrogenic Neurologic Disorders Orbital, facial, dental surgery

From Goetz CG, editor. Textbook of clinical neurology. 2nd ed. Philadelphia: Saunders; 2003.
AIDS, Acquired immunodeficiency syndrome; HIV, human immunodeficiency virus.

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 CHAPTER 95 Cranial Nerve Disorders

and oblique in appearance, and because of loss of masseter in patients with a lower motor neuron lesion and accentuated
muscle strength, the chin may be deviated toward the affected in patients with a supranuclear lesion.
side.
Until proved otherwise, neuropathies of cranial nerve V, the Treatment
chin (numb chin; V3), and the suborbital region (numb cheek) A trial of carbamazepine can be therapeutic as well as diag-
should be presumed to be due to malignancies.11 nostic because failure to improve with carbamazepine sug-
gests some other cause. Treatment options are listed in Box
TIC DOULOUREUX  The term tic douloureux was coined by 95.3.3 Surgical approaches are considered when medication
Nicolaus André, a French surgeon, in 1756. Its mechanism is cannot control the pain or pain medication is not tolerated.13
probably compression of the trigeminal nerve root within mil-
limeters of entry into the pons.12 The maxillary and mandibu- CRANIAL NERVE VI (ABDUCENS NERVE)
lar divisions are most commonly affected, either alone or in Anatomy
combination. In one longitudinal case series, no cases of tri- The abducens nerve is a pure motor nerve that supplies the
geminal neuralgia affecting both the ophthalmic and man- ipsilateral lateral rectus muscle of the eye and controls globe
dibular divisions were reported.2 Causes of tic douloureux are abduction.
listed in Box 95.2.
The International Association for the Study of Pain defines PRESENTING SIGNS AND SYMPTOMS
tic douloureux as “a sudden usually unilateral, severe, brief, Patients with an abducens nerve palsy usually complain of
stabbing, recurrent pain in the distribution of one or more double vision. The head may be turned away from the affected
branches of the fifth cranial nerve.” The pain is classically side to maintain binocularity. Diabetes and hypertension are
precipitated by normal activities such as eating, talking, common risk factors. Another common sign is “crossed eyes”
washing the face, or cleaning the teeth. (esotropia or strabismus) (Fig. 95.4).14

Diagnostic Testing Differential Diagnosis

The presence or absence of a corneal reflex should be checked. Children are more likely to have a tumor as the principal
An intact reflex indicates normal function of the afferent V1 cause, and older individuals are more likely to have an isch-
division, as well as normal cranial nerve VII motor efferent emic cause such as temporal arteritis.
function. Absence of a corneal reflex can be caused by tumors An abducens nerve palsy occurring in isolation is rare.
in the posterior fossa or cerebellopontine angle, multiple scle- Usually, the seventh and eighth cranial nerves are also
rosis, brainstem strokes (Wallenberg or lateral medullary syn- involved, which signals a central cause. Causes of abducens
drome), and Parkinson disease. nerve palsy are listed in Box 95.4.
Motor function is evaluated by having the patient open and
close the mouth and laterally deviate the jaw against resis-
tance. Loss of muscle bulk or the presence of fasciculations
in the temporalis or masseter musculature indicates a lower
BOX 95.3  Treatment of Trigeminal Neuralgia
motor neuron lesion.
The jaw jerk reflex test determines the integrity of the V3
division. The examiner places a thumb on the patient’s chin, First-Line Agent
after which the patient is instructed to relax the jaw com- Carbamazepine (Tegretol)—Start at 150 mg daily and
pletely with the mouth closed, and the examiner then taps the increase by 100 mg every 3 days as needed to a total
chin to elicit the jaw jerk reflex. The reflex will be diminished daily dose of 800 to 1600 mg divided into three doses.
Second-Line Agents
Oxcarbazepine (Trileptal)—Start at 300 mg daily and increase
by 300 mg every 3 days as needed to a total daily dose
BOX 95.2  Causes of Tic Douloureux of 1200 to 1800 mg divided into two doses.
Gabapentin (Neurontin)—Start at 300 mg three times daily
Vascular compression by an artery or vein and increase as needed to a total daily dose of 3600 mg
Saccular aneurysm divided into three doses. Also commonly used as first-line
Arteriovenous malformation therapy.
Vestibular schwannomas Phenytoin (Dilantin)—Start at 300 mg daily and increase as
Meningioma needed, divided into two or three doses.
Epidermoid cyst Third-Line Agents (Add-On Therapy or Monotherapy)
Tumor Lamotrigine (Lamictal)—Start at 25 mg daily and increase by
Primary demyelinating disorders 25 mg every 7 days as needed to a total daily dose of 200
• Multiple sclerosis to 400 mg divided into two doses.
• Charcot-Marie-Tooth disease (rare) Baclofen (Lioresal)—Start at 15 mg daily and increase by
Infiltrative disorders 5 mg every 3 days as needed to a total daily dose of 60
• Trigeminal amyloidoma to 80 mg divided into three doses.
Nondemyelinating lesions
• Small infarct or angioma in the brainstem Reprinted with permission from Prasad S, Galetta S. Trigeminal neuralgia:
Familial historical notes and current concepts. Neurologist 2009;15:87-94.

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SECTION IX NERVOUS SYSTEM DISORDERS

BOX 95.4  Causes of Abducens Nerve Palsy

Trauma—a blowout fracture of the orbit may result in a

trapped medial rectus muscle and mimic a sixth nerve
palsy
Subarachnoid disorders—hemorrhage, infection (meningitis),
tumor
A Vascular—intracavernous aneurysms; sixth nerve palsies are
almost always the first clinical feature because of this
nerve’s close relationship to the carotid artery and the fact
that it is unsupported by a fibrous covering
Giant cell arteritis
Pontine glioma (in children)
Pseudotumor cerebri—may be manifested as an isolated
abducens nerve palsy in 30% of cases
Inflammatory (postviral or demyelinating) leptomeningeal
B involvement secondary to carcinomatous meningitis;
inflammatory or infiltrating lesions of the cavernous sinus
Metabolic—vitamin B deficiency, Wernicke-Korsakoff
syndrome
Congenital absence of cranial nerve VI (Duane syndrome)

C
in the literature. The palsy is often preceded by a viral syn-
Fig. 95.4  This 62-year-old man reported acute left retroorbital
drome, and a correlation has been noted with herpes simplex
pain of 1 week’s duration. Double vision developed, a rash
appeared on his forehead, and he had restricted abduction in
virus (HSV). Its association with shingles and the character-
his left eye; this finding is diagnostic of a left sixth cranial nerve istic blistering (from varicella-zoster virus [VZV]) is given
palsy (right, center, and left gaze seen in panels A, B, and C, the designation Ramsay Hunt syndrome. Reactivation of VZV
respectively) and binocular horizontal diplopia. A diagnosis of has also been theorized as a cause. In addition, Bell palsy may
herpes zoster ophthalmicus was made. The patient was treated be seen in patients with Lyme disease in places where the
with gabapentin and acyclovir for 1 week. Six weeks later, he disease is endemic.
had minimal residual diplopia with no postherpetic neuralgia. Diabetes, hypertension, human immunodeficiency virus
(Reproduced with permission from Jude E, Chakraborty A. Images infection, sarcoidosis, Sjögren syndrome, parotid nerve
in clinical medicine. Left sixth cranial nerve palsy with herpes zoster tumors, eclampsia, amyloidosis, and the intranasal influenza
ophthalmicus. N Engl J Med 2005;353:e14.)
vaccine have been associated with the development of Bell
palsy.5,16 Other common triggers include stress, trauma, fever,
tooth extraction, and a chilling episode from exposure to
drafts and cold.
Treatment Complete facial weakness, severe non–ear-related pain
Truly isolated sixth nerve palsies are often caused by micro- (e.g., retroauricular, cheek), late onset of recovery or no recov-
vascular ischemia secondary to hypertension or diabetes. A ery by 3 weeks, diabetes, pregnancy, age older than 60 years,
thorough work-up must be performed to rule out a central, hypertension, and Ramsay Hunt syndrome are risk factors for
inflammatory, infectious, or neoplastic cause. Close follow-up incomplete recovery.17,18
by a neurologist over a 6-month period is indicated; most Electroneurographic studies demonstrate a steady decline
cases resolve within 3 to 6 months. in electrical activity on days 4 to 10. When excitability
is retained, 90% of patients recover fully, but when
CRANIAL NERVE VII (FACIAL NERVE)— excitability diminishes to absence, only 20% of patients
BELL PALSY recover completely.5
Mechanisms
The pathophysiology of Bell palsy has not been clearly estab- Anatomy
lished. Several theories have been proposed, including infec- Cranial nerve VII is a mixed motor and sensory cranial nerve,
tious or ischemic inflammation leading to nerve compression which accounts for the varied symptoms. It travels adjacent
within the narrow canal as the nerve exits the stylomastoid to cranial nerves V, VI, and VIII as it traverses the cerebel-
foramen. Because the nerve is encased in a tight dural sheath lopontine angle, the internal auditory meatus, and the tempo-
within the temporal bone, this edema then causes additional ral bone.
compression of the vascular supply to the nerve.15 Motor function involves the muscles of facial expression,
The cause of Bell palsy is most commonly idiopathic the posterior digastric muscle, the stylohyoid muscle, and the
(66%).4 Numerous observed associations have been described stapedius muscle of the inner ear.

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 CHAPTER 95 Cranial Nerve Disorders

Fig. 95.5  “Raise your eyebrows.” A patient with a peripheral

seventh nerve palsy (i.e., Bell palsy) will have loss of forehead
wrinkles at rest and an inability to wrinkle the forehead and raise
the eyebrow on the affected side (right side in this patient). Fig. 95.6  “Show me your teeth”; “wrinkle your nose.” The
risorius and orbicularis oris muscles are denervated. Notice the
inability to corrugate the nose on the affected right side because of
loss of function of the nasal and buccal musculature.

Parasympathetic innervation includes the lacrimal glands,

the mucous membranes of the nose, the hard and soft palate,
BOX 95.5  Signs and Symptoms of Bell Palsy
and the submandibular and sublingual glands.
The geniculate ganglion contains the nerve cell bodies of
Ipsilateral tongue numbness
the sensory taste fibers of the anterior two thirds of the
Loss of taste or a dull taste
tongue.19
Overt paralysis preceded by a sensation of subjective numb-
ness or weakness on the affected side
Presenting Signs and Symptoms
Ear pain in the external auditory canal
To the patient, the most alarming symptom of Bell palsy is
Retroauricular pain
the abrupt onset of unilateral facial paralysis. Approximately
Occipital headache
50% of patients believe that they have suffered a stroke, 25%
Hyperacusis
think that they have an intracranial tumor, and the remaining
Fullness or snapping sound in the affected ear
25% have no clear conception of what is wrong but are
Tinnitus
extremely anxious.4
Drooling
The EP may note drooping of the eyebrow or the corner of
Inability to keep liquids in the mouth or chew
the mouth (or both) and loss of wrinkles on the forehead or
Noticeable dryness of the oral and nasal mucous membranes
the nasolabial folds (or both). Inability to raise the eyebrow
on the affected side
and furrow the forehead is a cardinal sign of Bell palsy (Fig.
Anxiety
95.5). Preservation of forehead motor neuron innervation
should raise suspicion for a central cause.17 Because the fore-
head receives bilateral upper motor neuron innervation, a
central stroke will spare the forehead and allow the patient to
raise the eyebrow. If the patient can do this, it is not Bell palsy. Diagnostic Testing and Differential Diagnosis
Loss of nasolabial fold and nasal flaring is common. Loss The “blow out your cheeks” test (Fig. 95.7) demonstrates loss
of buccinator strength causes an inability to blow out the of buccinator function. A sensitive variation of this test is to
cheeks. An inability to close the eye on the affected side is a ask patients to hold water in their mouth and contract the
hallmark of Bell palsy. Speech is affected and may sound buccal muscles. The water will either dribble out of the corner
slurred or garbled, similar to dysarthria from a stroke. An of the mouth or shoot across the room.
asymmetric smile is often noted on examination (Fig. 95.6). On testing of hearing, hyperacusis may be observed on the
The signs and symptoms vary depending on the site of the affected side because of denervation of the stapedius. The
affected nerve. They are listed in Box 95.5. patient should have no hearing loss.

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SECTION IX NERVOUS SYSTEM DISORDERS

Fig. 95.7  “Blow out your cheeks.” Loss of buccinator function

prevents pursing of the lips and allows air (and food and liquids) to
escape.

Fig. 95.9  Characteristic auricular rash of Ramsay Hunt

syndrome.

Treatment
The algorithm shown in Figure 95.10 outlines the treatment
of patients with Bell palsy.
Patients can be discharged home with oral medication,
instructions for eye care, and expedited follow-up with a neu-
rologist. Additional investigation for Lyme disease may be
indicated for patients at risk.
The evidence available indicates that steroids are safe and
effective in shortening the course of the neurologic deficit and
Fig. 95.8  Buccal herpetic lesions in an individual with improving facial function.5,20-24 Patients receiving steroid
Ramsay Hunt syndrome. therapy are up to 1.2 times more likely to attain good func-
tional outcomes than untreated patients are.25 Corticosteroids
reduce the risk for unsatisfactory recovery by 9%, with the
number needed to benefit (NNTB) being 11.21 Corticosteroids
To evaluate taste sensation, a few granules of sugar are were also associated with a 14% absolute reduction in risk for
placed on the tip of the patient’s tongue on the affected side. synkinesis and autonomic dysfunction, with an NNTB of 7.22
Decreased taste sensation may be noted. No studies have demonstrated significantly worse facial
Other cranial nerves should be normal. The abducens functional outcomes in patients treated with steroids.21,22,25
nucleus lies at the level of the genu of cranial nerve VII; The most commonly reported treatment regimen is oral
infarction in the area can cause concomitant palsy of cranial prednisone, 1 mg/kg up to 70 mg/day for a 10-day course.
nerve VI, which signals an upper motor neuron lesion rather Dosing can be once daily or split into twice daily. The starting
than Bell palsy. No evidence of expressive or receptive aphasia dose is continued for 6 days and tapered over the next 4
should be present. days.22,25 Alternatively, prednisone, 1 mg/kg/day, may be given
The presence of vesicles on the tympanic membrane or in for 7 days without a taper.4
the oropharynx (Fig. 95.8) or the presence of grouped vesicu- Recent studies and metaanalyses have questioned the
lar lesions on the face or around the ear (Fig. 95.9) suggests benefit of using antivirals for the treatment of Bell palsy.20-24,26
a diagnosis of Ramsay Hunt syndrome. Antiviral agents used alone did not provide any benefit over
Residual synkinesis can result from abnormal regeneration placebo, and their use as the sole therapeutic agent is not
of nerve fibers. This can be manifested as abnormal motor recommended.20 When combined with corticosteroid therapy,
function (e.g., blinking causes involuntary contracture of the antiviral therapy may have incremental benefit,21 but this
risorius); as abnormal parasympathetic function, which is remains to be shown conclusively. Therefore, until definitive
classically accompanied by “crocodile tears”—lacrimation studies are performed, clinical judgment will probably guide
after a salivary stimulus; or as hemifacial spasm, which can the use of antiviral therapy in cases in which a viral cause is
be bothersome, especially when the patient is tired. strongly suspected (i.e., patients in whom HSV or VZV is

826
 CHAPTER 95 Cranial Nerve Disorders

more significant than the physical disability. Patients report

self-consciousness about the facial disfigurement, fear of
Acute facial permanent disfigurement, loss of self-esteem, and social
weakness ostracism.

CRANIAL NERVE VIII (VESTIBULOCOCHLEAR

NERVE)
Anatomy
The vestibulocochlear nerve is a special sensory nerve that
Central or transmits auditory signals from the cochlea (hearing) and
peripheral? signals from the semicircular canals (balance). The vestibular
apparatus also sits in the petrous temporal bone and is com-
posed of a body consisting of the saccule and utricle and three
semicircular canals aligned in three different planes. Hair cells
Peripheral Central within the endolymph of the canals detect angular movement
and transmit the impulses to the vestibular nuclear complex
1) MRI to evaluate for in the floor of the fourth ventricle. The hair cells collectively
Herpes ischemia and for
infectious and combine to form the vestibular ganglion.
zoster
oticus inflammatory
diseases Presenting Signs and Symptoms
2) Consider: Patients with vestibulocochlear nerve dysfunction usually
a. CSF
No b. ESR
exhibit various degrees of hearing loss, tinnitus, vertigo,
c. Serologic studies falling, and imbalance. The mechanism is asymmetric integra-
Bell palsy Ramsay Hunt for syphilis, HIV, tion of vestibular input to the central nervous system or asym-
syndrome and vasculitis metric disruption of sensory input from the vestibular organs.31
3) Admit patient If the vertigo is severe, nausea and vomiting also occur.
1) Prednisone (started within 2-14
days of onset), 1 mg/kg/day for
Symptoms may be constant or episodic. Vestibular neuronitis
7-14 days. Taper to 10 mg/day causes vertigo that lasts for weeks, and central vertigo may
if more than 7 days persist for years.32
2) Valacyclovir 1000 mg three times Patients should be asked about triggers, particularly posi-
daily for 7 days as clinically tional triggers because this may indicate benign paroxysmal
indicated and only in conjunction
with corticosteroids positional vertigo. Recent viral and upper respiratory tract
infections may be significant because they predispose to ves-
Fig. 95.10  Algorithm outlining the treatment of patients with tibular neuronitis. The history should also include the use of
Bell palsy. CSF, Cerebrospinal fluid; ESR, erythrocyte medications such as anticonvulsants, antihypertensives, seda-
sedimentation rate; HIV, human immunodeficiency virus; MRI, tives, and ototoxic drugs.
magnetic resonance imaging. The examination should be focused on determining repro-
ducibility of the symptoms, gait, balance, and ataxia; on
evaluation of possible acute stroke symptoms; and on the
character of the nystagmus and severity of the ataxia. The
presence or absence of associated cerebellar signs such as
contributory). Valacyclovir, 1 g three times daily for 7 days, lateralizing dysmetria, motor weakness, sensory loss, and
can be prescribed in conjunction with corticosteroids. abnormal reflexes should be noted, as well as the Babinski
An eye shield or an eye patch should be worn during the reflex and cranial nerve abnormalities such as ophthalmople-
night to prevent drying of the cornea. Liberal use of artificial gia, dysarthria, and Horner syndrome.31 Abnormalities in cer-
tears during the day and an ophthalmic ointment such as ebellar function should prompt consideration of a central
Lacri-Lube at night should be prescribed to prevent drying of cause. Patients should also be examined for vertical and rota-
the cornea. Pain medication should be prescribed because the tory nystagmus, which are not typically present in patients
otalgia and cephalgia can be debilitatingly painful. with peripheral vertigo; their presence warrants imaging and
neurologic evaluation.
Prognosis
In a prospective study describing the spontaneous untreated Diagnostic Testing and Differential Diagnosis
course of idiopathic peripheral nerve palsy in patients with The Dix-Hallpike maneuver is commonly used to elicit posi-
diabetes, 38% of patients had complete palsies, and only 25% tional nystagmus (see Fig. 96.1), which is associated with
regained normal facial muscle function.4 This is significantly benign paroxysmal positional vertigo and usually lasts 5 to 60
worse than the observed rate of spontaneous full recovery in seconds. Prolonged nystagmus is unlikely to be a result of this
nondiabetic patients.27 Recurrence is rare (6.3%28) and should disorder. Gait and balance can be assessed with tandem
prompt a work-up for other causes such as myasthenia gravis, walking and the Romberg test. Ataxia and lateralizing dys-
lymphoma, sarcoidosis, Lyme disease, and rarely, Guillain- metria can be assessed with finger-to-nose and heel-knee-shin
Barré syndrome.5,29,30 testing. Hearing can be evaluated with the finger rub or finger
Although the prognosis for recovery is good, the psycho- snap, the Weber test, and the Rinne test. The ear and external
logic consequences can be long-lasting and are perhaps auditory canal should be examined for evidence of cerumen,

827
SECTION IX NERVOUS SYSTEM DISORDERS

otitis media, perforation of the tympanic membrane, and mass bradycardia or asystole caused by vagus nerve cardioinhibi-
lesions. tory input. Medical management is similar to that for trigemi-
CT lacks sensitivity in the evaluation of cranial nerve VIII nal neuralgia. If involvement of other cranial nerves is evident
disorders but may be useful in evaluating the bony temporal on examination, the patient should be admitted for further
region. MRI with gadolinium enhancement is useful in iden- evaluation and neurologic consultation.
tifying acoustic neuroma.
When a central cause is suspected because of abnormalities
on cerebellar testing or clinical suspicion, MRI or magnetic CRANIAL NERVE X (VAGUS NERVE)
resonance angiography (or both) should be performed to rule Anatomy
out a posterior circulation stroke as a central cause of the The vagus nerve is a mixed motor and sensory nerve
vertigo. that provides motor function to striated muscle of the pharynx,
The differential diagnosis should include other cranial tongue, larynx, and tensor veli palatini, as well as motor
nerve deficits that are not typically present in benign causes function to smooth muscle and glands of the pharynx, larynx,
of cranial nerve VIII dysfunction. Acoustic neuromas may and thoracic and abdominal viscera. Cranial nerve X provides
compress the trigeminal nerve when they attain a size of 3 cm general sensation from the skin at the back of the ear,
or greater; patients with complaints of facial numbness should the external auditory meatus, the pharynx, and part of the
therefore be evaluated for trigeminal neuropathy, as well for external surface of the tympanic membrane, as well as vis-
a mass lesion. Because large tumors can affect cranial nerves ceral sensation from the larynx, trachea, esophagus, and
IX, X, and XI, these nerves should also be tested. thoracic and abdominal viscera; from chemoreceptors in the
aortic bodies; and from stretch receptors in the walls of the
Treatment aortic arch.
Some patients who come to the emergency department with
sudden or severe symptoms may not be able to comply with Presenting Signs and Symptoms
testing because the severity of the symptoms limits the ability Patients with palsies of the vagus nerve generally have
to open their eyes and turn their head without experiencing hoarseness or difficulty swallowing. A history of recent
nausea and vomiting or exacerbating the symptoms. In these carotid or thyroid surgery should prompt suspicion for
cases it is appropriate to treat the patient symptomatically, a recurrent laryngeal nerve injury. The patient may also
initiate a work-up, and reassess clinically for improvement complain of regurgitation of food and liquid into the
before attempting to move the patient or perform provocative nose. Oropharyngeal examination usually reveals a drooped
testing. arch of the soft palate and uvular deviation away from
the affected side.
CRANIAL NERVE IX (GLOSSOPHARYNGEAL
NERVE) Treatment
Anatomy A CT scan of the head without contrast enhancement should
The glossopharyngeal nerve provides branchial motor func- be performed to evaluate for a cerebrovascular accident (hem-
tion to the stylopharyngeus muscle; visceral motor function orrhagic or ischemic) or skull-based lesions. Further inpatient
to the otic ganglion and parotid gland; visceral sensory func- evaluation may include MRI of the head and neck and work-
tion from the carotid body; somatic sensory function to the ups for metabolic, infectious, or inflammatory disorders as
posterior third of the tongue, the skin of the external ear, and warranted.
the internal surface of the tympanic membrane; and the
special sensory function of taste sensation from the posterior
third of the tongue. CRANIAL NERVE XI (ACCESSORY NERVE)
Anatomy
Presenting Signs and Symptoms The accessory nerve provides motor function to the sterno-
Patients with glossopharyngeal nerve palsy usually have asso- cleidomastoid and trapezius muscles.
ciated symptoms involving other cranial nerves, most com-
monly cranial nerves X and XI. The most common symptoms Presenting Signs and Symptoms
are dysphagia and choking. If the vagus nerve is involved, the Patients with accessory nerve palsies have neck and
patient complains of hoarseness and demonstrates ipsilateral shoulder weakness on the affected side. Inspection may reveal
paralysis of the soft palate. Head, neck, and oral trauma or a “dropped” shoulder—that is, the affected shoulder lying
surgery can cause acute dysfunction of cranial nerve IX. Glos- downward and in lateral rotation. Testing of the sterno­­
sopharyngeal nerve palsy is a known complication of tonsil- cleidomastoid reveals weakness when turning the head
lectomy surgery.33 against resistance to the contralateral side. Because of the
Glossopharyngeal neuralgia is a rare disorder consisting of proximity of cranial nerves IX and X, particular attention
paroxysms of pain in the back of the throat and tongue. The should be paid to these nerve functions on examination.
pain is similar to that of trigeminal neuralgia in that the attacks The most common causes are postoperative trauma (e.g.,
are brief, lasting seconds to minutes. It is unilateral and usually from cervical lymph node dissection) and a cerebrovascular
triggered by chewing, swallowing, coughing, or sneezing. accident.

Treatment Treatment
CT scanning is warranted to evaluate for a cerebrovascular Treatment and disposition are similar to that for cranial nerves
event or tumor. Rarely, vasovagal syncope can result from IX and X.

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 CHAPTER 95 Cranial Nerve Disorders

CRANIAL NERVE XII (HYPOGLOSSAL NERVE) neuron lesion causes contralateral tongue deviation and fas-
Anatomy ciculations, and tongue atrophy is absent. A lower motor
The hypoglossal nerve provides motor function to all the neuron lesion causes ipsilateral tongue deviation and fascicu-
intrinsic tongue muscles and three of the four extrinsic tongue lations, and tongue atrophy is present. A 26-year review of
muscles: the genioglossus, styloglossus, and hypoglossus. 100 cases of hypoglossal nerve palsy revealed that tumors,
predominantly malignant ones, produced nearly half of the
Presenting Signs and Symptoms palsies. Only 15% of patients made a complete or nearly
Patients with hypoglossal nerve palsies usually have unilateral complete recovery.34 External lesions that cause compression
tongue weakness. or stretching of the nerve include internal carotid artery dis-
section or aneurysm, intracranial tumor, abscess, and other
Differential Diagnosis pharyngeal space tumors.
The primary diagnostic consideration is distinguishing an
upper from a lower motor neuron lesion. An upper motor Treatment
Treatment and disposition are similar to that for cranial nerves
IX, X, and XI. If there is concern for a cerebrovascular acci-
dent or space-occupying lesion, the patient should be admitted
TIPS AND TRICKS for evaluation.

Patients with palsies of any of the 12 cranial nerves have

heterogeneous symptoms reflecting the intrinsic function SUGGESTED READINGS
of each nerve.
de Almeida JR, Al Khabori M, Guyatt GH, et al. Combined corticosteroid and
Patients with cranial nerve disorders generally have visual antiviral treatment for Bell palsy: a systematic review and meta-analysis. JAMA
disturbances, facial weakness or pain, or paresthesias, 2009;302:985-93.
depending on the nerve or nerves involved. Engström M, Berg T, Stjernquist-Desatnik A, et al. Prednisolone and valaciclovir in
Knowledge of the function of each of the cranial nerves helps Bell’s palsy: a randomised, double-blind, placebo-controlled, multicentre trial.
Lancet Neurol 2008;7:993-1000.
the emergency physician recognize the classic signs and Gilden DH. Clinical practice. Bell’s palsy. N Engl J Med 2004;351:1323-31.
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Trigeminal neuralgia and Bell palsy are common cranial Lancet Neurol 2008;7:976–7.
nerve disorders encountered in the emergency Salinas RA, Alvarez G, Daly F, et al. Corticosteroids for Bell’s palsy (idiopathic facial
paralysis). Cochrane Database Syst Rev 2010;3:CD001942.
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 CHAPTER 95 Cranial Nerve Disorders

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