Motor Disturbance in Children
Motor Disturbance in Children
Motor Disturbance in Children
disturbance
About Children who trip and
stumble . . .
Common Patterns
Toe walking
Common Patterns
Toe Walking
Clumsiness, Stumbling and Tripping
Common Patterns
Hypotonia
Common Patterns
Clumsiness, Stumbling and Tripping
Toe walking
Hypotonia
Imbalance
Common Patterns
Clumsiness, Stumbling and Tripping
Toe walking
Imbalance
Hypotonia
Peculiar walking patterns
Common Patterns
Clumsiness, Stumbling and Tripping
Toe walking
Imbalance
Hypotonia
Peculiar movements
Weakness
Common Patterns
Clumsiness, Stumbling and Tripping
Toe walking
Imbalance
Peculiar movements
Weakness
Deformities
Common Patterns
Clumsiness, Stumbling and Tripping
Toe walking
Imbalance
Peculiar movements
Weakness
Deformities
Very Peculiar Signs
Motor Development
Newborn
5 months
7 months
10-11 mo
Newborn
6 weeks
3 months
(12 weeks)
5 months
6 months
5 months
6 months
7 months
4 months
6 months
5 months
7 months
5 months
4 months
6 months
7 mo
8 mo
9 mo
10 mo
Verticalization
11 mo
7 months
8 months
9th months
9 10 months
10-11 months
PHENOMENOLOGY
Gross motor clumsiness
Fine motor clumsiness
Abnormal movements
Abnormal posture and gait
Muscular weakness
Hypotonia or Spasticity
LOCALIZATION
CENTRAL MOTOR NEURON
CORTEX
CORTICO-SPINAL
TRACTS
Cells
Motor Roots and Peripheral Nerves
Neuro-Muscular Junction
Muscles
ETIOLOGY
Static Dysfunction
cerebral
palsy
Trauma
Acute
Infection , Toxicity
CVA
Progressive Condition
Metabolic,
Genetic
Autoimmine
Chronic Infection
Hypotonia
CENTRAL
PERIPHERAL
CEREBRAL HYPOTONIA
Encephalopathy present !
Change in Alertness and Activity
Seizures *Cognitive Delay
Signs of Upper Motor Neuron Disease:
Reflexes Increased
Pathologic reflexes
Persistence of Primitive Reflexes
Motor Delay
Functional Asymmetry
Progression to Spasticity
PERIPHERAL HYPOTONIA
Normal Brain Functions
Signs of Lower Motor Neuron Disease:
Reflexes Depressed (Myopathy) of Absent
Signs of Denervation of Muscles: Amyotrophy,
Fasciculations
Low Tone in Extremities
No Spasticity
Pathologic reflexes Absent
Decreased Movements and Postural Reactions
NO Functional Asymmetry
Electrophysiologic Findings
Fibrillation Potentials
Abnormal Nerve Conduction
CEREBRAL SYNDROMES
Benign Hypotonia of Infancy
(Common, Self-limited)
Hypotonia of the Prematurity
Hypoxic Ischemic Encephalopathy
Encephalopathic Syndromes
GENETIC SYNDROMES
Downs Syndrome
21 translocation, deletion, mosaicism
Prader-Willi Syndrome 5 (q11-13) deletion
HHHH: -Hypotonia,
-Hypogonadism,
-Hypomentia;
Hypo Hyperphagia
Small Hands and Feet
Microcephaly, Short Stature
Congenital Hip Dislocation
Work Up:
Imaging studies
Nerve Conduction Studies and EMG
EEG
Laboratory Studies: CPK; SGOT/SGPT
Screening Tests; Amino Acids and Organic
Acids; Long Chain Fatty Acids; Lactate and
Pyruvate; Chromosomal DNA testing
Muscle and/or Nerve Biopsy
Right Tibi
Ankle 100.0mA
O
P
R
O
Right Perone
Poplit 100.0mA
0.53 mV [>3.0]
43.75 m/s [>41.0]
Amp:
Vel:
Ankle 100.0mA
P
R
O
T
Amp:
Vel:
0.18 mV [>2.5]
43.0 m/s [>40]
EMG - Left
100
2
Dysmorphic Features
Hypotonia, H/o clubbed feet
Posture and Gait Abnormality
Distal Legs Wasting:
Work Up:
Brain Imaging
Cervical Imaging (r/o syrinx)
EMG and Nerve Conduction
Studies:
Normal Cognitive
Development
Chronic Presentation
Subcompensated course
Gowers Sign
Toe Walking
car accident
Conversion reaction
Ataxia
Ataxia Differential:
Brain Abnormalities:
Leukodystrophies (Krabbe)
Cerebellar
Frontal
Posterior Columns
Spino-Cerebellar Ataxias
Ataxia-Telangiectasia
Neuropathies
Postinfectious (Varicella Cerebellitis)
Autoimmune (Miller-Fisher Variant of GBS)
MUSCLE
DISEASE
Muscle Weakness
Gowers Sign:
Muscle Weakness
Gowers Sign:
Paretic Gait:
Muscle Weakness
Gowers Sign
Reflex abnormalities
Tone Abnormalities:
Muscle Weakness
Gowers Sign
Reflex abnormalities
Tone Abnormalities
Muscle Deformities:
Muscle Phenomena
Myotonia:
Muscle Phenomena
Myotonia:
Neuropathies
Myopathy
Muscular Dystrophies
Myasthenia
Myotonic Dystrophy
Dystonia Differential:
Degenerative Encephalopathies:
Pelizaeus-Merzbacher
Lesch-Nyhan
Infantile seroid lipofuscinosis
Niemann-Pick
Mitochondrial diseases
Genetic Syndromes
Metabolic Syndromes
Biotinidase deficiency,
Methylmalonic aciduria, Glutaric aciduria
Pediatric neurotransmitters deficiencies
Bilirubin Encephalopathy
Potentially Treatable
-Glucose transporter deficiency; Biotin Deficiency;
DOPA-responsive, Homocystinuria; Wilson's D-se;