Pediatric
Neurology Review
Lorraine
Lazar, MD, PhD
Division
of Child Neurology
Goryeb
Children’s Hospital
Atlantic
Health
Topics to be reviewed
Headache
Seizures and
Epilepsy
Peripheral
Nervous System Disorders
Topics for Self-Study
Radiology
and Picture Book Review
Ataxia
Neurocutaneous
Syndromes
Review questions
and answers
HEADACHE
IN CHILDREN
Epidemiology of
Headache
uncommon
before 4 years
prevalence of all types increases with age
< 10-12
years equal among
sexes, male:female
1 : 1
> 10-12
years greater prevalence
in girls (1 : 1.5)
most are MIGRAINE
or TENSION
remission
occurs in 70% of
cases ages 9-16 years
1/3 remain
headache free after 6 years,
2/3 remain headache free after 16 years
Classification of Headache
PRIMARY =
Benign (Migraine, Tension, Cluster)
exam normal
no papilledema
normal neuroimaging
no fever /
meningismus, normal CSF
SECONDARY
= malignant, symptomatic
Something’s
wrong
Migraine
Genetic
predisposition, esp. “classic”
with aura
“common” migraine without aura - 70-85
% children
Triggers: sleep deprived, hunger,
illness, travel, stress
(only 50 % migraineurs can identify trigger)
Frontotemporal
pain (anterior, uni-
or bilateral)
Pulsating
quality (throbbing,
pounding)
Must have autonomic
symptoms:
Nausea/vomiting
or photo-/phonophobia, pallor
May be preceded
by transient aura (< 1 hr, 15-30 min)
Visual aura
most common
Association
of migraines in children with other conditions:
Somatic pain
complaints
Abdominal
(diffuse non-localizing crampiness)
8-15 % epileptic
children
21 % psychiatrically ill children
major depression
panic attacks
or other anxiety disorder
Migraine-related syndromes (variants)
Benign paroxysmal
vertigo
recurrent
stereotyped bouts of vertigo
often with
nausea, vomiting, nystagmus
Cyclic vomiting
recurrent
severe sudden nausea and vomiting
attacks last
hours to days
symptom-free
between attacks
Alternating
hemiplegia
repeated attacks
of L or R hemiplegia
onset before
18 months
normal at
birth, neurodevelopmental issues after onset
Paroxysmal
torticollis
benign intermittent
self-limited episodes of head tilt
spells last
hours to days
start in 1st
year of life, resolve by age 5 years
“Chronic
Daily Headaches” (months)
5+ per
week
15+ per
month
No underlying
pathology
Migraines that have changed
character:
Poor pain
control
Psychosocial
causes
Medication
overuse (aka “rebound
headaches”)
Tension
Pain typically posterior
> anterior, or band-like
Squeezing quality (tight, vice-like)
Neck muscles
sore
Common trigger: STRESS !
NO autonomic symptoms
NO nausea/vomiting
or photo/phonophobia
NO aura
Best treatments:
NSAIDs,
relaxation / biofeedback
Work-up of chronic recurrent
headache
Diagnosis
based on H &
P
No neuroimaging
if exam normal
Inadequate
evidence to support the value of
routine labs, or CSF
analysis
EEG may be normal or show non-specific abnormalities
(focal slowing, occipital spikes)
Does not
distinguish headache types
Does not distinguish
headache cause
NOT RECOMMENDED
for routine evaluation
Treatment for primary recurrent headache
Practice parameters
adapted from adult studies
Avoid / minimize triggers (MIGRAINES)
Optimize
hydration
Good sleep
hygiene / avoid sleep deprivation
Avoid hunger
Avoid food
triggers (aged cheeses, chocolate, caffeine/ soda, processed deli meats,
MSG, red wine)
Mind-Body
approach - minimize
stress (TENSION)
Biofeedback
/ relaxation
Acupuncture
Self-hypnosis
Acute treatments for migraines
Goals:
reduce / ablate pain, restore function, minimize need for rescue medications
Treat promptly at onset
Include anti-emetics
(if nausea / vomiting):
metoclopramide
(Reglan)
prochlorperazine
(Compazine)
promethazine
(Phenergan)
Avoid medication
overuse (meds
< 2-3 x per week)
1st line meds: NSAIDs
Triptans (serotonin
1B/1D receptor agonists):
sumatriptan
(Imitrex) intranasal or oral tablets (>
12 yo)
Prophylactic
treatments for migraines
Indicated
if headaches 1-2 x/ week or prolonged/ debilitating
propranolol (Inderal)
side effects
– hypotension, bradycardia
avoid in asthmatics, depressed
amitriptyline (Elavil)
side effects
– drowsiness, orthostasis, dysrhythmia
(EKG)
may require
6-12 week treatment to determine efficacy
anti-epileptics (topiramate, valproic acid,
carbamazepine, neurontin)
calcium channel
blockers (verapamil)
serotonin
agonists (cyproheptadine,
methysergide)
vitamins (B2 / riboflavin, magnesium)
Rethink the diagnosis of benign
headache
when:
headache is
always in the same
location
headache fails to respond
to multiple medical therapies
focal neurologic
findings appear (in
first 2-6 months)
VI n. palsy,
diplopia, new onset strabismus, papilledema
Hemiparesis,
ataxia
progressively
increasing frequency
/ severity of headache, headache worse with valsalva
headache awakens from sleep, worse in
the morning, AM vomiting
at-risk hx
or condition: VPS,
neurocutaneous disorder
Secondary
“symptomatic” headache
Increased
intracranial pressure
(brain tumor, brain abscess, hemorrhage, hydrocephalus, pseudotumor,
meningitis, VPS malfunction)
Vascular (stroke, intracerebral hemorrhage,
vasculitis, ruptured aneurysm or AVM)
Epilepsy (postictal or ictal)
Head and Neck
pathology (sinusitis,
dental abscess, trigeminal neuralgia, TMJ pain, carotid dissection)
Systemic Illness (HTN, DM, cardiac disease-source
of emboli/stroke)
Drug Use (analgesic overuse/rebound,
drug abuse-cocaine, psychostimulants, OCPs, steroids)
Psychological (depression)
NEUROIMAGING for headache (before
LP) if:
abnormal neurologic exam
altered
mental status
papilledema,
VI nerve palsy, diplopia, new onset strabismus
focal findings
(hemiparesis)
nuchal rigidity,
fever
change in headache frequency, intensity,
type
studies of
choice:
CT – BONE (skull fracture), BLOOD
(intracranial hemorrhage), ventricles (hydrocephalus), sinuses, mass
lesions, EMERGENCY (altered MS)
MRI – hydrocephalus, sinuses,
mass lesions, acute STROKE, vascular malformation
LP – NOT with focal mass lesion
on CT or MRI, but OK for pseudotumor, meningitis, subarachnoid hemorrhage after CT
CT of the brain
revealing contrast
enhancing
frontoparietal
tumor
(the white
mass) with surrounding
edema
(the darkened
region surrounding the
tumor) and mild effacement
of the left lateral ventricle.
Headache due to
Brain Tumor
Headache due to Intracranial
hemorrhage
EPIDURAL – CT of the brain revealing
acute
(white) blood collection (lens shaped)
causing significant mass
effect
SUBDURAL – CT of the brain revealing
sub-acute (gray) blood collection with
less severe mass effect
MRI
CT (C-)
Angio
Headache
due to
Intracranial
Hemorrhage:
Ruptured
AVM
Headache
followed by
acute
deterioration in
mental status
CT (C-)
CT (C+)
Angio
Left temporal
focal headache,
nausea, vomiting,
nuchal rigidity, photophobia
(**meningeal
irritation due
to blood,
not infection)
Headache
due to
Intracranial
Hemorrhage:
Saccular aneurysm
beginning to
rupture
Headache due to Hydrocephalus:
Choroid Plexus Papilloma
(CSF secreting intraventricular
tumor)
Obstructive / Non-communicating Hydrocephalus
due to Aqueductal
Stenosis
CT of the brain
reveals large
frontal and temporal
horns of the lateral
ventricles and a large
third ventricle, but
the
4th ventricle
is small.
If this were a male
with
flexed thumbs, think
X-linked
Hydrocephalus.
4th
Obstructive / Non-communicating Hydrocephalus
due to Chiari
Malformation:
low lying tonsils alone (Chiari I) – usually asymptomatic
low lying tonsils + hydrocephalus (Chiari II) – diffuse headache
*Type II with lumbosacral
myelomeningocele
Non-Obstructive / Communicating Hydrocephalus
due to Meningitis
CT of the brain
reveals enlarged frontal
and temporal horns of
the lateral ventricles
and
enlarged 3rd
and 4th
ventricles.
Headache, photophobia,
fever,
nuchal rigidity
(meningeal
irritation due to infection
and inflammation).
4th
3rd
MRI of the brain
revealing posterior
circulation strokes
(occipital cortex,
cerebellum and
brainstem)
Child with
sickle cell anemia
presenting with
headache, ataxia
and cranial
nerve palsies.
Headache due to Stroke
Traumatic dissection of
right internal
carotid
artery (ex.
running with pencil
in mouth, ex. whiplash
on
amusement park ride):
-“string sign” on
angio
- right MCA stroke on
CT
Headache due to
Neck Trauma
SEIZURES AND EPILEPSY
IN CHILDREN
Seizures and Epilepsy
Neonatal Seizures
(not epilepsy)
Febrile Seizures
(not epilepsy)
Infantile
Spasms (epilepsy)
Lennox-Gastaut
Syndrome (epilepsy)
Childhood
Absence (Petit Mal) Epilepsy
Juvenile Absence
Epilepsy
Juvenile Myoclonic
Epilepsy
Benign Rolandic
Epilepsy
Complex Partial
Epilepsy
Epidemiology of Seizures
and Epilepsy
4-6 % incidence of a single seizure in childhood
1% incidence
of epilepsy (>
2 unprovoked seizures) in childhood
70-80 % of
children “outgrow” their seizures
HISTORY is the most important tool in
differentiating a seizure from a non-seizure look-alike
EEG is an adjunctive
test to clinical
history
40% recurrence risk after 1st
unprovoked seizure
(up
to 80% recurrence
risk if EEG abnormal)
For 2nd
unprovoked seizure, 50%
occur within 6 months of 1st seizure
Epidemiology of Seizures
and Epilepsy
Increased recurrence risk if:
symptomatic
etiology (dev delay, MR / CP)
abnormal EEG
complex febrile
seizures
Todd’s paresis
nocturnal
seizures
+ FHx childhood
onset epileptic seizures
Factors that
do NOT influence recurrence risk:
patient age
seizure duration
Neonatal Seizures
(not epilepsy)
Benign Neonatal
Familial Convulsions
Onset 2nd
or 3rd day of life
No perinatal
complications
Autosomal
dominant condition (+FHx)
chromosomes
20 and 8
affected gene
product: alpha-subunit of Ach Receptor
Mixed seizure
types
apneic, clonic,
tonic, autonomic, oculofacial
Typically
easy to control seizures which resolve in 1st year of life
Neuroimaging
and EEG normal
Neonatal Seizures
(may progress to epilepsy)
Symptomatic
(secondary) neonatal seizures
Multiple Causes
Hypoxia-Ischemia
(HIE)
Infection
(meningitis, sepsis)
Hemorrhage
(IVH, subarachnoid, intraparenchymal)
Infarction
(thrombotic, hemorrhagic)
Metabolic
derangement (low sodium, low calcium, glucose)
Inborn errors
of metabolism
CNS malformation
Treatments:
IV phenobarbital, IV phenytoin,
IV benzodiazepines, **trial of IV pyridoxine 100 mg
Febrile Seizures (not
epilepsy)
2-4 % of children
age ~ 6 months – 6 years
Provoked by
a sudden spike in temp usually with URI, Acute OM, AGE (genetic predisposition)
“Simple”
Generalized
convulsion (whole body shaking)
Brief (<
15-20 minutes)
Only one in
the course of an illness
Future risk
of epilepsy 1% like other children
“Complex”
focal seizure (one side of body shaking, staring)
prolonged (> 15-20 minutes)
multiple in 24 hours
Complex febrile seizures hint at an increased risk of future epilepsy
Treatment of Febrile
Seizures (not epilepsy)
Considered
benign not warranting daily anti-seizure
medication
but phenobarbital
or valproic acid provide some prevention
Rectal Diastat (valium
gel) may be used to:
abort prolonged
complex febrile seizure
prevent complex febrile seizure clusters
(if child known to cluster)
prevent febrile seizure
recurrence during
a febrile illness
Anti-pyretics have NOT been proven to decrease the
risk of recurrent febrile seizures
Infantile Spasms (West
Syndrome) –
a severe epilepsy
Clinical spasms (1-2 secs)
a subtle
momentary flexion or
extension of the
body
occur in
clusters when drowsy
(waking or falling
asleep)
Severely abnormal EEG
pattern:
disorganized, discontinuous,
high amplitude, multifocal
spikes
called HYPSARRHYTHMIA
Treatment: ACTH
Infantile spasms
may be mistaken
for colic, reflux, hiccups, or a startle !
called symptomatic
if etiology identified:
brain insult
at birth (ex. hypoxia-ischemia, meningitis)
brain malformation
neurocutaneous
disorder (Tuberous Sclerosis)
metabolic
disorder
ARX Aristaless X-linked homeobox
gene mutation
called cryptogenic
if NO identifiable
cause
prognosis
best (10% good outcome)
if idiopathic
normal development
at onset of infantile spasms
extensive
etiology testing negative
prognosis
poor for:
seizure control (infantile spasms and future
seizures)
future neurocognitive
and developmental abilities
Lennox-Gastaut Syndrome
–
a severe epilepsy
Often evolves
from infantile spasms
Neurodevelopmentally
impaired children
Syndrome defined
by a TRIAD of:
1. mixed
seizure types: atonic,
atypical absence, myoclonic, tonic-clonic, partial
2. developmental
delay
3. abnormal EEG pattern: slow (< 2.5 Hz) spike wave
discharges
Symptomatic
or cyptogenic etiologies (like IS)
Prognosis
poor
Absence (Petit Mal)
Epilepsy
- Sudden onset of staring,
interrupting speech or activity
Occurs multiple times per day
Short duration
(seconds)
- Occurs in school aged
children ~ 4-12 years, otherwise
normal
Absence (Petit Mal)
Epilepsy
EEG
findings characteristic:
- bilateral generalized 3 Hz spike-and-wave
discharges
-
provoked by hyperventilation and photic
stimulation
Effective treatment: ethosuximide
(Zarontin)
Commonly resolves by
adolescence
Presumed genetic cause:
chromosome 8 (8q24) and 5 (5q31)
Juvenile Absence Epilepsy
onset a bit older than childhood absence epilepsy
in adolescence
(closer to middle school than elementary school)
similar staring seizures
but:
longer duration
fewer in frequency
higher risk for
other generalized seizures:
GTC
Myoclonic
less likely
to outgrow
EEG generalized spike wave discharges:
Faster than 3 Hz (4-6 Hz)
Juvenile Myoclonic
Epilepsy (JME)
Seizure types:
- myoclonic
in AM
- “grand mal”
- absence
EEG: bilateral
generalized
4-6 Hz
spike-wave or
polyspike-wave activity
Juvenile Myoclonic
Epilepsy (JME)
Seizures provoked by:
sleep deprivation
or arousals from sleep
photic stimulation
alcohol intake
Mean age at
onset 14 years
EEG: 4-6 Hz
spike wave provoked by photic stimulation (photosensitive)
Chance of
relapse 90% if medications discontinued—felt to require
lifelong treatment
Genetic predisposition
Candidate
gene on chromosome 6
Onset 3-13
years old, boys > girls
15% of epileptic
children
Normal IQ,
normal exam, normal MRI
May have +
FHx sz
Seizure description:
When awake:
twitching
and/or tingling on one side of body
speech arrest,
speech difficulty, may drool / gag
no loss of
consciousness, usually < 2 minutes
When asleep
(nocturnal):
“grand mal” with focal features
Benign Rolandic Epilepsy
Benign Rolandic Epilepsy
Aka Benign
Focal Epilepsy of Childhood
with Centrotemporal Spikes
EEG has
characteristic
pattern:
bilateral
independent
centrotemporal
spikes
Benign Rolandic Epilepsy
Treatment recommended only if:
Seizures
frequent (which is unusual)
Socially stigmatizing
if occur in wakefulness
Anxiety provoking
for parents if occur in sleep
Effective
treatments:
Avoidance
of sleep deprivation
Medications: carbamazepine, oxcarbazepine
Time (outgrown by adolescence)
Other Epilepsy Syndromes
Landau-Kleffner
Syndrome
an acquired EPILEPTIC APHASIA in a PREVIOUSLY
NORMAL child, usually
3-7 years old
Gradual or
sudden inability to understand or use spoken language (“word deafness”)
Must have EEG abnormalities in slow sleep (sleep activated)
Additional behavioral
and psychomotor disorders (hyperactivity, aggressiveness, depression, autistic features)
May have additional
overt clinical seizures (80 %) in sleep
Other Epilepsy Syndromes
Rett Syndrome
Occurs only
in girls (X-linked lethal mutation) – MECP2 gene mutation
Initial normal
development  dev
regression / autistic (loss
of motor / language / social skills)
Acquired microcephaly (deceleration of head growth)
Hand wringing / alternating hand movements
Irregular
breathing patterns
Apnea
Hyperpnea
Breathholding
Seizures
Partial (Focal) Epilepsy
onset of seizure
begins in one area of one cerebral hemisphere (apparent clinically or
via the EEG)
“simple”: no impairment of consciousness
“complex”: impairment of consciousness
(staring)
“secondarily
generalized”: a
simple or complex partial seizure that ends in a generalized convulsion
Most frequently
involved brain regions:
Temporal
Lobe (80 %) >
Frontal Lobe >>
Parietal or Occipital
MRI or CT:
Normal or
Abnormal
Neurologic
exam:
Normal or
Abnormal
Anatomic Onset of
Focal Epilepsies
Mesiotemporal sclerosis
Differentiating “Staring”
Seizures
Complex Partial
Seizures
+ aura
+ incontinence
+ postictal
lethargy
EEG with focal
spikes
lasts minutes
(but can
be shorter)
Absence Seizures
NO aura
NO incontinence
NO postictal
period (immediate recovery)
EEG with generalized
3 Hz spike wave activity
lasts seconds
(but can
be longer)
Spells that mimic
seizures
Apnea / ALTE
GER
Sleep disorders
(nocturnal myoclonus, night terrors, narcolepsy/cataplexy)
Migraine variants
(esp. aura)
Benign breathholding
spells
No neuro
consult / lab / EEG / CT, Fe for cyanotic type
Syncope
Movement Disorders
(tics, tremor, dystonia)
ADD
Behavioral
Stereotypies (PDD)
Pseudoseizures
(psychogenic seizures)
Strange
posturing, back arching, writhing
Alternating
L and R limb shaking during same seizure
Psychosocial
stressor
Medical triggers of
seizures (acute symptomatic seizures)
Hypoglycemia,
hyperglycemia
hypocalcemia
hyponatremia
/ hypernatremia
CNS infection
(meningitis, encephalitis)
acute trauma
toxic exposure
acute hypertension
Treatment of epileptic
seizures
often not
until after the second unprovoked seizure
choice of
AED based on maximum efficacy for that particular seizure type and minimal
side effects
70% become seizure
free on monotherapy
an additional 15%
become seizure free on polypharmacy
15% remain intractable
Discontinue
AED after 2 years seizure free EXCEPT for JME
Alternate
treatments:
Ketogenic
diet (high fat diet)
Vagal nerve
stimulator – FDA
approved for partial seizures in 12 years+
Epilepsy surgery
Classic side effects
of AEDs
valproic acid
(Depakote): hepatotoxicity,
weight gain,
acute
pancreatitis
lamotrigine
(Lamictal): Stevens-Johnson
syndrome
phenytoin
(Dilantin):
gingival hypertrophy, acute ataxia, osteoporosis
phenobarbital: adverse behavior / hyperactivity
carbamazepine
(Tegretol):
agranulocytosis, aplastic anemia
oxcarbazepine
(Trileptal):
hyponatremia
ethosuximide
(Zarontin):
lupus-like reaction
topiramate
(Topamax): weight
loss, acidosis, renal stones
felbamate
(Felbatol):
aplastic anemia
Status Epilepticus
Def:
seizure
lasting > 30 minutes or
repeated seizures
> 30 minutes without recovery in mental status between seizures
seizures >
1 hour associated with neuronal injury due to glutamate excitotoxicity
Evaluation
and treatment if seizure lasts > 5 minutes:
ABC’s
(RR, HR, BP)
check temp,
glucose, electrolytes, CBC, renal and hepatic function, AED levels
Benzodiazepine 
phenytoin 
phenobarbital
PERIPHERAL NERVOUS SYSTEM
DISORDERS
Weakness with NO UMN signs—
no hyperreflexia, no clonus,
no upgoing toes
Anterior horn
cell
Peripheral
nerve*
Neuromuscular
junction
Muscle
skin
*motor and sensory
Peripheral Nervous
System Disorders – motor impairment
Anterior Horn
Cell Disorders
Spinal Muscular
Atrophy (SMA) (genetic)
Poliomyelitis
(acquired)
Peripheral
Nerve Disorders
Guillain-Barre
Syndrome (acquired)
Charcot-Marie-Tooth
disease (genetic)
Neuromuscular
Junction Disorders
Myasthenia
Gravis
Infant botulism
Muscle Disorders
Duchenne and
Becker Muscular Dystrophy
ANTERIOR HORN
CELL
Peripheral
nerve
Neuromuscular
junction
Muscle
skin
Spinal muscular atrophy
(SMA)
Type 1 SMA = Werdnig-Hoffman (most severe)
Neonatal
/ early infancy onset
severe hypotonia,
breathing
/ swallowing difficulties
absent reflexes
tongue fasciculations
NO face / eye weakness
Prenatal
history – decreased fetal movements
Motor milestones: typically never sit
Autosomal
recessive SMN (survival
motor neuron)
gene mutation, gene
location 5q11.2-13.3
Type 2 – less severe, less slowly progressive
Motor milestones: typically sit, don’t walk
Type 3 (Kugelberg- Welander) – least
severe
Motor milestones:
may walk, but ultimately wheelchair bound too
Diagnosis of SMA:
Genetic
analysis – highly
sensitive and specific
If gene study
positive, no additional testing required
If gene study
negative,
EMG 
fibrillations (NCV portion of study  normal)
Muscle biopsy
– grouped atrophy
SMA Muscle biopsy reveals
grouped atrophy - grouped
fascicles of
muscle fibers lose innervation
Treatment considerations for SMAs:
aggressive
and early respiratory toilet
assisted ventilation for most type 1 SMA + many type
2 SMA
physical therapy to avoid / minimize contractures
encouragement
of full educational pursuits– intellect
unaffected
Poliomyelitis (infantile
paralysis)
viral infection
and destruction of anterior horn cells (spinal motor neurons)
flaccid asymmetric
paralysis usually of the legs
may involve
bulbar muscles
decreased
or absent reflexes
Anterior horn
cell
PERIPHERAL
NERVE*
Neuromuscular
junction
Muscle
skin
*motor and sensory
Guillain-Barre Syndrome
(GBS)
Most common ACUTE neuropathy in children
Most common cause of rapidly
progressive weakness
any age
ascending
bilateral paralysis
(acute / hours-days)
reflexes reduced or absent
“pins and
needles” (sensory
symptoms) in hands
and feet
back and hip
pain common in children
medical emergency if autonomic nerves
affected (ex. cardiac dysrhythmia) or if respiration affected
symptoms may worsen
in 1st 4
weeks
Miller-Fisher
variant = Areflexia
+ Ataxia + CN palsies
(ophthalmoplegia,
facial diplegia / “flat affect” / “decreased facial movements”)
Guillain-Barre Syndrome
(GBS)
aka Acute Inflammatory Demyelinating Polyradiculoneuropathy
(AIDP)
2/3 report antecedent infection 1-3 weeks prior
Campylobacter
jejuni (esp. China)
CMV
EBV
Hepatitis
Flu
mycoplasma
HSV
Diagnosis
of GBS:
*CSF (> 1 week) – elevated protein,
normal cells
Nerve Conduction
Velocities (NCVs) – slowing
MRI – may
show enhancement of spinal roots
Send titers
for suspected pathogens
Management:
No treatment if symptoms minimal, have nadired
or are improving
IVIG or plasmapharesis if ventilation affected, or
rapidly worsening and has not nadired
OT/PT
Prognosis:
Usually good
(~75%), recovery may take weeks to months
Poor prognostic
signs:
rapidly
progressive weakness < 7 days
assisted ventilation
Axonal involvement
(not just demyelination) – seen on NCVs as decreased amplitudes
Charcot-Marie-Tooth
(CMT) Disease
the most common CHRONIC neuropathy
in children, slowly progressive over decades
a hereditary
sensory motor neuropathy (HSMN)
Initial symptoms
noted > 10 years:
“pes cavus” – high pedal arches
“champagne
glass deformity” -
muscle atrophy below the knees
bilateral
foot drops - slaps
feet when walks, difficult to heel walk
may toe walk
reflexes reduced or absent
CMT
“Champagne-Glass Deformity”:
Distal
Muscular Atrophy of
Lower Extremities
High
Arched
Foot Deformity
“Pes
Cavus”