Peripheral Neuropathy

Neurology Rotation Lecture Series

Last Updated by Lindsay Pagano
Summer 2013

Case Presentation
You are consulted by the NICU to see a 3500 g newborn male with
decreased movement of his right upper extremity.
born at 39 weeks via SVD, vacuum assist
Pregnancy complicated by LGA, mom with IDDM
Complicated by nuchal cord x1, shoulder dystocia with left humeral fracture
Apgars 0, 2, 6 at 1, 5, 10 minutes of life, respectively
Resuscitated, intubated, and brought to the NICU
PE: normal except for
S
Neuro: normal except for
MS: mildly sedated
Motor: RUE with hypotonia, internally rotated, with elbow extension
and forearm pronation; no spontaneous motion of RUE
Sensory: no movement of RUE to pain
Reflexes: trace-absent in RUE
Differential?

Brachial Plexus Injuries

Injuries to nerve roots arising from C5-T1 from traction, compression, tear or avulsion
0.5-2 per 100 live births

More common in term infants

More common in RUE
Associated with LGA, GDM, AMA, shoulder dystocia, clavicular fractures
Also seen in nontraumatic births, and in utero

Erbs Palsy
Injury to superior trunk, C5-6
waiters tip: internal rotation and adduction of the shoulder, elbow extension, forearm
pronation, finger flexion

Klumpkes Palsy
Injury to inferior trunk, C8-T1
Elbow flexion, forearm supination, wrist extension, claw deformity of the hand
+/- Horner Syndrome due to sympathetic fiber involvement

Prognosis
Recovery over 3-6 months is predictive of overall recovery; PT/OT important
May require surgery to reanneal or graft the injured nerves (earlier [by 6 months] is better)

Bells Palsy

Acute facial nerve palsy of unknown etiology; may be congenital

2/10,000 per year
Onset over hours of unilateral facial palsy
Includes the ENTIRE face (LMN)
May also include hyperacusis, decreased
tearing, loss of taste
May be associated with

OM
Lyme
Mobus syndrome (with ocular abduction palsy)
Viral infections: varicella, zoster, coxsackie

Diagnosis: clinical
Treatment: No clear evidence for steroids or acyclovir in children (AAP insufficient
evidence to recommend treatment)

CIDP

Aka Chronic Inflammatory Demyelinating Polyneuropathy

Like a chronic form of Guillan-Barr, which is also known as AIDP (acute
inflammatory demyelinating polyneuropathy)
Immune mediated demyelinating polyneuropathy
Presents with lower extremity weakness and sensory changes, and less commonly, CN
neuropathies
One of three clinical syndromes:
1. Monophasic illness with symptoms lasting > 2 months
2. Slow progressive course
3. Relapsing/remitting course
NCS consistent with demyelination
LP with increased protein
Treatment:
Scheduled IVIg
Other options: PLEX, steroids

Hereditary Motor and Sensory Neuropathy

aka Charcot-Marie-Tooth disease type 1A

AD, duplication in the peripheral myelin protein 22 (PMP22) gene on 17p11.2-12

Progressive distal polyneuropathy
Begins in intrinsic foot muscles, and then progresses to include proximal weakness, as
well as to involve hand muscles
Hammer toes, pronounced arch, thin tapered appearance of legs
Onset in first 2 decades
First symptoms include:

Difficulty with heel walking

Pes cavus
Calf atrophy
Decreased reflexes

Progresses to include
Worsening of above
Impairment of vibration and proprioception

Touch, pain and temperature sensation less commonly involved

Diagnosis
EMG/NCS
Genetic testing

Other Peripheral Neuropathy Differential Diagnoses

Toxins/heavy metals
Eg arsenic, lead, thallium
Hypothyroidism
Systemic conditions: diabetes, uremia, porphyria, SLE, Churg-Strauss,
polyarteritis nodosa, lyme, rabies, sarcoid
Older individual lab screening
CBC, CMP, SPEP, glucose tolerance test, ESR, B12, ANA, ANCA, RF,
SSA, SSB, B6, B1, PNP, anti-gliadan Ab
Complication of metabolic disease
Not usually the presenting anomaly
Leukodystrophies, lipid storage diseases (Gaucher, Tay-Sachs),
peroxisomal disorders, mitochondrial disorders

PREP question
A 14 year old boy presents to your office because the side of his face is drooping.
His mother states that he complained yesterday of decreased food taste. Today, while at
school, he could not use the microscope in science class because he couldnt close his left
eye, and his teacher noted that his smile was crooked.
PE reveals no abnormalities and no vesicles in his ears. Mental status on neuro
exam is normal, pupil responses are normal, extraocular movements are full, and there is no
nystagmus or reported double vision. He is unable to close his left eye or raise his left
eyebrow, has decreased left-side nasolabial folds, and cannot close his mouth to puff out his
cheeks. His palate and tongue movements are normal. Motor examination reveals normal
proximal and distal strength in both arms and normal regular and tandem gait.
Of the following, the MOST appropriate initial diagnostic procedure is
A.Blood test for antistreptococcal antibodies
B.Brain magnetic resonance imaging
C.Edrophonium (Tensilon) test
D.No further testing
E.Noncontrast head computed tomography scan

D. No further testing
Evaluating for Bells palsy
Localize the weakness
Look for sensory involvement
No clear evidence for steroids or acyclovir in
children (AAP insufficient evidence to
recommend treatment)

Regarding the other choices:

A. ASO: typically associated with abnormal movements, not a fixed deficit
B. MRIbrain: doesnt fit localization
C. Edrophonium test: not consistent with MG (UL, ptosis, resp symptoms)
E. Noncon CThead: see B