Peripheral Neuropathies &

Other Disorders

dr. Yetty Hutahaean, Sp.S

Kuliah Modul-3 Blok 15

Fakultas Kedokteran Universitas Mulawarman
Kamis, 21 Desember 2010
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 Peripheral Neuropathy
• Disease or injury of peripheral sensory,
motor or autonomic nerves
• May be pure motor, pure sensory or mixed
sensorimotor
• May occur as mononeuropathy,
polyneuropathy or multifocal neuropathy

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Fig 1. Peripheral motor & sensory unit 3
Fig 2. Cross section of a mixed peripheral nerve
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 General Consideration

• Mononeuropathy
• Clinical syndrome of idiopathic acute
unilateral facial paralysis
• Incidence 1: 5000 people
• More common during pregnancy &
elderly

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Fig 3. True Bell palsy is idiopathic by definition7
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 Clinical Findings
• Unilateral facial weakness/facial drop,
most around mouth
• ↓ forehead wrinkling & raising eyebrow,
incomplete eyelid closure
• Patients may report exposure to cold
preceded their symptpms
• Facial numbness or stiffness without
objective sensory deficit
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 Clinical Findings

• Mild-moderate pain at the angel of jaw

• May be loss of teste 2/3 ant tongue
• Decrease tearing and ipsilateral
hyperacusis
• Not life-threatening  may have severe
functional, aesthetic & psycological
consequences

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 Treatment

• Pharmacotherapy
- Prednisone 1mg/kg/day for a week
- Antiviral (still controversial): acyclovir
400mg,5x/day, 7-10 days
if renal function is normal

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 Treatment
• Surgical Therapy: decompression as acute
therapy based on hypotesis ‘neuronal
swelling within temporal bone’  highly
invasive & risk permanent hearing loss
• Suportive Care
- Artificial tears & ophthalmic ointment
- Glasses or google

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 Prognosis
• 60 - 80% cases complete recovery in 1-2
months without treatment
• 90% achieve complete recovery with
corticosteroid
• 5% cases  permanent sequele
• During recovery period, may experience
involuntary activation of facial muscle in
ipsilateral region as a result of aberrant
reinnervation (synkinesias & crocodile
tear)
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 General Consideration

• Mononeuropathy
• Carpal tunnel, formed by carpal bones
& coverd ventrally by flexor
retinaculum
• Prone to compression with repetitive
flexion & extension
• Most common occupational diseases

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 Clinical Findings

• Pain, paresthesias or numbness in the

first 3 digits
• Worsen w/ repetitive/sustained wrist
flexion or extension
• Intermittent  constant
• Severe condition: hand weakness 
thenar flattening

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 Clinical Findings

• Tinel & Phalen test (+)

• Medical condition that may increase
risk: diabetes mellitus, hypothyroidism,
pregnancy, rheumatoid arthritis, obesity

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 Diagnostic Studies

• Electromyography & nerve conduction

studies
• Motor & sensory conduction time
through the carpal tunnel loss motor
amplitudes : worrisome sign
• Blood studies to asses for the risk factor
mention above

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 Treatment

• Pharmacotherapy
- anti-inflammatory agents
- Vitamin B6
- Short course of oral prednisone
- local corticosteroid injection

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 Treatment

• Conservative therapy: wrist splinting in

netral position at night & during
activities
• Surgical: carpal tunnel release surgery 
transection of the transverse carpal
ligament  excellent success w/ few
complication

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 General Consideration
• Acquired polyneuropathy
• Group of immune-mediated disorders
• Annual incidence 1-2 cases per 100.000
people
• Most common form: acute
inflammatory demyelinating
polyradiculoneuropathy (AIDP)

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 General Consideration
• Progressive weakness of extremities
w/ attenuation or loss of reflexes
• Target antigen susp on myelin sheat
• Patologically, demyelination begin in
the proximal nerves  distal

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 General Consideration
• Upper respiratory infection,
gastrointestinal infection or non
specific febrile illness, precedes
neurologic symptoms
• Campylobacter jejuni: most frequently
identified organism

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 General Consideration
• Subtypes:
1. Acute inflammatory demyelinating
polyradikuloneuropathy (AIDP)
2. Acute motor axonal neuropathy
(AMAN)
3. Acute motor sensory axonal
neuropathy (AMSAN)
4. Miller fisher syndorme

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 Clinical Findings
• Often begins 1-3 weeks after infection
• 70% patients initially have parestesias or
numbness in hands & feet  symmetris
weakness (typically ascending) 
progress days – weeks
• Paralysis max about 2 weeks (50%) & 1
month in > 90% pts.

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 Clinical Findings
• Respiratory paralysis may rapidly appear
(± ¼ AIDP)  necessitating intubation &
mechanical ventilation
• Autonomic nervous system involvement:
dangerous fluctuation od blood pressure
or cardiac arrythmia

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 Clinical Findings
• Examination:
- symmetric flaccid weakness
- normal sensation, despite sensory
symptom
- impending respiratory failure
- autonomic involvement: cardiac
arrythmia, BP fluctuation, flushing,
sweating, abnormal GI motility
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 Diagnostic Studies
• Lumbal puncture:
- CSF protein ↑ few days after
onset, peak in 4-6 weaks
- cell count normal/ mild
lymphocytic pleocytosis
 cyto-albumin dissociation
• Nerve conduction studies
• EMG : ↓ motor unit recruitment
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 Treatment
• Intravenous immunoglobulin: IVIG 0,4
g/kg/day for 5 days
• Plasmapheresis: 5-6 exchanges over 1-2
weeks
• Combination of both gives no additional
benefit
• Randomized trials of oral & intravenous
corticosteroid: failed to show benefit

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 Prognosis

• Most patients return to normal function

• After progression stops, symptoms
usually plateu for 2-4 weeks  gradual
recovery
• 20-25% require mechanical ventilation
 5% die

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 Prognosis
• Residual motor weakness in 25% after 1
year
• Poor prognostic factors: Older age,
ventilatory support, rapid progression
(<7 days), low motor amplitude  < 20%
probability of walkin independently at 6
months

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 General Consideration
• Most common of neuromuscular junction
disordes
• Acquired, predominantly antibody
mediated autoimmune disease
• Antibodies targeted against nicotinic
acetylcholine receptor (AChR)
• Prevalence 1: 10.000-20.000 people
• Comorbid thyroid disease (> 10%)

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 Clinical Findings
• Fluctuating, fatigable weakness of
commonly used muscles
• Hallmark features: ptosis, diplopia,
disarthria, dysphagia, respiratory &
limb muscles weakness
• Half of patients present w/ ocular
findings, usually bilateral, asymmetric

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 Clinical Findings
• Myasthenia crisis: respiratory
symptoms in conjunction with severe
bulbar symptom (15-20% patients) 
mechanical ventilation
• Risk ↑ pregnant women during first
trimester, improve during second &
third trimester

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 Diagnostic Studies
• Tensilon (edrophonium) test: 1mg iv 
3mg iv (3x)  Observe respon in 30-60’’
(negative if no response until 2’)
• Laboratory studies:
- AChR binding antibody
- AChR modulating antibody
• Electrodiagnostic studies
• Asses the present of tymoma by chest
CT-Scan/MRI
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 Treatment
• Symptomatic treatment
- Acetycholinesterase inhibitor
(pyridostigmin, at least 3-4x daily)
• Immunosupressive treatment
- Thymectomy
- Medical therapy: corticosteroid
- Plasmapheresis
- IVIG
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 Prognosis
• 80%  general myasthenic
• Cholinesterase inhibitor, low-
dosecorticosteroid may control symptom
• Patients w/ thymoma  more
aggressive disease course

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