Fact-sheet: Guillain Barre Syndrom - Acute inflammatory polyradiculoneuritis
Updated on 08/23/2017 at 7:16 AM
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Definition
Acute inflammatory polyradiculoneuropathy with multifocal segmental demyelination of autoimmune origin. Epidemiology: - Incidence: 1/100,000 - Two age peaks: young adults and the elderly - Slight male predominance. Triggering factors: - viral infection (Campylobacter jejuni gastroenteritis, CMV, EBV) - vaccination - trauma - surgical procedure
Clinical features
May be preceded by a flu-like syndrome (viral infection).
THREE-PHASE COURSE:
1) Progression phase (4 weeks):
- Relatively symmetric, ascending, progressive peripheral paralysis (loss of deep tendon reflexes) predominating proximally, then involving the extremities.
- Cranial nerve involvement is rare.
- Sensory involvement is frequent and mostly subjective, ascending.
- Pain: myalgias, lower limb radicular pain.
- Respiratory involvement determines vital prognosis.
2) Plateau phase (variable, up to several months):
- Fixed motor and sensory deficit of variable intensity.
- Autonomic nervous system involvement is frequent (tachycardia, orthostatic hypotension, sweating abnormalities, constipation) in severe forms.
3) Recovery phase (variable, up to several months).
In children: symptoms are more subtle and present as severe hypotonia, respiratory distress, vomiting, headache, and limb pain.
Laboratory findings
LUMBAR PUNCTURE:
- elevated CSF protein, which may exceed 1 g/L, delayed by 3 to 10 days relative to clinical onset.
- absence of cellular reaction
( ---> ALBUMINOCYTOLOGIC DISSOCIATION
MRI
Enhancement and thickening of the cauda equina nerve roots and around the conus terminale.
Management
Treatment with IV immunoglobulins.
Differential diagnosis
- Neoplastic: leptomeningeal carcinomatosis, leptomeningeal lymphoma
- Infectious: Lyme disease, tick-borne encephalitis
- Sarcoidosis
- Arachnoiditis
- Krabbe disease