Guillain-Barre syndrome

Background

acute idiopathic inflammatory demyelinating polyneuropathy characterized by progressive muscle weakness and areflexia usually associated with sponatneous remission
Post-infectious autoimmune destruction of peripheral nerves
may be associated with hx of antecedent respiratory tract or GI infection (camphylobacter)
Characterized by:
Ascending paralysis; symmetric in legs. rapid onset.
Autonomic dysfunction: 50%
arrhythmias, brady/tachy, hypotension, sweating, urinary retention, respiratory failure
symptoms progress and peak ~2-4 weeks after onset, plateau for 2-4 weeks then remit from weeks to months
Intubation in 25% of pts.
90% full recovery in months with 5% Mortality
Forms:
Acute Inflammatory Demyelinating Polyneuropathy (AIDP)
demyelination is immunologically mediated
Acute Motor Axonal Neuropathy (AMAN)
pure motor form
seasonal incidence, associated with Camphylobacter infection
pts with this form tend to require ventilatory assistance
Acute Motor Sensory Axonal Neuropathy (AMSAN)
motor and sensory symptoms
Miller-Fisher (4%) variant is descending: ophthalmoplegia, ataxia, mainly affects CN's

Physical Exam:
symmetric weakness with diminished/absent reflexes
minimal loss of sensation
signs of autonomic dysfunction: dysarrythmias, orthostatic hypotension, transient/persistent hypertension, paralytic ileus, bladder dysfunction, abnormal sweating

nerve conduction study: slowed nerve conduction velocities, partial motor conduction block, abnormal temporal dispersion, prolonged distal latencies
LP: normal pressure, few cells (mononuclear), elevated protein (>50mg/dL)

Admit to neuro / ICU
Intubation (if indicated)
20/30/40 rule: patient with vital capacity <20mL/kg, max inspiratory pressure <30 cmH20, or max expiratory <40 cm H20 will generally progress to require mechanical ventilation
Supportive care, ABC's, close monitoring
Steroids iv (no proven benefit)
plasmaphoresis vs IVIG
DVT Prophylaxis (subQ heparin and SCDs)

Authors: