Guillain-Barré Syndrome

The core features of Guillain-Barré syndrome are the acute onset of an ascending, predominantly motor polyradiculoneuropathy associated with areflexia and elevated cerebrospinal fluid protein. Tests of autonomic function are often abnormal. Orthostatic hypotension, impaired cardiovagal, sudomotor, and adrenergic vasomotor function are relatively common. The disorder is likely to be immune-mediated although the antigen is unknown. The evidence is based on the sural nerve biopsy finding of perivascular round cell infiltration, the presence of relevant serum antibodies causing demyelination, frequent antecedent infections, the selectivity of involvement by fiber type, and response to immunotherapy and the overlap with cases of pandysautonomia. Septic and autonomic complications are the major causes of death in GBS as respiratory failure is usually successfully managed with mechanical ventilation. Typically, autonomic neuropathy in GBS improves in concert with improvement in motor and sensory nerve function. Patients with autonomic instability require close monitoring of heart rate and BP. Paroxysmal hypertension may alternate with hypotension and patients may be supersensitive to pressor and depressor agents.