Guillain-Barré Syndrome

Abstract

Guillain-Barré Syndrome (GBS) is an acute inflammatory demyelinating polyradiculoneuropathy due to immune-mediated attack against surface antigens in peripheral nerve myelin sheaths. Approximately two-thirds of patients have a history of recent upper respiratory tract infection, gastroenteritis (especially Campylobacter jejuni), immunization, or surgery. Patients usually present with a history of rapidly progressive symmetrical ascending weakness. This starts in lower extremities initially and then may progress to involve the trunk, upper extremities, respiratory muscles, and cranial muscles. Paresthesias and other sensory symptoms as well as back pain may occur early in many patients.

Initially, it is important to evaluate respiratory parameters which may indicate the need for urgent intubation and ventilation. Examination may reveal lower extremity-predominant hypotonia, weakness, and hypo- or areflexia. When there is more rostral involvement, facial diplegia and ophthalmoplegia may be present. Serology for Campylobacter jejuni may be added to routine labs, and a contrasted MRI of the spine may reveal lumbar root enhancement. Lumbar puncture with CSF analysis typically shows albumino-cytological dissociation, and electrodiagnostic studies show acquired demyelinating features. Treatment is centered on immune-modulating therapy with the use of either intravenous immunoglobulin or plasmapheresis.

Guillain-Barré syndrome may manifest with clinical variants including the Miller Fisher syndrome (with ophthalmoparesis, ataxia, and areflexia) and an acute axonal variant.