Guillain-Barré Syndrome

This article reviews the epidemiology, clinical symptoms, examination findings, pathogenesis, diagnostic criteria, and recent guidelines of Guillain-Barré syndrome (GBS) and its variants, including information on outcome measures, management, and ongoing research.

CSF examination and electrodiagnostic testing are useful in some cases to support the diagnosis, particularly when GBS variants are suspected. Antibody testing is of limited value in typical sensorimotor GBS, but useful when GBS variants and nodopathies are considered. According to evidence-based guidelines for the diagnosis and treatment of GBS developed by the European Academy of Neurology and Peripheral Nerve Society, IV immunoglobulin (IVIg) or plasma exchange are the recommended treatment options. The guidelines recommend against using a second IVIg course, or corticosteroids or plasma exchange followed immediately by IVIg. For treatment of pain, gabapentinoids, tricyclic antidepressants, or carbamazepine have been recommended. The Modified Erasmus GBS outcome score and Modified Erasmus GBS Respiratory Insufficiency Score have been recommended. Research is ongoing to find new treatment options.

GBS is a life-threatening neuromuscular emergency seen frequently worldwide. Symptom progression for less than 4 weeks and findings of sensorimotor deficits and areflexia are required to make the diagnosis. Some patients may present with pain, cranial nerve involvement, autonomic dysfunction, or respiratory insufficiency. If progression continues after 8 weeks from onset, consider the diagnosis of acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which is seen in 5% of patients initially diagnosed with GBS. Approximately 20% of patients have significant residual deficits and poor prognosis despite receiving available treatments.