Guillain-Barré syndrome

Guillain-Barré syndrome (GBS), also known as acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in its most common form, is an acquired autoimmune disorder affecting the peripheral nervous system. It occurs when the body's immune system mistakenly attacks the peripheral nerves, damaging the myelin sheath (the protective covering around nerve fibers) and sometimes the nerve axons themselves. GBS is typically triggered by a preceding infection — most commonly Campylobacter jejuni gastroenteritis, but also respiratory infections, Epstein-Barr virus, cytomegalovirus, Zika virus, and occasionally following surgery or vaccination. The condition is not inherited and is considered a sporadic, immune-mediated disorder. The hallmark symptom of GBS is rapidly progressive, ascending, symmetrical muscle weakness that typically begins in the legs and spreads upward to the arms, trunk, and face. Patients often experience tingling, numbness, and pain in the extremities. Deep tendon reflexes are usually diminished or absent (areflexia). In severe cases, respiratory muscles can become paralyzed, requiring mechanical ventilation — a life-threatening complication occurring in approximately 20-30% of patients. Autonomic dysfunction, including blood pressure instability, cardiac arrhythmias, and bladder dysfunction, may also occur. Several clinical variants exist, including Miller Fisher syndrome (characterized by ophthalmoplegia, ataxia, and areflexia), acute motor axonal neuropathy (AMAN), and acute motor-sensory axonal neuropathy (AMSAN). Diagnosis is based on clinical presentation, cerebrospinal fluid analysis (showing elevated protein with normal cell count, known as albuminocytologic dissociation), and nerve conduction studies. Treatment involves supportive care and immunomodulatory therapies, primarily intravenous immunoglobulin (IVIg) or plasma exchange (plasmapheresis), both of which have been shown to hasten recovery when administered early in the disease course. Corticosteroids alone are not effective. Most patients recover, though the recovery period can range from weeks to months or even years. Approximately 20% of patients have significant residual disability, and mortality is estimated at 3-7%, primarily from respiratory failure, pulmonary embolism, or cardiac arrest. Rehabilitation, including physical and occupational therapy, plays an important role in recovery.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Guillain-Barré syndrome