What is Guillain-Barré Syndrome (Guillain-Barré)

From the Guidelines

Guillain-Barré syndrome is a rare autoimmune disorder where the immune system attacks the peripheral nervous system, causing muscle weakness that typically starts in the legs and can progress upward to the arms and face. Guillain-Barré syndrome is characterized by muscle weakness, which can lead to significant morbidity and mortality if not promptly treated. The treatment of Guillain-Barré syndrome focuses on immunotherapy, with intravenous immunoglobulin (IVIG) at 0.4g/kg daily for 5 days or plasma exchange (5 exchanges over 2 weeks) being first-line options 1. Supportive care is crucial, including respiratory monitoring, deep vein thrombosis prophylaxis, pain management, and physical therapy. Patients often require hospitalization, with approximately 25% needing mechanical ventilation due to respiratory muscle weakness. Recovery typically begins within weeks but can take months to years, with about 80% of patients achieving full recovery 1. The condition is thought to be triggered by an infection (often respiratory or gastrointestinal) that causes the immune system to mistakenly attack nerve cells. Early treatment improves outcomes, so prompt diagnosis and intervention are essential when symptoms appear. Some key points to consider in the management of Guillain-Barré syndrome include:

• The importance of early treatment with IVIG or plasma exchange to improve outcomes
• The need for supportive care, including respiratory monitoring and pain management
• The potential for long-term residual problems, including incomplete recovery of motor and sensory function, fatigue, pain, and psychological distress 1
• The importance of planning rehabilitation before discharge to address these potential long-term effects.

From the Research

Definition and Characteristics of Guillain-Barré Syndrome

• Guillain-Barré Syndrome (GBS) is an acute polyradiculoneuropathy characterized by acute flaccid paralysis with or without sensory/autonomous nerve dysfunction 2.
• Symptoms may vary greatly in presentation and severity, including weakness, sensory disturbances, cranial nerve involvement, respiratory insufficiency, autonomic dysfunction, and pain 3.
• GBS includes acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, acute motor and sensory axonal neuropathy, Miller Fisher syndrome, and acute pandysautonomia 4.

Diagnosis of Guillain-Barré Syndrome

• Diagnosis is based on a combination of clinical presentation, cerebrospinal fluid (CSF) examination, electrodiagnostic testing, and testing for anti-ganglioside antibodies 3.
• CSF examination is valuable, particularly when the diagnosis is less certain, and electrodiagnostic testing is advised to support the diagnosis 3.
• Testing for anti-ganglioside antibodies is of limited clinical value in most patients with typical motor-sensory GBS, but anti-GQ1b antibody testing should be considered when Miller Fisher syndrome (MFS) is suspected 3.

Treatment of Guillain-Barré Syndrome

• Intravenous immunoglobulin (IVIg) and plasma exchange (PE) are effective treatments for GBS, with IVIg being more likely to be completed than PE 5, 6.
• IVIg started within two weeks from onset hastens recovery as much as PE, and giving IVIg after PE does not confer significant extra benefit 5, 6.
• The use of corticosteroids alone is ineffective, and more research is needed in mild disease and in patients whose treatment starts more than two weeks after onset 5, 6.