Guillain-Barré Syndrome (GBS)
Guillain-Barré Syndrome is an autoimmune disease characterized by progressive bilateral weakness, decreased or absent deep tendon reflexes, and sensory disturbances, typically triggered by a preceding infection, requiring prompt diagnosis and treatment with either intravenous immunoglobulin or plasma exchange to improve outcomes. 1
Clinical Presentation
- Progressive bilateral weakness: Typically ascending, starting in the legs and moving upward
- Decreased or absent deep tendon reflexes in affected limbs
- Sensory symptoms: Distal paresthesias or sensory loss
- Pain: Often severe and may precede weakness
- Autonomic dysfunction: Blood pressure/heart rate instability, pupillary dysfunction, bowel/bladder issues
- Cranial nerve involvement: Facial weakness, difficulty swallowing
- Disease progression: Most patients reach peak disability within 2 weeks, followed by a plateau phase lasting days to weeks/months 1
Diagnostic Features
Key clinical findings:
- Progressive bilateral weakness of arms and legs
- Absent or decreased tendon reflexes in affected limbs
- Relative symmetry of symptoms
- Progressive phase lasting days to 4 weeks
- History of recent diarrhea or respiratory infection (approximately two-thirds of patients report infection 4-6 weeks preceding onset) 1
Laboratory testing:
- Cerebrospinal fluid (CSF): Typically shows albumino-cytological dissociation (elevated protein with normal cell count)
- Electrodiagnostic studies: EMG and nerve conduction studies showing sensorimotor polyradiculoneuropathy/polyneuropathy
- Anti-ganglioside antibody testing: Limited value in typical GBS, but anti-GQ1b antibodies found in up to 90% of Miller Fisher syndrome cases 1
GBS Variants
- Acute inflammatory demyelinating polyneuropathy (AIDP): Most common form in Western countries
- Acute motor axonal neuropathy (AMAN): More common in Asia and Japan
- Miller Fisher syndrome (MFS): Characterized by ophthalmoplegia, ataxia, and areflexia
- GBS-MFS overlap syndromes 2, 3
Treatment
First-line therapies (should be initiated as soon as possible after diagnosis):
Not recommended:
- Corticosteroids as monotherapy
- Combination of PE followed immediately by IVIg 2
Pain management:
Respiratory Management
- Up to 30% of patients develop respiratory failure requiring mechanical ventilation 4
- Monitor for signs of respiratory compromise:
- Declining forced vital capacity
- Shortness of breath
- Difficulty clearing secretions
- Use of accessory muscles of respiration 5
Prognosis and Outcomes
- 60-80% of patients can walk independently 6 months after disease onset
- Recovery may continue for more than 3 years
- Approximately 20% remain unable to walk after 6 months
- Mortality rate: 3-10% despite optimal treatment 1
Poor Prognostic Factors
- Advanced age
- Rapid progression of symptoms
- Need for mechanical ventilation
- Severe axonopathy on electrophysiological studies 1
Long-term Complications
- Pain, fatigue, and other residual symptoms may persist for months to years
- Recurrence is rare (2-5% of patients) 1
Special Considerations
- About 10% of GBS patients experience treatment-related fluctuations requiring repeated IVIg treatment
- Approximately 5% of patients initially diagnosed with GBS develop chronic inflammatory demyelinating polyradiculoneuropathy with acute onset (A-CIDP) 2, 3
- Consider changing diagnosis to A-CIDP if progression continues after 8 weeks from onset 2
Prognostic Tools
- Modified Erasmus GBS outcome score (mEGOS): Predicts recovery
- Modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS): Assesses risk of requiring artificial ventilation 2
GBS remains a serious neurological emergency requiring prompt recognition and treatment to minimize long-term disability and mortality. The clinical presentation can be variable, and diagnosis is often delayed, with only 25-30% of patients diagnosed on their initial healthcare visit 5.