Treatment of Guillain-Barré Syndrome
The first-line treatment for Guillain-Barré Syndrome (GBS) is intravenous immunoglobulin (IVIG) at a dose of 0.4 g/kg daily for 5 days (total dose 2 g/kg), which should be initiated within 2 weeks of symptom onset. 1 This treatment has been shown to be equally effective as plasma exchange but is preferred due to its ease of administration, higher completion rates, and fewer adverse effects.
Diagnostic Evaluation
Before initiating treatment, a thorough diagnostic workup should include:
- Neurology consultation
- MRI of spine with and without contrast (to rule out compressive lesions)
- Lumbar puncture (CSF typically shows elevated protein)
- Serum antiganglioside antibody tests
- Electrodiagnostic studies to evaluate polyneuropathy 2
Treatment Algorithm
Step 1: Assess Severity and Determine Treatment Setting
- All grades of GBS warrant immediate intervention due to potential for respiratory compromise 2
- Consider ICU admission for:
- Evolving respiratory distress
- Severe autonomic dysfunction
- Swallowing difficulties or diminished cough reflex
- Rapidly progressive weakness 2
Step 2: Initiate First-Line Treatment
- IVIG 0.4 g/kg/day for 5 days (total dose 2 g/kg) 2, 1
- Treatment should be started within 2 weeks of symptom onset for maximum efficacy 1
Step 3: Alternative Treatment Option
- Plasma exchange (200-250 ml plasma/kg in five sessions) if IVIG is unavailable or contraindicated 2, 1
- Note: Plasma exchange has higher discontinuation rates than IVIG 3
Step 4: Monitoring and Supportive Care
- Frequent neurological assessments
- Pulmonary function monitoring (vital capacity, respiratory muscle strength)
- Monitor for autonomic dysfunction
- Provide non-opioid management for neuropathic pain
- Address constipation/ileus 2
Special Considerations
Variant-Specific Approaches
- Miller Fisher Syndrome: Generally does not require treatment due to good spontaneous recovery, but close monitoring is recommended 1
- Bickerstaff Brainstem Encephalitis: Treatment with IVIG or plasma exchange is justified due to disease severity 2, 1
Important Caveats
Avoid corticosteroids: Despite their anti-inflammatory properties, corticosteroids have shown no benefit and may have negative effects in GBS 1, 3
Combination therapy: Using plasma exchange followed by IVIG is no more effective than either treatment alone 1
Pharmacokinetic variability: Patients show significant variation in IgG level increases after standard IVIG treatment, with lower increases associated with poorer outcomes 4, 5
Respiratory assessment: Use the "20/30/40 rule" to assess respiratory function (vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, maximum expiratory pressure <40 cmH₂O) 1
Antimicrobial treatment: Generally not needed as preceding infections have usually resolved before GBS onset, but can be considered if ongoing infection is present 2
Monitoring Response
- Regularly assess muscle strength using the Medical Research Council grading scale
- Monitor functional disability using the GBS disability scale
- Consider the Erasmus GBS Respiratory Insufficiency Score (EGRIS) to predict need for ventilation 1
- Patients with poor response to initial treatment may benefit from additional evaluation, though evidence for second courses of IVIG is limited 5
By following this treatment approach, most patients with GBS can expect significant improvement, though recovery time varies considerably between individuals. Early intervention with appropriate immunotherapy is crucial for optimizing outcomes and reducing long-term morbidity.