What is the recommended treatment for Guillain-Barré Syndrome (GBS)?

Treatment of Guillain-Barré Syndrome (GBS)

Intravenous immunoglobulin (IVIg) at a dose of 0.4 g/kg daily for 5 days is the first-line treatment for Guillain-Barré Syndrome due to its effectiveness, safety profile, and ease of administration. 1

First-Line Treatment Options

IVIg Therapy

• Standard dosing: 0.4 g/kg daily for 5 days (total dose 2 g/kg) 1, 2
• Should be initiated within 2 weeks of symptom onset for optimal effectiveness 3
• Indicated for patients who cannot walk independently (GBS disability score ≥3) 3
• Benefits:
  • Equal efficacy to plasma exchange 1, 4
  • Easier to administer and more widely available 1
  • Higher completion rates compared to plasma exchange 4
  • Fewer adverse effects 1

Plasma Exchange (Alternative First-Line)

• Standard protocol: 200-250 ml plasma/kg in five sessions over 1-2 weeks 1, 3
• Should be initiated within 4 weeks of symptom onset 3
• Indicated when:
  • IVIg is unavailable or contraindicated
  • Cost is a significant factor (less expensive than IVIg) 1
• Limitations:
  • Requires specialized equipment and expertise
  • Higher discontinuation rates than IVIg 1
  • More complications, particularly in children 1

Treatment Considerations for Specific Patient Groups

Children

• IVIg is preferred over plasma exchange due to:
  • Lower complication rates
  • Less discomfort
  • Greater availability 1
• Standard adult dosing regimen (5-day course) is recommended over accelerated 2-day regimen due to fewer treatment-related fluctuations 1

Pregnant Women

• Both IVIg and plasma exchange are safe during pregnancy
• IVIg is generally preferred due to simpler monitoring requirements 1

GBS Variants

• Miller Fisher Syndrome (MFS):
  • Treatment generally not required for pure MFS due to good spontaneous recovery 1
  • Close monitoring recommended as some patients may develop limb weakness or respiratory failure
• Bickerstaff Brainstem Encephalitis (BBE):
  • IVIg or plasma exchange recommended due to disease severity 1

Monitoring and Supportive Care

Respiratory Function Monitoring

• Regular assessment using the "20/30/40 rule" 1:
  • Vital capacity <20 ml/kg
  • Maximum inspiratory pressure <30 cmH₂O
  • Maximum expiratory pressure <40 cmH₂O
• Consider ICU admission for:
  • Evolving respiratory distress
  • Severe autonomic dysfunction
  • Severe swallowing dysfunction
  • Rapidly progressive weakness 1
• Use the Erasmus GBS Respiratory Insufficiency Score (EGRIS) to predict need for ventilation 1

Muscle Strength and Disability Assessment

• Regular assessment using Medical Research Council grading scale
• Monitor functional disability using GBS disability scale 1

Important Caveats and Pitfalls

1. Avoid corticosteroids: Despite their anti-inflammatory properties, corticosteroids have shown no benefit and may even have negative effects in GBS 1, 3

2. Avoid combination therapy: Plasma exchange followed by IVIg is no more effective than either treatment alone 1, 4

3. Watch for treatment-related fluctuations: About 10% of patients experience secondary deterioration within 8 weeks after starting IVIg, requiring repeated treatment 5

4. Consider CIDP misdiagnosis: Approximately 5% of patients initially diagnosed with GBS actually have acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) if progression continues beyond 8 weeks 3, 5

5. Resource limitations: In low and middle-income countries, small-volume plasma exchange may be considered as a more economical alternative, though further research is needed to establish efficacy 1

6. Antimicrobial treatment: Generally not needed as preceding infections have usually resolved before GBS onset, but can be considered if ongoing infection is present 1

By following these evidence-based treatment guidelines and monitoring protocols, the morbidity and mortality associated with Guillain-Barré Syndrome can be significantly reduced.