What is the treatment for Guillain-Barré Syndrome?

Treatment of Guillain-Barré Syndrome

Intravenous immunoglobulin (IVIg) at 0.4 g/kg daily for 5 days is the first-line treatment for Guillain-Barré Syndrome due to its ease of administration, wide availability, and favorable safety profile compared to plasma exchange. 1

First-Line Treatment Options

IVIg Therapy

• Dosage: 0.4 g/kg body weight daily for 5 days (total dose 2 g/kg)
• Advantages:
  • Easier to administer than plasma exchange
  • More widely available
  • Associated with fewer adverse effects
  • Less likely to be discontinued due to complications 1
• Timing: Most effective when started within 2 weeks of symptom onset 1

Plasma Exchange (Alternative First-Line)

• Dosage: 200-250 ml plasma/kg body weight in five sessions
• Efficacy: Equal to IVIg in improving recovery speed and reducing disability 1, 2
• Considerations:
  • More technically demanding
  • Requires specialized equipment and expertise
  • Higher discontinuation rates due to complications
  • May be considered when IVIg is contraindicated or unavailable 1

Treatment Algorithm Based on Disease Severity

Mild GBS (able to walk)

• Monitor closely for progression
• Consider 2 sessions of plasma exchange if treatment is needed 3
• For pure Miller Fisher Syndrome variant: treatment generally not recommended, but close monitoring required 1

Moderate GBS (unable to walk independently)

• First choice: IVIg 0.4 g/kg/day for 5 days
• Alternative: Plasma exchange (4 sessions better than 2 for moderate cases) 3

Severe GBS (requiring ventilation or rapidly progressing)

• First choice: IVIg 0.4 g/kg/day for 5 days
• Alternative: Plasma exchange (4 sessions, as 6 sessions show no additional benefit over 4) 3
• Consider ICU admission for monitoring
• Early respiratory assessment using the Erasmus GBS Respiratory Insufficiency Score (EGRIS) to predict need for ventilation 1

Special Patient Populations

Children

• IVIg is preferred first-line therapy (same adult dosing)
• Standard 5-day regimen (0.4 g/kg/day) preferred over accelerated 2-day regimen
• 5-day regimen associated with fewer treatment-related fluctuations (0/23 vs 5/23 with 2-day regimen) 1

Pregnant Women

• Both IVIg and plasma exchange are safe
• IVIg generally preferred due to fewer monitoring requirements 1

GBS Variants

• Miller Fisher Syndrome: Generally no treatment needed, but monitor closely
• Bickerstaff Brainstem Encephalitis: Treatment with IVIg or plasma exchange justified due to severity 1

Important Monitoring Parameters

• Respiratory function (vital capacity, maximum inspiratory/expiratory pressures)
• Use the "20/30/40 rule" for respiratory risk assessment:
  • Vital capacity <20 ml/kg
  • Maximum inspiratory pressure <30 cmH₂O
  • Maximum expiratory pressure <40 cmH₂O 1
• Regular muscle strength assessment using Medical Research Council scale
• Functional disability using GBS disability scale
• Swallowing and coughing difficulties
• Autonomic function (heart rate, blood pressure, bowel/bladder function)

Common Pitfalls to Avoid

1. Corticosteroid use: Despite theoretical benefits, corticosteroids have shown no significant benefit in GBS and oral corticosteroids may worsen outcomes 1

2. Combined treatments: Plasma exchange followed by IVIg shows no additional benefit over either treatment alone 1, 2

3. Delayed treatment: Treatment efficacy decreases when initiated later than 7 days after symptom onset, though benefits may still be seen up to 30 days 3

4. Inadequate respiratory monitoring: Respiratory failure can develop rapidly; regular assessment is critical even in patients without obvious dyspnea 1

5. Missing autonomic dysfunction: Cardiovascular complications from autonomic involvement are a major cause of death in GBS 1

6. Overlooking treatment in specific variants: While pure Miller Fisher Syndrome may not require treatment, other variants like Bickerstaff Brainstem Encephalitis benefit from immunotherapy 1