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

Treatment of Guillain-Barré Syndrome (GBS)

Intravenous immunoglobulin (IVIG) at 0.4 g/kg/day for 5 consecutive days is the recommended first-line treatment for patients with Guillain-Barré Syndrome who are unable to walk unaided and are within 2 weeks of symptom onset. 1

First-Line Treatment Options

IVIG Therapy

• Recommended dose: 0.4 g/kg/day for 5 consecutive days 1, 2
• Should be initiated within 2 weeks of symptom onset (can be considered up to 4 weeks) 2
• Indications: Patients unable to walk unaided 1
• Advantages: Greater availability, lower complication rates, and higher completion rates compared to plasma exchange 1, 3

Plasma Exchange (PE)

• Alternative first-line therapy when IVIG is unavailable or contraindicated 1
• Dosing: 12-15 L in 4-5 exchanges over 1-2 weeks 2
• Should be initiated within 4 weeks of symptom onset 2
• Equally effective as IVIG but has higher complication rates and requires specialized equipment 1, 4
• Higher risk of treatment discontinuation compared to IVIG 4

Special Populations

Children

• IVIG is preferred over plasma exchange due to lower complication rates 1
• Standard 5-day IVIG regimen is preferred over accelerated 2-day regimen (which is associated with more treatment-related fluctuations) 1

Pregnant Women

• IVIG is preferred over plasma exchange, though neither is contraindicated 1
• Careful monitoring of both mother and fetus is essential 1

Treatment Combinations and Contraindications

• Combination therapy (plasma exchange followed by IVIG) is not recommended as it shows no additional benefit 1, 2, 3
• Corticosteroids (oral or IV) are not recommended as they show no benefit and may have negative effects 1, 2

Management of Complications

Pain Management

• First-line: Gabapentinoids (pregabalin, gabapentin) 1, 2
• Alternatives: Tricyclic antidepressants or carbamazepine 1, 2
• Avoid opioids when possible 1

Respiratory Care

• Apply the "20/30/40 rule" for respiratory monitoring 1:
  • Vital capacity < 20 ml/kg
  • Maximum inspiratory pressure < 30 cmH₂O
  • Maximum expiratory pressure < 40 cmH₂O
• Single breath count ≤ 19 predicts need for mechanical ventilation 1
• Use the Erasmus GBS Respiratory Insufficiency Score (EGRIS) to identify patients at risk of requiring mechanical ventilation 1

Monitoring and Follow-up

• Regular assessment of muscle strength using Medical Research Council grading scale 1
• Monitor functional disability using GBS disability scale 1
• Watch for treatment-related fluctuations (TRFs) which occur in 6-10% of cases within 2 months of treatment 1
• Be alert for progression beyond 8 weeks, which may indicate acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) in approximately 5% of patients 1, 5
• Use the modified Erasmus GBS outcome score (mEGOS) to predict recovery of walking ability 1, 2

Prognosis

• Approximately 80% of patients regain independent walking by 6 months 1
• Recovery can continue for more than 3 years after onset 1
• Mortality rate is 3-10% despite optimal care 1
• Recurrence is rare (2-5% of cases) 1
• Long-term residual complaints such as pain, weakness, and fatigue are common 1

Important Considerations

• Early treatment is crucial for better outcomes
• Despite current treatments, GBS remains a severe disease with significant morbidity
• A comprehensive rehabilitation program should be arranged before discharge to address potential long-term issues 1
• Second courses of IVIG are not recommended for patients with poor prognosis 2