Immediate Treatment for Guillain-Barré Syndrome (GBS)
Intravenous immunoglobulin (IVIg) at a dose of 0.4 g/kg body weight daily for 5 days is the preferred first-line treatment for patients diagnosed with Guillain-Barré Syndrome and should be initiated as early as possible in the disease course. 1, 2, 3
First-Line Treatment Options
- IVIg (0.4 g/kg body weight daily for 5 days) is recommended as the preferred first-line therapy due to easier administration, better tolerability, and higher completion rates compared to plasma exchange 2, 3
- Plasma exchange (200-250 ml plasma/kg body weight in five sessions over 2 weeks) is an effective alternative when IVIg is contraindicated, not tolerated, or unavailable 1, 2
- Treatment should be initiated as early as possible to maximize effectiveness, ideally within the first two weeks of symptom onset 2, 4
- Corticosteroids alone are not recommended for GBS treatment as they have shown no significant benefit and may even have negative effects on outcomes 1, 2
Immediate Patient Assessment and Monitoring
- All patients with suspected GBS require immediate neurological consultation and admission to an inpatient unit with capability for rapid transfer to intensive care 3
- Close monitoring for respiratory failure is essential using the "20/30/40 rule": patient at risk if vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 2
- Regular assessment of respiratory function should include vital capacity, maximum inspiratory/expiratory pressures, single breath count, and monitoring for use of accessory respiratory muscles 1, 3
- Monitor for swallowing and coughing difficulties to prevent aspiration 1
Management of Complications
- Multidisciplinary supportive care is crucial and should include pain management, as pain is common in GBS patients 1
- Implement preventive measures for pressure ulcers, hospital-acquired infections, and deep vein thrombosis 1, 3
- Monitor for autonomic dysfunction through regular assessment of heart rate, blood pressure, and bowel/bladder function 1, 3
- Provide psychological support for anxiety, depression, and hallucinations which are frequent in GBS patients 1
- Avoid medications that can worsen neuromuscular function, such as β-blockers, IV magnesium, fluoroquinolones, aminoglycosides, and macrolides 2
Management of Disease Progression
- About 40% of patients do not show improvement in the first 4 weeks following treatment, which doesn't necessarily indicate treatment ineffectiveness 1, 2
- Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement 1, 3
- For patients with TRFs, repeating the full course of IVIg or switching to plasma exchange is common practice, although evidence supporting this approach is limited 1, 2
- In approximately 5% of cases initially diagnosed as GBS, the diagnosis may change to acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) if repeated relapses occur or progression continues after 8 weeks from onset 1, 4
Special Patient Considerations
- In children, IVIg is preferred over plasma exchange due to better tolerability and fewer complications 1, 2
- In pregnant women, both IVIg and plasma exchange are not contraindicated, but IVIg is generally preferred due to fewer monitoring requirements 2
- For immune checkpoint inhibitor-related GBS, discontinuing the causative agent permanently and considering concurrent corticosteroids with IVIg or plasma exchange is recommended 2
Prognosis
- About 80% of patients regain walking ability at 6 months after disease onset 2, 3
- Mortality occurs in 3-10% of cases, most commonly due to cardiovascular and respiratory complications 2, 3
- Long-term residual complaints can include neuropathic pain, weakness, and fatigue, but recovery may still occur >5 years after disease onset 1, 3