What is the latest treatment for Guillain Barre syndrome?

Latest Treatment of Guillain-Barré Syndrome

Intravenous immunoglobulin (IVIG) at 0.4 g/kg/day for 5 consecutive days is the first-line treatment for Guillain-Barré syndrome patients who cannot walk unaided, and should be initiated as early as possible, preferably within 2 weeks of symptom onset. 1, 2

First-Line Immunotherapy

IVIG is preferred over plasma exchange as the primary treatment because it is easier to administer, more widely available, has higher completion rates, and causes fewer adverse effects, despite being more expensive. 1, 3 Both therapies are equally effective in hastening recovery and reducing long-term morbidity, but IVIG's practical advantages make it the treatment of choice in most settings. 2, 4

Standard IVIG Protocol

• Dose: 0.4 g/kg body weight daily for 5 consecutive days (total cumulative dose of 2 g/kg) 1, 3
• Timing: Must be initiated within 2 weeks of symptom onset for optimal efficacy 1, 2
• Indication: GBS disability score ≥3 (unable to walk unaided) 1

Plasma Exchange as Alternative

• Protocol: 200-250 ml/kg total plasma volume, typically divided into 5 sessions over 2 weeks 3
• Cost advantage: Significantly less expensive (~$4,500-5,000 vs $12,000-16,000 for IVIG), which may be relevant in resource-limited settings 5, 3
• Consideration: Small volume plasma exchange (SVPE) is emerging as a novel, low-cost option (~$500) in resource-limited settings like India and Bangladesh, but requires large-scale validation studies before routine implementation 5, 3

Critical Respiratory Monitoring

Approximately 20% of GBS patients require mechanical ventilation, making aggressive respiratory monitoring mandatory. 1, 2 Use the "20/30/40 Rule" to assess risk of respiratory failure requiring intubation: 1, 2, 3

• Vital capacity <20 ml/kg
• Maximum inspiratory pressure <30 cmH₂O
• Maximum expiratory pressure <40 cmH₂O

All patients must be admitted to an inpatient unit with rapid ICU transfer capability. 5

Medications That MUST Be Avoided

The following medications worsen neuromuscular function and are contraindicated in GBS: 1, 2, 3

• β-blockers
• Aminoglycosides
• IV magnesium
• Fluoroquinolones
• Macrolide antibiotics

Essential Supportive Care

Pain Management

Neuropathic pain is common and requires aggressive treatment with: 2, 3

• Gabapentin or pregabalin (first-line)
• Duloxetine
• Tricyclic antidepressants
• Carbamazepine

Additional Supportive Measures

• DVT prophylaxis due to immobility 2, 3
• Pressure ulcer prevention through regular repositioning 3
• Prevention of hospital-acquired infections (pneumonia, UTIs) 3
• Constipation/ileus management 1

Special Populations

Children

IVIG is strongly preferred over plasma exchange in pediatric patients due to better tolerability and fewer complications, using the same dose of 0.4 g/kg/day for 5 days. 1, 2, 3

Pregnant Women

IVIG is preferred over plasma exchange due to fewer monitoring requirements and additional safety considerations. 1, 2

Immune Checkpoint Inhibitor-Related GBS

For patients developing GBS on immune checkpoint inhibitors, the approach differs: 5

• Grade 2 (moderate): Discontinue checkpoint inhibitor, neurology consultation 5
• Grade 3-4 (severe): Permanently discontinue checkpoint inhibitor, admit with ICU capability, initiate IVIG (0.4 g/kg/day for 5 days) or plasmapheresis 5
• Corticosteroids: Consider methylprednisolone 2-4 mg/kg/day or pulse dosing (1 g/day for 5 days) along with IVIG or plasmapheresis, followed by slow taper 5

Note: Corticosteroids are NOT recommended for idiopathic GBS, but may be reasonable in checkpoint inhibitor-related cases. 5

Managing Treatment Non-Response

40% of patients do not improve in the first 4 weeks following treatment—this does NOT indicate treatment failure. 3 Recovery can continue for more than 5 years after disease onset. 3

Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement and may require repeat IVIG courses. 5, 3

Prognosis After Treatment

• 80% of patients regain walking ability at 6 months after disease onset 1, 2, 3
• Mortality is 3-10%, primarily from cardiovascular and respiratory complications 1, 2, 3
• Risk factors for poor outcome: Advanced age, severe disease at onset, and lack of ICU support when needed 1, 2, 3
• Recurrence is rare (2-5%) but higher than general population lifetime risk (0.1%) 3

Common Pitfalls to Avoid

1. Delaying treatment beyond 2 weeks of symptom onset significantly reduces efficacy 1, 2
2. Failing to monitor respiratory function closely can lead to preventable respiratory arrest 1, 2
3. Continuing contraindicated medications (β-blockers, fluoroquinolones, etc.) worsens neuromuscular function 1, 2, 3
4. Misinterpreting lack of improvement at 4 weeks as treatment failure when recovery may take months to years 3
5. Inadequate pain management leads to unnecessary suffering, as neuropathic pain can persist for >10 years 3