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

Treatment of Guillain-Barré Syndrome

Intravenous immunoglobulin (IVIg) at 0.4 g/kg daily for 5 consecutive days is the first-line treatment for GBS and should be initiated as early as possible in patients with significant disability. 1, 2

First-Line Immunotherapy

IVIg is preferred over plasma exchange (PE) as first-line therapy because it is easier to administer, more widely available, has higher completion rates, and is associated with fewer adverse effects. 1, 2 Both treatments are equally effective in improving disability outcomes, but IVIg's practical advantages make it the preferred choice. 3, 4

IVIg Protocol

• Dose: 0.4 g/kg body weight daily for 5 consecutive days 1, 5, 2
• Timing: Most effective when started within 2 weeks of symptom onset 6, 2
• Patient selection: Initiate in patients with GBS disability score ≥3 (unable to walk independently) 6, 2
• Duration preference: The 5-day regimen is preferred over shorter 2-day regimens due to higher rates of treatment-related fluctuations with abbreviated courses 5

Plasma Exchange as Alternative

• Dose: 200-250 mL/kg total plasma volume, typically divided into 5 sessions over 2 weeks 6, 2
• Efficacy: Equally effective as IVIg with mean disability improvement difference of only 0.02 grades (95% CI -0.20 to 0.25) 3
• Cost consideration: Significantly less expensive than IVIg (~$4,500-5,000 vs $12,000-16,000), which may be relevant in resource-limited settings 6, 2
• Completion rates: Lower than IVIg, making it less reliable in practice 3

Critical Respiratory Monitoring

Use the "20/30/40 Rule" to identify patients at high risk for respiratory failure requiring mechanical ventilation: 1, 5, 2

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

Approximately 20% of GBS patients will require mechanical ventilation, making close respiratory monitoring essential. 5, 2 Additional warning signs include single breath count ≤19, use of accessory respiratory muscles, and inability to cough effectively. 5

Medications to AVOID

The following medications can worsen neuromuscular function and must be avoided in GBS patients: 1, 2

• β-blockers
• Intravenous magnesium
• Fluoroquinolones
• Aminoglycosides
• Macrolides

Management of Treatment Non-Response

Approximately 40% of patients do not improve in the first 4 weeks following treatment—this does NOT necessarily indicate treatment failure. 1, 5, 2 Recovery can continue for more than 5 years after disease onset. 5, 2

Treatment-Related Fluctuations (TRFs)

• Occur in 6-10% of patients within 2 months of initial improvement 1, 5, 2
• Management: Repeating the full course of IVIg or PE is common practice, though evidence supporting this approach is limited 5

Combined Therapy Considerations

Adding IVIg after PE does not confer significant extra benefit (mean grade improvement difference of only 0.2,95% CI -0.14 to 0.54). 2, 4 The combination should not be routinely used based on current evidence. 3, 4

However, one novel approach called the "zipper method" (alternating PE sessions with IVIg infusions for 5 cycles) showed promising results in severe pediatric cases requiring mechanical ventilation, with mean ventilation duration of only 7 days and all patients walking unaided by day 28. 7 This remains investigational and requires larger studies.

Essential Supportive Care

Multidisciplinary supportive care is equally important as immunotherapy: 5

Pain Management

• Neuropathic pain is common and requires aggressive treatment with neuropathic pain medications 5, 2

Complication Prevention

• DVT prophylaxis due to immobility 2
• Pressure ulcer prevention through regular repositioning 5, 2
• Hospital-acquired infection prevention (pneumonia, urinary tract infections) 5, 2

Autonomic Monitoring

• Monitor via electrocardiography, heart rate, blood pressure, and bowel/bladder function 5
• Address constipation/ileus, which is common in GBS patients 1

Psychological Support

• Provide support for anxiety, depression, and hallucinations, which are frequent in GBS patients 5

Special Populations

Children

In pediatric patients, IVIg is strongly preferred over PE (0.4 g/kg daily for 5 days) due to better tolerability and fewer complications. 1, 2 Low-quality evidence suggests IVIg hastens recovery compared with supportive care alone in children. 3

Pregnant Women

Both IVIg and PE are not contraindicated in pregnancy, but IVIg is generally preferred due to fewer monitoring requirements. 1

Immune Checkpoint Inhibitor-Related GBS

Discontinue the causative agent permanently and consider concurrent corticosteroids with IVIg or PE. 1

Resource-Limited Settings

Small volume plasma exchange (SVPE) is a novel, low-cost option (~$500) that has shown safety and feasibility in India and Bangladesh, but requires large-scale studies before routine implementation. 6, 2

What NOT to Use

Corticosteroids alone are not recommended for GBS treatment, as randomized controlled trials have shown no significant benefit and oral corticosteroids may even have negative effects on outcomes. 1

Prognosis

• 80% of patients regain walking ability at 6 months after disease onset 1, 5, 2
• Mortality is 3-10%, most commonly from cardiovascular and respiratory complications 1, 2
• Risk factors for mortality include advanced age, severe disease at onset, and lack of ICU support when needed 2
• Recurrence is rare (2-5%) but higher than general population lifetime risk (0.1%) 5, 2
• Long-term residual complaints include neuropathic pain, weakness, and fatigue 5, 2