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 days is the first-line treatment for GBS and should be initiated as early as possible in patients with significant disability (GBS disability score ≥3). 1

First-Line Immunotherapy Options

Both IVIg and plasma exchange (PE) are equally effective treatments for GBS, but IVIg is generally preferred as first-line therapy due to easier administration, wider availability, higher completion rates, and fewer adverse effects compared to PE. 2, 1

IVIg Protocol

• Dose: 0.4 g/kg body weight daily for 5 consecutive days 1, 3
• Timing: Most effective when started within 2 weeks of symptom onset 2
• Completion rates: Significantly higher than PE, making it more practical in clinical practice 4

Plasma Exchange Protocol

• Dose: 200-250 ml/kg total plasma volume, typically divided into 5 sessions over 2 weeks 2
• Timing: Can be effective up to 4 weeks after onset, though benefit decreases after 7 days 5, 6
• Number of sessions: 2 sessions for mild GBS, 4 sessions for moderate GBS, and 4-6 sessions for severe GBS (6 sessions show no additional benefit over 4) 5
• Cost consideration: PE is less expensive than IVIg (~$4,500-5,000 vs $12,000-16,000), which may be relevant in resource-limited settings 2

Treatment Selection Algorithm

1. For most patients: Start with IVIg due to ease of administration and safety profile 1, 4
2. Consider PE when: IVIg is contraindicated, not tolerated, unavailable, or cost is prohibitive 2, 7
3. In children: Strongly prefer IVIg over PE due to better tolerability and fewer complications 1, 7
4. In pregnant women: Both treatments are safe, but IVIg is preferred due to fewer monitoring requirements 1

What NOT to Do

Corticosteroids alone are NOT recommended for GBS treatment, as randomized controlled trials have shown no significant benefit and oral corticosteroids may even worsen outcomes. 1, 6

Sequential treatment with PE followed by IVIg is NOT recommended, as one trial with 249 participants showed no clinically significant additional benefit (mean improvement difference of only 0.2 grades). 2, 6

Critical Monitoring Requirements

Respiratory Assessment (The "20/30/40 Rule")

Patient is at high risk for respiratory failure requiring mechanical ventilation if: 1, 3

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

Additional Respiratory Monitoring

• Single breath count (≤19 predicts need for mechanical ventilation) 3
• Use of accessory respiratory muscles 1
• Ability to cough and swallow (to prevent aspiration) 7
• Approximately 20% of GBS patients will require mechanical ventilation 3

ICU Admission Criteria

Admit to ICU or unit with rapid ICU transfer capability for: 2, 1

• Imminent respiratory insufficiency
• Severe autonomic dysfunction with cardiovascular instability
• Severe swallowing dysfunction and/or diminished cough reflex
• Rapidly progressive weakness

Autonomic Monitoring

• Continuous electrocardiography 1
• Frequent heart rate and blood pressure monitoring 1
• Bowel and bladder function assessment 1

Medications to AVOID

The following medications can worsen neuromuscular function and must be avoided: 1, 3

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

Management of Treatment Non-Response and Fluctuations

Expected Timeline

• 40% of patients do not improve in the first 4 weeks following treatment—this does NOT necessarily indicate treatment failure 1, 3
• Recovery can continue for more than 5 years after disease onset 3, 7

Treatment-Related Fluctuations (TRFs)

• Occur in 6-10% of patients within 2 months of initial improvement 1, 3
• Management: Repeat the full course of IVIg or PE (though evidence supporting this is limited) 3, 7
• If repeated relapses occur (~5% of cases), consider diagnosis change to acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) 7

Essential Supportive Care

Multidisciplinary Management Required

• Pain management: Pain is common in GBS and requires aggressive treatment with neuropathic pain medications 3, 7
• DVT prophylaxis: Essential due to immobility 1, 7
• Prevention of pressure ulcers through regular repositioning 1, 7
• Prevention of hospital-acquired infections (pneumonia, UTIs) 3, 7
• Psychological support: Anxiety, depression, and hallucinations are frequent 1, 7
• Bowel management: Address constipation/ileus which is common 1

Rehabilitation

A structured rehabilitation program is crucial and should include: 7

• Rehabilitation specialist, physiotherapist, and occupational therapist involvement
• Range-of-motion exercises
• Stationary cycling and walking as tolerated
• Strength training
• Caution: Monitor exercise intensity closely as overwork can cause significant fatigue 7

Special Populations and Considerations

Children

• IVIg is strongly preferred over PE (0.4 g/kg daily for 5 days) 7
• Low-quality evidence suggests IVIg hastens recovery compared to supportive care alone in children 4
• Treatment options are the same as adults, but tolerability favors IVIg 7, 6

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 2
• Only 10-12% of patients in some low-income countries receive standard treatment due to cost barriers 2

Immune Checkpoint Inhibitor-Related GBS

• Permanently discontinue the causative agent 1
• Consider concurrent corticosteroids WITH IVIg or PE (this is an exception to the no-corticosteroids rule) 1

Prognosis

• 80% of patients regain walking ability at 6 months after disease onset 1, 3
• Mortality: 3-10%, most commonly from cardiovascular and respiratory complications 1, 3
• Risk factors for mortality: Advanced age, severe disease at onset, and lack of ICU support when needed 2, 1
• Recurrence is rare (2-5%) but higher than general population lifetime risk (0.1%) 3
• Long-term residual complaints include neuropathic pain, weakness, and fatigue 3, 7