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

Guillain-Barré Syndrome: Treatment and Prognosis

First-Line Treatment

Intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days is the preferred first-line treatment for GBS patients unable to walk unaided (GBS disability score ≥3), and should be initiated within 2 weeks of symptom onset. 1, 2

Treatment Selection Algorithm

• IVIg is preferred over plasma exchange due to easier administration, wider availability, higher completion rates, and fewer adverse effects, despite higher cost 3, 1, 2
• Plasma exchange (PE) is equally effective when IVIg is unavailable or contraindicated: 200-250 ml/kg total plasma volume divided into 5 sessions over 2 weeks 3, 1
• Both treatments work best when started early: IVIg within 2 weeks, PE within 4 weeks of weakness onset 3, 4
• Do NOT combine PE followed immediately by IVIg - this approach is not recommended 4

Cost Considerations in Resource-Limited Settings

• IVIg costs approximately $12,000-16,000 vs PE at $4,500-5,000 3
• Small volume plasma exchange (SVPE) is emerging as a low-cost option (~$500) in India and Bangladesh, but requires large-scale validation before routine use 3

Critical Respiratory Monitoring

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

Medications That MUST Be Avoided

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

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

Managing Treatment Non-Response

40% of patients show no improvement in the first 4 weeks after treatment - this does NOT indicate treatment failure. 1, 6

• Recovery can continue for more than 5 years after disease onset 1, 6
• Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement 1, 5
• For TRFs, repeat the full course of IVIg or PE, though evidence supporting this is limited 6, 5
• Consider changing diagnosis to acute-onset CIDP (A-CIDP) if progression continues beyond 8 weeks - this occurs in ~5% of initially diagnosed GBS patients 5, 4

Essential Supportive Care

Multidisciplinary supportive care is mandatory and includes: 1, 2

• Pain management: Use gabapentinoids (gabapentin, pregabalin), tricyclic antidepressants, or carbamazepine for neuropathic pain 1, 4
• DVT prophylaxis: Essential due to immobility 1, 2
• Pressure ulcer prevention: Regular repositioning 1, 2
• Prevention of hospital-acquired infections: Pneumonia and UTIs 1, 2
• Psychological support: For anxiety, depression, and hallucinations 6

Special Populations

Children

• IVIg is strongly preferred over PE in pediatric patients (0.4 g/kg/day × 5 days) due to better tolerability and fewer complications 2, 7

Pregnant Women

• IVIg is preferred over PE due to fewer monitoring requirements 2

Pure Motor Variants (AMAN)

• Same treatment approach with IVIg or PE applies to pure motor variants 6
• These variants may be more prevalent in Asia and low-middle income countries 3

Prognosis

Short-Term Outcomes

• 80% of patients regain walking ability at 6 months after disease onset 1, 5
• Mortality is 3-10%, most commonly from cardiovascular and respiratory complications 1, 5

Risk Factors for Poor Outcome

• Advanced age 1, 2
• Severe disease at onset 1, 2
• Lack of ICU support when needed 1, 2

Long-Term Sequelae

• About 25% of patients require artificial ventilation during the acute phase 5
• 20% remain unable to walk at 6 months 5
• Long-term residual complaints include neuropathic pain, weakness, and fatigue 1, 6
• Recurrence is rare (2-5%) but higher than general population lifetime risk (0.1%) 1

Prognostic Tools

• Use the modified Erasmus GBS Outcome Score (mEGOS) to assess functional outcome 4
• Use the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess risk of requiring artificial ventilation 4

Common Pitfalls to Avoid

• Do not withhold treatment in patients who present later than 2 weeks - treatment may still be beneficial up to 4 weeks for PE 3, 4
• Do not use corticosteroids alone - they are ineffective in GBS 5, 4
• Do not give a second IVIg course to patients with poor prognosis as routine practice - this is not recommended based on current evidence 4
• Do not misdiagnose A-CIDP as treatment-resistant GBS - consider A-CIDP if progression continues beyond 8 weeks 5, 4