What is the primary treatment for a patient with Guillain-Barré Syndrome (GBS)?

Primary Treatment for Guillain-Barré Syndrome

Intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days is the primary treatment for patients with Guillain-Barré Syndrome who cannot walk unaided or have rapidly progressive symptoms. 1, 2, 3

First-Line Treatment Selection

IVIg is preferred over plasma exchange (PE) as first-line therapy despite both treatments being equally effective, because IVIg offers several practical advantages 1, 2, 4:

• Easier administration - no specialized equipment or vascular access required 5, 1
• Higher completion rates - patients are significantly more likely to complete the full IVIg course compared to PE 5, 4
• Wider availability - can be administered in more healthcare settings 5, 1
• Fewer adverse effects - lower frequency of complications compared to PE 5, 6

Treatment Protocol

The standard IVIg regimen consists of 1, 2, 3:

• Dose: 0.4 g/kg body weight daily
• Duration: 5 consecutive days (total dose 2 g/kg)
• Timing: Most effective when started within 2 weeks of symptom onset 2

When to Initiate Treatment

Start IVIg immediately in patients with 1, 3:

• Moderate to severe weakness (GBS disability score ≥3)
• Inability to walk unaided
• Rapidly progressive symptoms
• Any signs of respiratory compromise, dysphagia, facial weakness, or bulbar weakness

Plasma Exchange as Alternative

PE remains an equally effective alternative when 2, 4:

• Dose: 200-250 ml/kg total plasma volume over 5 sessions in 2 weeks 2
• Cost consideration: PE costs ~$4,500-5,000 versus IVIg at ~$12,000-16,000, making it potentially preferable in resource-limited settings 5, 2
• Contraindications to IVIg exist (e.g., IgA deficiency with anti-IgA antibodies) 1

However, PE has practical limitations including need for specialized equipment, central venous access, and higher complication rates 5, 6.

What NOT to Use

Corticosteroids alone are NOT recommended - randomized controlled trials show no significant benefit and oral corticosteroids may worsen outcomes 1, 3. This is a critical pitfall to avoid, as steroids are ineffective despite being beneficial in other autoimmune conditions 7.

Special Populations

Children: Use the same 5-day IVIg regimen (0.4 g/kg/day for 5 days) rather than accelerated 2-day protocols, as treatment-related fluctuations occur more frequently with shorter regimens 1, 3. IVIg is strongly preferred over PE in pediatric patients due to better tolerability and fewer complications 2, 3.

Pregnant women: IVIg is preferred over PE because it requires fewer monitoring considerations, though neither treatment is contraindicated during pregnancy 1, 3.

Mildly affected patients: In children with mild GBS (able to walk), one small trial showed significantly more improvement with IVIg versus supportive care alone (mean difference 1.42 grades, 95% CI 0.27 to 2.57) 4. However, evidence in mildly affected adults is limited 4.

Combined Treatment Approach

Adding IVIg after PE does not provide significant extra benefit - one trial with 249 participants showed only 0.2 grade more improvement (95% CI -0.14 to 0.54) with combined treatment versus PE alone, which is not clinically significant 5, 2.

Managing Treatment Non-Response

Approximately 40% of patients do not improve in the first 4 weeks following treatment 5, 2. This does NOT indicate treatment failure - progression might have been worse without therapy 5. Currently, no evidence supports repeating treatment or switching therapies in these patients, though a clinical trial investigating second-dose IVIg is ongoing 5.

Treatment-Related Fluctuations (TRFs)

TRFs occur in 6-10% of patients within 2 months of initial improvement 5, 2. Repeating the full course of IVIg or PE is common practice for TRFs, as this suggests the treatment effect has worn off while inflammation continues 5, 2.

Critical Medications to AVOID

The following medications worsen neuromuscular function and must be avoided during treatment 1, 2:

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

Essential Monitoring During Treatment

Patients require close monitoring for respiratory failure using the "20/30/40 rule" 5, 1, 3:

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

Any of these parameters indicate high risk for respiratory failure requiring mechanical ventilation (needed in ~20% of GBS patients) 2, 3.

Additionally, monitor rigorously during and after each IVIg infusion for 1:

• Neurological function (motor strength, reflexes, bulbar symptoms)
• Potential adverse reactions to infusion
• Cardiovascular stability and autonomic dysfunction 5