Treatment of Guillain-Barré Syndrome
Intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days is the first-line treatment for GBS patients who cannot walk unaided, and should be initiated as early as possible, preferably within 2 weeks of symptom onset. 1, 2
Why IVIg is Preferred Over Plasma Exchange
While IVIg and plasma exchange are equally effective in hastening recovery and reducing long-term morbidity, IVIg is strongly preferred as first-line therapy for several critical reasons 1, 2:
- Easier administration - no need for specialized equipment or vascular access 1
- Higher completion rates - patients are significantly more likely to complete the full IVIg course compared to plasma exchange 3
- Wider availability - particularly important in resource-limited settings 4, 1
- Fewer adverse effects - reduced frequency of complications compared to plasma exchange 4, 5
Critical Monitoring Requirements
Approximately 20% of GBS patients require mechanical ventilation, making respiratory monitoring essential 2. Use the "20/30/40 Rule" to assess risk of respiratory failure 1, 2:
- Vital capacity <20 ml/kg = high risk 1
- Maximum inspiratory pressure <30 cmH₂O = high risk 1
- Maximum expiratory pressure <40 cmH₂O = high risk 1
The modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) can calculate probability of requiring ventilation 2. Monitor swallowing and coughing difficulties to prevent aspiration 6.
Medications to AVOID
The following medications worsen neuromuscular function and must be avoided 2:
Special Populations
Children
IVIg is the preferred treatment in pediatric GBS due to better tolerability and fewer complications compared to plasma exchange 2, 6. Use the same 5-day 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.
Pregnant Women
IVIg is preferred during pregnancy because it requires fewer monitoring considerations and additional precautions, though neither IVIg nor plasma exchange are contraindicated 1, 2.
Managing Treatment Response
About 40% of patients do not improve in the first 4 weeks following treatment - this does not necessarily indicate treatment ineffectiveness, as progression might have been worse without therapy 4, 6.
Treatment-Related Fluctuations (TRFs)
TRFs occur in 6-10% of patients within 2 months of initial improvement 4, 2. Repeating the full course of IVIg or plasma exchange is common practice for TRFs, though evidence supporting this approach is limited 4, 6.
When NOT to Combine Treatments
Adding IVIg after plasma exchange does not confer significant extra benefit 3. One trial with 249 participants showed the mean grade improvement was only 0.2 grades better (95% CI -0.14 to 0.54) with combined treatment - not clinically significant 3.
Subtype-Specific Considerations
IVIg efficacy varies by GBS subtype 7:
- AIDP (Acute Inflammatory Demyelinating Polyradiculoneuropathy): IVIg results in significantly less poor recovery at 6 months (0.8% vs. 6.6%, p = 0.03) 7
- AMAN (Acute Motor Axonal Neuropathy): IVIg therapy did not significantly alter outcomes compared to natural course 7
This is particularly relevant in low- and middle-income countries where AMAN and AMSAN subtypes are more prevalent 4.
Essential Supportive Care
Neuropathic pain management is critical and should include 2:
DVT prophylaxis is mandatory due to immobility 2. Multidisciplinary care should include physiotherapists, rehabilitation specialists, occupational therapists, speech therapists, and dietitians 4.
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, 2.
Prognosis and Risk Factors
About 80% of patients regain walking ability at 6 months after disease onset 4, 2. However, mortality occurs in 3-10% of cases, most commonly from cardiovascular and respiratory complications 4, 2.
Risk factors for poor outcome include 4, 2:
Common Pitfall: IgA Deficiency
Verify serum IgA levels before the first IVIg infusion, as IgA deficiency increases the risk of anaphylaxis 1. Use IVIg preparations with reduced IgA levels if deficiency is confirmed 1.