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 and should be initiated immediately in patients who cannot walk unaided or show signs of respiratory compromise, dysphagia, or bulbar weakness. 1, 2, 3
First-Line Immunotherapy
IVIg is preferred over plasma exchange despite equal efficacy because it is easier to administer, more widely available, has higher completion rates, and is associated with fewer adverse effects. 1, 3, 4 Both treatments are equally effective in hastening recovery and reducing long-term morbidity when started within 2 weeks of symptom onset. 1, 3
IVIg Dosing Protocol
- Standard regimen: 0.4 g/kg body weight daily for 5 consecutive days (total dose 2 g/kg) 1, 2, 3
- Use the 5-day regimen rather than accelerated 2-day protocols, as treatment-related fluctuations occur more frequently with shorter regimens 1, 2
- Treatment should be initiated as early as possible, preferably within 2 weeks of symptom onset 3, 4
Plasma Exchange Alternative
- Five sessions at 200-250 ml/kg are equally effective as IVIg 5
- For mild GBS: 2 sessions are superior to none 6
- For moderate GBS: 4 sessions are superior to 2 6
- For severe GBS requiring ventilation: 6 sessions are no better than 4 6
- Plasma exchange is beneficial when started within 7 days but remains effective up to 30 days after onset 6
Critical Monitoring Requirements
Respiratory Assessment
Use the "20/30/40 Rule" to assess risk of respiratory failure: 1, 2, 3
- Vital capacity <20 ml/kg
- Maximum inspiratory pressure <30 cmH₂O
- Maximum expiratory pressure <40 cmH₂O
Approximately 20% of GBS patients require mechanical ventilation, necessitating admission to a unit with rapid ICU transfer capability. 3
Additional respiratory monitoring includes single breath count (≤19 predicts need for mechanical ventilation), use of accessory respiratory muscles, and ability to cough. 2
Autonomic Monitoring
Monitor via electrocardiography, heart rate, blood pressure, and bowel/bladder function. 2
Medications to AVOID
The following medications worsen neuromuscular function and must be avoided: 1, 2, 3
- β-blockers
- IV magnesium
- Fluoroquinolones
- Aminoglycosides
- Macrolides
Essential Supportive Care
Pain Management
For neuropathic pain (common in GBS), use: 3
- Gabapentinoids (gabapentin or pregabalin)
- Tricyclic antidepressants
- Duloxetine
- Carbamazepine
Avoid opioids for neuropathic pain. 1
Complication Prevention
- DVT prophylaxis due to immobility 3
- Pressure ulcer prevention 2, 3
- Evaluate for dysphagia and provide nutritional support if necessary 1
- Address constipation/ileus, which is common 1
- Psychological support for anxiety, depression, and hallucinations 2
Management of Treatment Response
Treatment-Related Fluctuations (TRFs)
TRFs occur in 6-10% of patients within 2 months of initial improvement. 1, 2 Repeating the full course of IVIg or plasma exchange is common practice for TRFs, although evidence is limited. 2
Lack of Early Improvement
About 40% of patients do not improve in the first 4 weeks following treatment, which doesn't necessarily indicate treatment ineffectiveness. 1, 2 Continue supportive care and monitoring.
Combination Therapy
Adding IVIg after plasma exchange does not confer significant extra benefit and is not recommended. 3, 4 One trial showed no clinically significant difference (mean grade improvement 0.2,95% CI -0.14 to 0.54). 4
Special Populations
Children
IVIg is preferred over plasma exchange in children due to better tolerability and fewer complications. 1, 3 Use the same 5-day regimen (0.4 g/kg/day) rather than accelerated protocols. 1
Pregnant Women
IVIg is preferred during pregnancy because it requires fewer monitoring considerations and additional precautions, though neither treatment is contraindicated. 1, 3
Miller-Fisher Syndrome
Treatment is generally not recommended as most patients recover completely within 6 months without intervention, though close monitoring is essential. 1
Prognosis
- 80% of patients regain walking ability at 6 months after disease onset 1, 3
- Mortality is 3-10%, most commonly from cardiovascular and respiratory complications 1, 3
- Risk factors for poor outcome include advanced age, severe disease at onset, and lack of ICU support when needed 3
- Recovery can continue for more than 5 years after disease onset 2
- Recurrence is rare (2-5%) but higher than the general population lifetime risk (0.1%) 2
Rehabilitation
A rehabilitation program with a rehabilitation specialist, physiotherapist, and occupational therapist is crucial for recovery. 2 Long-term residual complaints can include neuropathic pain, weakness, and fatigue. 2
Important Caveats
Corticosteroids alone are not recommended for GBS treatment, as randomized controlled trials have shown no significant benefit and oral corticosteroids may have negative effects on outcomes. 1
Verify serum IgA levels before the first IVIg infusion, as IgA deficiency increases the risk of anaphylaxis; use preparations with reduced IgA levels if deficiency is confirmed. 1