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

Treatment of Guillain-Barré Syndrome

Intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) is the first-line treatment for GBS and should be initiated immediately in patients unable to walk independently or showing signs of respiratory compromise, bulbar weakness, or rapid progression. 1, 2

Why IVIg is Preferred Over Plasma Exchange

IVIg is the preferred first-line therapy because it is equally effective as plasma exchange but offers significant practical advantages:

• Easier administration with higher completion rates compared to plasma exchange 1
• More widely available in most hospital settings 1
• Better tolerability with fewer procedural complications, particularly in children 1
• Fewer monitoring requirements, making it especially preferred in pregnant women 1

The evidence shows no significant difference in disability improvement between IVIg and plasma exchange (mean difference of 0.02 grade on a 7-point scale, 95% CI -0.20 to 0.25), but IVIg is significantly more likely to be completed 3, 4

Treatment Initiation Criteria

Start IVIg immediately if the patient has: 1, 2

• Inability to walk independently (functional grade ≥3)
• Moderate to severe weakness with rapid progression
• Any signs of respiratory compromise
• Dysphagia or bulbar weakness
• Facial weakness

Treatment should be initiated as early as possible in the disease course to maximize effectiveness, ideally within 2 weeks of symptom onset 1, 4

Alternative Treatment Option

Plasma exchange (12-15 L over 4-5 sessions in 1-2 weeks) is an equivalent alternative if IVIg is contraindicated, unavailable, or shows no benefit 2, 4. However, it requires more intensive monitoring and has lower completion rates 3

What NOT to Do

Corticosteroids alone are NOT recommended for idiopathic GBS, as randomized controlled trials show no significant benefit and oral corticosteroids may worsen outcomes 1, 2, 4

Do NOT combine plasma exchange followed immediately by IVIg as this provides no additional benefit over either treatment alone 1, 4

Avoid medications that worsen neuromuscular function: 1, 2

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

Critical Monitoring Requirements

Admit patients to a unit with rapid ICU transfer capability for close respiratory and autonomic monitoring 1, 2

Assess respiratory failure risk using the "20/30/40 rule": 1, 2

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

Any of these parameters indicate high risk for mechanical ventilation 2

Monitor continuously for: 2

• Neurological progression (motor strength, reflexes, bulbar symptoms)
• Autonomic dysfunction (arrhythmias, blood pressure fluctuations)
• Respiratory function (vital capacity, inspiratory/expiratory pressures)

Special Populations

Children: Use the standard 5-day IVIg regimen (0.4 g/kg/day × 5 days) rather than accelerated 2-day protocols, as treatment-related fluctuations occur more frequently with shorter regimens 1

Pregnant women: IVIg is preferred over plasma exchange due to fewer monitoring requirements, though neither is contraindicated 1

Miller-Fisher Syndrome: Treatment is generally not recommended as most patients recover completely within 6 months without intervention, though close monitoring remains essential 1

Immune checkpoint inhibitor-related GBS: Discontinue the causative agent permanently and consider concurrent corticosteroids (methylprednisolone 2-4 mg/kg/day) with IVIg 1, 2

Managing Inadequate Response

Approximately 40% of patients do not improve in the first 4 weeks following treatment, which does not necessarily indicate treatment failure 1, 2

Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement 1, 2. For TRFs, repeat the full course of IVIg or plasma exchange 1, 2

Consider chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) if the patient experiences three or more TRFs or clinical deterioration ≥8 weeks after onset 5

Essential Supportive Care

Pain management (neuropathic pain is common and impacts quality of life): 2, 5

• First-line: Gabapentin, pregabalin, or duloxetine
• Avoid opioids

Prevent complications: 2, 5

• Deep vein thrombosis prophylaxis
• Pressure ulcer prevention
• Assess for dysphagia and provide nutritional support
• Address constipation/ileus

Verify serum IgA levels before first IVIg infusion, as IgA deficiency increases anaphylaxis risk; use IgA-reduced preparations if deficiency is confirmed 1

Prognosis

Approximately 80% of patients regain independent walking ability at 6 months after disease onset 1, 2

Mortality occurs in 3-10% of cases, primarily from cardiovascular and respiratory complications, with risk factors including advanced age and severe disease at onset 1, 2. Notably, up to two-thirds of deaths occur during the recovery phase, requiring continued vigilance even after apparent improvement 2