What is the treatment for Guillain‑Barré syndrome?

Treatment of Guillain-Barré Syndrome

Intravenous immunoglobulin (IVIg) at 0.4 g/kg daily for 5 days is the first-line treatment for Guillain-Barré syndrome, as it is equally effective to plasma exchange but easier to administer, more widely available, and associated with fewer treatment discontinuations. 1

Primary Immunotherapy Options

Both IVIg and plasma exchange are proven equally effective treatments for GBS, but they differ in practical application 1:

IVIg (Preferred First-Line)

• Dose: 0.4 g/kg body weight daily for 5 days (total 2 g/kg) 1
• Advantages: Easier to administer, generally more widely available, and associated with reduced frequency of adverse effects compared to plasma exchange 1
• Timing: Most effective when initiated within 2 weeks of weakness onset 1

Plasma Exchange (Alternative First-Line)

• Protocol: 200-250 ml plasma/kg body weight over 5 sessions, administered on alternate days 1, 2
• Timing: Most effective within 4 weeks of symptom onset, ideally within 7 days 2
• Considerations: Less costly than IVIg but requires specialized equipment and expertise 1

Critical Treatment Principles

What NOT to Do

• Never use corticosteroids alone - eight randomized controlled trials showed no significant benefit, and oral corticosteroids actually had negative effects on outcome 1
• Do not combine plasma exchange followed by IVIg - this is no more effective than either treatment alone 1
• Avoid daily plasma exchange - the alternate-day protocol is standard and allows recovery between sessions 2

Medications to Strictly Avoid

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

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

Special Populations

Children

• Use standard adult IVIg dosing: 0.4 g/kg daily for 5 days 1
• Avoid the 2-day regimen (2 g/kg over 2 days) - one study showed treatment-related fluctuations occurred in 5 of 23 children with the 2-day regimen versus 0 of 23 with the 5-day regimen 1
• IVIg is preferred over plasma exchange due to greater discomfort and higher complication rates with plasma exchange in children 1

Pregnant Women

• Neither IVIg nor plasma exchange is contraindicated during pregnancy 1
• IVIg is preferred as plasma exchange requires additional monitoring considerations 1

Miller Fisher Syndrome (MFS)

• Treatment generally not recommended - patients tend to have mild disease and recover completely within 6 months without treatment 1
• Monitor closely as a subgroup can develop limb weakness, bulbar/facial palsy, or respiratory failure 1

Bickerstaff Brainstem Encephalitis (BBE)

• Disease severity justifies treatment with IVIg or plasma exchange, though evidence is limited 1

ICU Admission Criteria

Admit to ICU immediately if any of the following are present 1:

• Evolving respiratory distress with imminent respiratory insufficiency (vital capacity <15-20 ml/kg or <1 L, breathlessness at rest, inability to count to 15 in single breath, use of accessory respiratory muscles)
• Severe autonomic cardiovascular dysfunction (arrhythmias, marked blood pressure variation)
• Severe swallowing dysfunction or diminished cough reflex
• Rapid progression of weakness

Respiratory Monitoring ("20/30/40 Rule")

Patient is at high risk if 2:

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

Essential Monitoring During Treatment

Cardiovascular

• Monitor for arrhythmias and blood pressure instability due to autonomic dysfunction 2

Line-Related Complications

• Watch for thrombosis and infection related to central venous catheter if plasma exchange is used 2

Volume Shifts

• Particularly important in patients with dysautonomia during plasma exchange 2

Treatment-Related Fluctuations

• Occur in 6-10% of patients within 2 months of initial improvement 2
• Management: Repeat the full course of the original treatment (5 sessions of plasma exchange on alternate days OR 5 days of IVIg at 0.4 g/kg/day) 2

Resource-Limited Settings

Small Volume Plasma Exchange (SVPE)

• Novel, relatively low-cost technique (~$500 vs ~$4,500-5,000 for conventional plasma exchange) 1
• Shown to be safe and feasible in India and Bangladesh 1
• Cannot be recommended for general use until efficacy is established in large-scale trials 1

Emerging Therapies

• Complement inhibitors (eculizumab targeting complement factor 5, and complement factor 3 inhibitors) are under investigation in ongoing trials 1
• Not yet recommended for routine clinical practice 1

Supportive Care Essentials

Deep Vein Thrombosis Prevention

• Use heparin and graduated pressure stockings in bed-bound adult patients 3

Pain Management

• Consider carbamazepine or gabapentin 3
• Cautious use of narcotic analgesics may be needed 3

Rehabilitation

• Disabled patients should receive multidisciplinary rehabilitation with assistive exercise programs 3
• Persistent fatigue is common and may improve with exercise programs 3

Prognosis Considerations

• Overall mortality is 3-10%, primarily from cardiovascular and respiratory complications 2, 4
• Approximately 20% remain unable to walk at 6 months 4
• About 80% regain walking ability at 6 months 2
• The AMAN (acute motor axonal neuropathy) variant typically has worse outcomes than demyelinating forms due to axonal damage 2