What is the treatment for Guillain-Barré Syndrome?

Treatment of Guillain-Barré Syndrome

Intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days is the first-line treatment for Guillain-Barré syndrome in patients who cannot walk independently or show any signs of respiratory compromise, dysphagia, facial weakness, or bulbar weakness. 1, 2, 3

First-Line Immunotherapy

IVIg is strongly preferred over plasma exchange as the initial treatment because it is easier to administer, more widely available, has higher completion rates (fewer patients discontinue treatment), and causes fewer adverse effects. 1, 2, 3 Both treatments are equally effective in terms of clinical outcomes, but the practical advantages of IVIg make it the superior choice in most clinical settings. 4, 5

IVIg Dosing Protocol

• Dose: 0.4 g/kg body weight daily for 5 consecutive days (total dose 2 g/kg) 1, 2, 3
• Timing: Most effective when started within 2 weeks of symptom onset, though benefit persists up to 30 days 3, 6
• Pediatric dosing: Use the same 5-day regimen in children; avoid accelerated 2-day protocols due to higher rates of treatment-related fluctuations 2

Plasma Exchange as Alternative

• Dose: 200-250 ml/kg total plasma volume divided into 5 sessions over 2 weeks 3
• Cost consideration: Significantly less expensive than IVIg (~$4,500-5,000 vs $12,000-16,000), which may be relevant in resource-limited settings 7, 3
• Evidence: Two plasma exchanges are superior to none in mild GBS; four sessions are superior to two in moderate GBS; six sessions are no better than four in severe GBS requiring ventilation 6

Critical Monitoring Requirements

All patients with GBS require admission to a hospital unit with rapid ICU transfer capability because approximately 20% will require mechanical ventilation, and respiratory failure can develop suddenly. 1, 3

The "20/30/40 Rule" for Respiratory Assessment

A patient is at high risk for respiratory failure requiring mechanical ventilation if any of the following are present: 1, 3

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

Essential Monitoring Parameters

• Frequent neurochecks to assess progression of weakness, reflexes, and bulbar symptoms 7
• Pulmonary function testing including vital capacity and inspiratory/expiratory pressures 7, 2
• Autonomic dysfunction monitoring for cardiac arrhythmias and blood pressure instability 2

Medications to AVOID

The following medications worsen neuromuscular function and must be strictly avoided in all GBS patients: 7, 1, 2

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

Management of Treatment Non-Response and Fluctuations

40% of patients do not improve in the first 4 weeks following treatment—this does NOT indicate treatment failure. 2, 3 Recovery can continue for more than 5 years after disease onset. 1, 3

Treatment-Related Fluctuations (TRFs)

• Occur in 6-10% of patients within 2 months of initial improvement 2, 3
• Management: Repeat the full course of IVIg (0.4 g/kg/day for 5 days) or plasma exchange 7, 2

Essential Supportive Care

Pain Management

Neuropathic pain is common and requires aggressive treatment with: 1

• Gabapentinoids (gabapentin or pregabalin)
• Tricyclic antidepressants
• Duloxetine
• Carbamazepine

Avoid opioids for neuropathic pain management. 1

Additional Supportive Measures

• DVT prophylaxis due to immobility 3
• Pressure ulcer prevention through regular repositioning 3
• Nutritional support with dysphagia evaluation if bulbar weakness present 2
• Prevention of hospital-acquired infections (pneumonia, UTIs) 3

Diagnostic Work-Up

Before initiating treatment, obtain: 7

• Neurology consultation (mandatory)
• MRI of spine with or without contrast to rule out compressive lesions and evaluate for nerve root enhancement
• Lumbar puncture: CSF typically shows elevated protein; in cancer patients, also send cytology
• Serum antiganglioside antibody tests (e.g., anti-GQ1b for Miller Fisher variant)
• Electrodiagnostic studies to evaluate polyneuropathy and exclude other diagnoses
• Baseline pulmonary function testing

Special Populations

Immune Checkpoint Inhibitor-Related GBS

For patients developing GBS while on immune checkpoint inhibitors: 7

• Permanently discontinue the checkpoint inhibitor for Grade 3-4 severity
• Add corticosteroids (methylprednisolone 2-4 mg/kg/day) along with IVIg or plasma exchange—this differs from idiopathic GBS where corticosteroids alone are not beneficial 7, 8
• Consider pulse dosing: methylprednisolone 1 g/day for 5 days 7

Pregnant Women

• IVIg is preferred over plasma exchange due to fewer monitoring requirements, though neither is contraindicated 2

Children

• IVIg is strongly preferred over plasma exchange due to better tolerability and fewer complications 2, 3

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 mortality: Advanced age, severe disease at onset, lack of ICU support when needed 3
• Recurrence is rare (2-5%) but higher than general population lifetime risk (0.1%) 3
• Long-term residual complaints include neuropathic pain, weakness, and fatigue 3

Rehabilitation

All GBS patients require a comprehensive rehabilitation program involving a rehabilitation specialist, physiotherapist, and occupational therapist to optimize functional recovery. 1

Common Pitfalls to Avoid

• Do not wait for diagnostic confirmation to initiate treatment if clinical suspicion is high and patient meets severity criteria 2
• Do not use corticosteroids alone—they are ineffective for idiopathic GBS and may worsen outcomes 8
• Do not assume lack of improvement at 4 weeks means treatment failure—recovery is often prolonged 2, 3
• Do not discharge patients prematurely—respiratory compromise can occur even after initial stabilization 1