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

Treatment of Guillain-Barré Syndrome (GBS)

Intravenous immunoglobulin (IVIg) at a dose of 0.4 g/kg body weight daily for 5 days or plasma exchange (5 sessions) should be initiated as first-line treatment for patients with Guillain-Barré Syndrome who are unable to walk unaided. 1, 2

First-Line Treatment Options

• IVIg (0.4 g/kg/day for 5 days, total dose 2 g/kg) is recommended as first-line therapy for GBS within 2-4 weeks of symptom onset, with strongest evidence for use within the first 2 weeks 1, 2
• Plasma exchange (5 sessions at 200-250 ml/kg over 1-2 weeks) is an equally effective alternative to IVIg but is generally less preferred due to practical considerations including availability, technical requirements, and completion rates 1, 3
• IVIg is generally preferred over plasma exchange due to easier administration, wider availability, and higher completion rates 1, 3
• Corticosteroids alone are not recommended for GBS treatment as they have shown no significant benefit and may even have negative effects 1, 2

Patient Assessment and Monitoring

• All patients with GBS require close monitoring for respiratory failure using vital capacity measurements, maximum inspiratory/expiratory pressures, and assessment of accessory respiratory muscle use 1, 4
• The Erasmus GBS Respiratory Insufficiency Score (EGRIS) should be used to calculate the probability of requiring mechanical ventilation 1
• The "20/30/40 rule" can help assess risk of respiratory failure: patient at risk if vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 1
• Regular neurological assessments should be performed to monitor disease progression and treatment response 5, 4

Management Based on Severity

Mild GBS (Patient Can Walk Unaided)

• Limited evidence exists for treatment benefit in mild cases, but treatment should be considered on a case-by-case basis 1, 6
• Close monitoring for disease progression is essential 6

Moderate to Severe GBS (Patient Cannot Walk Unaided)

• Initiate IVIg or plasma exchange immediately 1, 2
• Admit to inpatient unit with capability for rapid transfer to intensive care monitoring 5, 4
• Monitor for respiratory compromise, autonomic dysfunction, and pain 5, 4

Critical GBS (Respiratory Failure/ICU Care)

• Discontinue any immune checkpoint inhibitors if GBS is treatment-related 5
• Provide mechanical ventilation as needed 4
• Continue immunotherapy with IVIg or plasma exchange 5, 4
• Monitor for concurrent autonomic dysfunction 5
• Consider tracheostomy if prolonged ventilation is anticipated 4

Adjunctive Management

• Implement nonopioid management of neuropathic pain with gabapentinoids, tricyclic antidepressants, or carbamazepine 7, 2
• Address constipation/ileus which is common in GBS patients 5, 7
• Avoid medications that can worsen neuromuscular function, such as β-blockers, IV magnesium, fluoroquinolones, aminoglycosides, and macrolides 5, 7
• Provide deep vein thrombosis prophylaxis for immobilized patients 4
• Implement early rehabilitation to prevent complications of immobility 4

Treatment of Clinical Fluctuations

• About 10% of patients experience treatment-related fluctuations (TRFs) within 8 weeks after initial improvement 1, 6
• For TRFs, repeating the full course of IVIg or plasma exchange is common practice 1
• About 5% of patients initially diagnosed with GBS may develop chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with acute onset (A-CIDP) 2, 6
• Consider changing diagnosis to A-CIDP if progression continues after 8 weeks from onset 2

Special Populations

• In children, IVIg is preferred over plasma exchange due to better tolerability and fewer complications 1
• In pregnant women, both IVIg and plasma exchange are not contraindicated, but IVIg is generally preferred 1
• For immune checkpoint inhibitor-related GBS, discontinue the causative agent permanently and consider concurrent corticosteroids with IVIg or plasma exchange 5

Experimental Approaches

• Combined treatments of plasma exchange followed by IVIg have not shown significant additional benefit compared to either treatment alone 2, 3
• The "zipper method" (alternating plasma exchange and IVIg) has shown promise in small studies for severe GBS requiring mechanical ventilation, but requires further validation before widespread implementation 8