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

Treatment of Guillain-Barré Syndrome (GBS)

The first-line treatment for Guillain-Barré Syndrome is intravenous immunoglobulin (IVIg) at a dose of 0.4 g/kg body weight daily for 5 days, which is equally effective as plasma exchange but generally preferred due to easier administration and better completion rates. 1

Initial Management and Treatment Options

• IVIg (0.4 g/kg body weight daily for 5 days) and plasma exchange (200-250 ml plasma/kg body weight in five sessions) are equally effective treatments for GBS 1
• IVIg is generally preferred as first-line therapy because it is easier to administer, more widely available, and has higher completion rates compared to plasma exchange 1
• Treatment should be initiated as early as possible in the disease course to maximize effectiveness 1
• Corticosteroids alone are not recommended for GBS treatment, as randomized controlled trials have shown no significant benefit and oral corticosteroids may even have negative effects on outcomes 1
• Combining plasma exchange followed by IVIg is no more effective than either treatment alone 1

Patient Assessment and Monitoring

• All patients with GBS require close monitoring for respiratory failure using the Erasmus GBS Respiratory Insufficiency Score (EGRIS) to calculate probability of requiring ventilation 1
• Regular respiratory function assessment is essential, including vital capacity, maximum inspiratory/expiratory pressures, and use of accessory respiratory muscles 1
• Consider the "20/30/40 rule": patient at risk of respiratory failure if vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 1
• Monitor for autonomic dysfunction via ECG, heart rate, blood pressure, and bowel/bladder function 1
• Assess for swallowing and coughing difficulties to prevent aspiration 1

ICU Admission Criteria

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

Special Patient Populations

Children

• IVIg is preferred over plasma exchange in children due to better tolerability and fewer complications 1
• Standard adult regimen of 2 g/kg over 5 days is recommended rather than accelerated 2-day regimen, as treatment-related fluctuations are more frequent with the 2-day protocol 1

Pregnant Women

• Both IVIg and plasma exchange are not contraindicated during pregnancy 1
• IVIg is generally preferred due to fewer monitoring requirements and considerations 1

GBS Variants

• Miller Fisher Syndrome (MFS): Treatment generally not recommended due to mild course and complete recovery within 6 months in most cases, but close monitoring is required 1
• Bickerstaff Brainstem Encephalitis (BBE): Treatment with IVIg or plasma exchange is justified due to severity, despite limited evidence 1
• For other variants, many experts administer IVIg or plasma exchange despite limited evidence 1

Management of Clinical Progression

• About 40% of patients do not improve in first 4 weeks following treatment, which doesn't necessarily indicate treatment ineffectiveness 1
• Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement 1
• Repeating the full course of IVIg or plasma exchange is common practice for TRFs, although evidence supporting this approach is limited 1
• Consider acute-onset chronic inflammatory demyelinating polyneuropathy (CIDP) in patients with ≥3 TRFs or clinical deterioration ≥8 weeks after onset 1

Complications Management

• Pain, hallucinations, anxiety, and depression are common and require early recognition and treatment 1
• Prevent complications specific to GBS: manage bulbar palsy, protect corneas in facial palsy, prevent limb contractures 1
• Standard preventive measures for pressure ulcers, hospital-acquired infections, and deep vein thrombosis are recommended 1
• A multidisciplinary team approach is advised, including nurses, physiotherapists, rehabilitation specialists, occupational therapists, speech therapists, and dietitians 1

Prognosis

• About 80% of patients regain walking ability at 6 months after disease onset 1
• Mortality occurs in 3-10% of cases, most commonly due to cardiovascular and respiratory complications 1
• Risk factors for mortality include advanced age and severe disease at onset 1
• Long-term residual complaints can include neuropathic pain, weakness, and fatigue 1