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

Treatment of Guillain-Barré Syndrome

Initiate intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days as first-line treatment for any patient with GBS who cannot walk unaided. 1

First-Line Treatment Selection

IVIg is preferred over plasma exchange because it is easier to administer, more widely available, has higher completion rates, and does not require special equipment. 1, 2 Both treatments are equally effective, but IVIg offers practical advantages in most clinical settings. 3, 4

Standard IVIg Protocol

• Dose: 0.4 g/kg body weight daily for 5 consecutive days (total dose 2 g/kg) 1, 5
• Timing: Start treatment as early as possible in the disease course to maximize effectiveness 1
• Indication threshold: Initiate immediately if the patient exhibits moderate to severe weakness, rapid progression, or any signs of respiratory compromise, dysphagia, facial weakness, or bulbar weakness 1

Plasma Exchange Alternative

• Protocol: 12-15 L over 4-5 exchanges during 1-2 weeks 2
• Timing window: Can be used within 4 weeks of weakness onset in patients unable to walk unaided 2
• Consider when: IVIg is contraindicated (IgA deficiency with anaphylaxis history) or unavailable 1

Critical Monitoring Requirements

Respiratory Assessment

Use the "20/30/40 rule" to identify patients at risk for respiratory failure: 1, 5

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

Monitor pulmonary function frequently including vital capacity, negative inspiratory force, and use of accessory respiratory muscles. 1, 6 Calculate the Erasmus GBS Respiratory Insufficiency Score (EGRIS) to determine probability of requiring mechanical ventilation. 1

Neurological Monitoring

• Assess motor strength, reflexes, and bulbar symptoms rigorously during and after each IVIg infusion 1
• Monitor for disease progression, autonomic dysfunction (arrhythmias, blood pressure fluctuations), and cardiac complications 6, 5
• Admit to a unit with rapid ICU transfer capability, as respiratory compromise can occur even during treatment 1, 5

Medications to Avoid

Never use these drugs in GBS patients as they worsen neuromuscular function: 1, 5

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

What NOT to Use

Do not use corticosteroids alone for GBS treatment—randomized controlled trials show no significant benefit, and oral corticosteroids may worsen outcomes. 1, 2 The European Academy of Neurology/Peripheral Nerve Society guideline weakly recommends against IV corticosteroids as well. 2

Do not combine plasma exchange followed immediately by IVIg—this approach is not recommended and provides no additional benefit. 2

Special Populations

Children

• Use the same 5-day IVIg regimen (0.4 g/kg/day for 5 days) rather than accelerated 2-day protocols, as treatment-related fluctuations occur more frequently with shorter regimens 1
• IVIg is preferred over plasma exchange due to better tolerability and fewer complications 1

Pregnant Women

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

IgA Deficiency

• Check serum IgA levels before first infusion—IgA deficiency increases anaphylaxis risk 1
• If deficiency confirmed, use IVIg preparations with reduced IgA content 1

Management of Insufficient Response

Expected Timeline

• Approximately 40% of patients do not improve within the first 4 weeks following standard IVIg treatment—this does not necessarily indicate treatment failure 1, 6
• Most recovery occurs within the first year, with 80% regaining independent walking ability by 6 months 6, 5

Treatment-Related Fluctuations (TRFs)

• Occur in 6-10% of patients within 2 months after initial improvement 1, 6
• Repeat the full course of IVIg or plasma exchange for TRFs 1, 6
• Consider switching from IVIg to plasma exchange (or vice versa) if no improvement by 4 weeks 6

Chronic Evolution

• Suspect chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) if patient experiences three or more TRFs and/or clinical deterioration ≥8 weeks after onset 6, 2
• Maintenance IVIg may be required if diagnosis changes to CIDP 6

Essential Supportive Care

Pain Management

Use nonopioid neuropathic pain medications: 6, 5, 2

• Gabapentin
• Pregabalin
• Duloxetine
• Avoid opioids 1

Preventive Measures

• Implement prophylaxis for deep vein thrombosis 1, 6
• Prevent pressure ulcers through positioning and skin care 6, 5
• Monitor for hospital-acquired infections (pneumonia, UTIs) 1, 5

Bulbar and Nutritional Support

• Assess swallowing safety in patients with bulbar weakness 1, 6
• Provide nutritional support as needed for dysphagia 1, 5
• Address constipation/ileus, which is common in GBS 1

Psychological Support

Address anxiety, depression, and hallucinations, which are common but often underrecognized complications. 6

Prognosis

• Mortality: 3-10%, primarily from cardiovascular and respiratory complications 1, 6, 5
• Risk factors for poor outcome include advanced age and severe disease at onset 1, 6
• Up to two-thirds of deaths occur during the recovery phase, requiring continued vigilance even after apparent improvement 5
• Use the modified Erasmus GBS outcome score (mEGOS) to calculate individual probability of regaining walking ability 6, 2