What is the recommended treatment with Intravenous Immunoglobulin (IVIG) for a patient with Guillain Barre Syndrome?

IVIG Treatment for Guillain-Barré Syndrome

First-Line Treatment Recommendation

Administer intravenous immunoglobulin (IVIG) at 0.4 g/kg body weight daily for 5 consecutive days (total dose 2 g/kg) as first-line treatment for patients with Guillain-Barré syndrome who are unable to walk unaided. 1

IVIG is preferred over plasma exchange because it is easier to administer, more widely available, and has higher completion rates, despite equivalent efficacy. 1, 2

Indications for Treatment

Initiate IVIG immediately in patients with: 1

• Moderate to severe weakness (unable to walk unaided within 2-4 weeks of symptom onset) 1
• Rapid progression of symptoms 1
• Any signs of respiratory compromise (vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O) 1
• Dysphagia, facial weakness, or bulbar weakness 1

Do not wait for antibody test results or electrodiagnostic confirmation if clinical suspicion is high—early treatment maximizes effectiveness. 1, 3

Dosing Protocol

Standard Regimen

• 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) 1
• Use ideal body weight for dosing calculations, not actual body weight, as IVIG distributes in plasma and extracellular fluid spaces that correlate with lean body mass rather than adipose tissue. 1
• When total dose exceeds 80 grams, it may be divided over 3-5 days at 0.4 g/kg to improve tolerability. 1

Special Populations

• Children: Use the same 5-day 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
• Pregnant women: IVIG is preferred over plasma exchange due to fewer monitoring requirements; neither treatment is contraindicated during pregnancy. 1
• Miller-Fisher Syndrome: Treatment is generally not recommended as most patients recover completely within 6 months without intervention, though close monitoring is essential. 1

Pre-Treatment Considerations

Critical Safety Check

Verify serum IgA levels before the first infusion—IgA deficiency increases the risk of anaphylaxis. If deficiency is confirmed, use IVIG preparations with reduced IgA levels. 1

Medications to Avoid

Do not administer the following medications during IVIG treatment, as they worsen neuromuscular function: 1

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

Monitoring During Treatment

Infusion Monitoring

Monitor rigorously during and after each infusion for: 1

• Neurological function: motor strength, reflexes, bulbar symptoms 1
• Adverse reactions: anaphylaxis (especially in IgA deficiency), thrombotic events, renal dysfunction 1

Respiratory and Autonomic Monitoring

• Admit to inpatient unit with rapid ICU transfer capability, as respiratory compromise can occur even during treatment 1
• Serial measurements of vital capacity, negative inspiratory force, and maximum inspiratory/expiratory pressures 3
• Continuous cardiac monitoring for arrhythmias and blood pressure instability 3

Treatment Response and Complications

Expected Timeline

• Approximately 40% of patients do not improve in the first 4 weeks following treatment—this does not necessarily indicate treatment failure, as progression might have been worse without therapy. 1, 3
• 80% of patients regain independent walking ability at 6 months. 1
• Recovery can continue for more than 3 years, with improvement possible even beyond 5 years. 3

Treatment-Related Fluctuations (TRFs)

• Occur in 6-10% of patients within 2 months after initial improvement 1
• Defined as disease progression following initial treatment-induced improvement or stabilization 3
• Repeat the full 5-day course of IVIG (0.4 g/kg/day for 5 days) for TRFs, though evidence for this practice is limited. 1

When to Reconsider Diagnosis

Consider acute-onset CIDP if: 3

• Progression continues after 8 weeks from onset 3
• Patient has three or more TRFs (occurs in approximately 5% of patients initially diagnosed with GBS) 3

Concurrent Supportive Care

Pain Management

• Gabapentin, pregabalin, or duloxetine for neuropathic pain and paresthesias—these can be initiated alongside IVIG without interaction or contraindication. 1, 3
• Avoid opioids for neuropathic pain management. 1

Additional Supportive Measures

• Deep vein thrombosis prophylaxis 1
• Pressure ulcer prevention 1
• Nutritional support with dysphagia evaluation if needed 1
• Treatment of constipation/ileus 1
• Psychological support for anxiety and depression 3

Alternative Treatment

Plasma exchange (200-250 ml/kg over 4-5 sessions) is equally effective as IVIG and should be considered when IVIG is contraindicated or unavailable. 1, 2 However, IVIG remains preferred due to ease of administration and higher completion rates. 1

Combining plasma exchange with IVIG does not confer significant advantage over either treatment alone. 2

Common Pitfalls

• Do not delay treatment waiting for CSF protein elevation—albumino-cytological dissociation may be absent in the first week. 3
• Do not use corticosteroids alone—randomized controlled trials show no significant benefit, and oral corticosteroids may have negative effects on outcomes. 1
• Do not dismiss GBS based on normal early electrodiagnostic studies—findings may not be evident within the first week. 3
• Do not withhold IVIG in patients with suspected infection—preceding infections have usually resolved before weakness onset, and treatment should not be delayed. 1