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

Treatment of Guillain-Barré Syndrome in Children

Intravenous immunoglobulin (IVIg) at 0.4 g/kg daily for 5 consecutive days is the recommended first-line treatment for children with GBS who cannot walk independently (GBS disability score ≥3). 1

First-Line Immunotherapy

IVIg is preferred over plasma exchange because it is easier to administer, more widely available, better tolerated, and associated with fewer complications in children. 1

IVIg Dosing Protocol

• Standard regimen: 0.4 g/kg/day for 5 consecutive days 2, 1
• The 5-day regimen is superior to the 2-day regimen: Treatment-related fluctuations occurred in 5/23 children (22%) with the 2-day regimen versus 0/23 children (0%) with the 5-day regimen 1
• Timing matters: Start treatment as early as possible, ideally within 2 weeks of symptom onset 2, 1
• Early treatment (within 10 days) significantly shortens time to maximum symptoms (6.9 vs 8.8 days) 3

Alternative: Plasma Exchange

• Use when IVIg is contraindicated, not tolerated, or unavailable 1
• Dosing: 200-250 ml/kg over 5 sessions 2, 1
• Plasma exchange and IVIg are equally effective but plasma exchange is more technically demanding 2

What NOT to Use

• Corticosteroids alone are not recommended as they show no significant benefit and may have negative effects 1, 4
• However, corticosteroids can be considered if acute-onset CIDP is suspected due to prolonged disease course 4

Critical Respiratory Monitoring

Use the "20/30/40 rule" to identify impending respiratory failure: 1, 5

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

Additional respiratory warning signs: 1, 5

• Single breath count ≤19 (predicts need for mechanical ventilation)
• Use of accessory respiratory muscles
• Inability to cough effectively
• Approximately 20% of GBS patients develop respiratory failure requiring mechanical ventilation 5

Autonomic and Cardiovascular Monitoring

Monitor continuously for autonomic dysfunction: 1, 5

• Electrocardiography
• Heart rate and blood pressure fluctuations
• Bowel and bladder function
• Critical: Up to two-thirds of GBS deaths occur during recovery phase due to cardiovascular and respiratory dysfunction 1

Managing Treatment Response and Complications

Expected Disease Course

• 40% of patients show no improvement in the first 4 weeks following treatment—this does NOT indicate treatment failure 1, 5
• Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement 1, 5
• For TRFs, repeat the full course of IVIg or plasma exchange 5

Essential Supportive Care

Pain management is crucial as pain is common in GBS patients 1, 5

Prevent complications: 1, 5

• Pressure ulcers
• Hospital-acquired infections (pneumonia, urinary tract infections)
• Deep vein thrombosis

Provide psychological support for anxiety, depression, and hallucinations which are frequent 1, 5

Avoid medications that worsen neuromuscular function: 5

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

Pediatric-Specific Considerations

Young children (<6 years) present atypically: 1

• Poorly localized pain
• Refusal to bear weight
• Irritability
• Meningism
• Unsteady gait
• These nonspecific features can delay diagnosis

Prognosis and Follow-Up

Recovery timeline: 1, 5

• 80% regain walking ability at 6 months
• Recovery can continue for more than 3 years (some sources indicate >5 years)
• Mortality: 3-10% of cases

Recurrence is rare (2-5%) but still higher than general population lifetime risk (0.1%) 5

Long-term sequelae may include: 5

• Neuropathic pain
• Residual weakness
• Chronic fatigue

Rehabilitation is essential: Involve rehabilitation specialists, physiotherapists, and occupational therapists early 5

Common Pitfalls to Avoid

• Do not delay treatment waiting for complete diagnostic certainty—clinical suspicion with supportive CSF/electrophysiology is sufficient 4
• Do not use the 2-day IVIg regimen—the 5-day regimen has significantly fewer treatment-related fluctuations 1
• Do not assume treatment failure if no improvement occurs in the first 4 weeks—this is expected in 40% of patients 1
• Do not miss respiratory decline—monitor closely using objective measures, not just clinical appearance 1, 5