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

Treatment of Guillain-Barré Syndrome in Children

Intravenous immunoglobulin (IVIg) at a dose of 0.4 g/kg body weight daily for 5 days is the recommended first-line treatment for children with Guillain-Barré Syndrome due to its easier administration, better tolerability, and fewer complications compared to plasma exchange. 1, 2

First-Line Treatment Options

• IVIg (0.4 g/kg body weight daily for 5 days) is preferred as first-line therapy in children due to better tolerability and fewer complications compared to plasma exchange 1, 2
• Plasma exchange (200-250 ml plasma/kg body weight in five sessions over 2 weeks) is an effective alternative when IVIg is contraindicated, not tolerated, or unavailable 1, 3
• 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 they have shown no significant benefit and may even have negative effects on outcomes 1

Monitoring and Respiratory Support

• Close respiratory monitoring is essential as up to 19% of pediatric patients may develop respiratory failure requiring mechanical ventilation 4
• The EGRIS-Kids score (which includes age, cranial nerve involvement, and GBS disability score) should be used to predict respiratory failure risk in children, with scores ranging from 4-50% probability 4
• Regular assessment of respiratory function should include:
  • Vital capacity (risk if <20 ml/kg)
  • Maximum inspiratory pressure (risk if <30 cmH₂O)
  • Maximum expiratory pressure (risk if <40 cmH₂O)
  • Single breath count and use of accessory respiratory muscles 3
• Swallowing and coughing difficulties should be monitored to prevent aspiration 3

Management of Disease Progression

• About 40% of patients do not show improvement in the first 4 weeks following treatment, which doesn't necessarily indicate treatment ineffectiveness 3
• Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement 3
• For patients with TRFs, repeating the full course of IVIg or switching to plasma exchange is common practice, although evidence supporting this approach is limited 3
• In approximately 5% of cases initially diagnosed as GBS, the diagnosis may change to acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) if repeated relapses occur 3

Supportive Care and Complication Management

• Multidisciplinary supportive care is crucial and should include:
  • Pain management, as pain is common in GBS patients 3
  • Prevention of pressure ulcers, hospital-acquired infections, and deep vein thrombosis 3
  • Management of autonomic dysfunction through monitoring of heart rate, blood pressure, and bowel/bladder function 3
  • Psychological support for anxiety, depression, and hallucinations which are frequent in GBS patients 3
• Patients should be monitored for cardiovascular complications, as up to two-thirds of deaths in GBS occur during the recovery phase due to cardiovascular and respiratory dysfunction 3

Prognosis

• With appropriate treatment, most children have a favorable outcome compared to adults 5
• Lower GBS disability score at nadir is the strongest predictor of recovery to independent walking 4
• Despite treatment, GBS remains a serious condition with potential for long-term complications including pain, fatigue, and residual weakness 5

Special Considerations

• For mildly affected patients who remain able to walk, the benefit of IVIg treatment is less clear, but treatment is often still provided 5
• For patients with poor prognosis or inadequate response to initial treatment, a second course of IVIg may be considered, although evidence for this approach is still being investigated 5