Management of Guillain-Barré Syndrome in Children
Intravenous immunoglobulin (IVIg) at a dose of 0.4 g/kg daily for 5 days is the preferred first-line treatment for children with Guillain-Barré Syndrome due to better tolerability and fewer complications compared to plasma exchange. 1, 2
First-Line Treatment Options
- IVIg (0.4 g/kg daily for 5 days) is recommended as the first-line therapy in children with GBS due to easier administration, better tolerability, and fewer complications compared to plasma exchange 3, 1
- The 5-day regimen is preferred over a 2-day regimen (total dose still 2 g/kg), as treatment-related fluctuations were more frequent with the 2-day regimen (5/23 children) compared to the 5-day regimen (0/23 children) 3
- Treatment should be initiated as early as possible to maximize effectiveness and potentially reduce mortality and need for mechanical ventilation 1, 4
- Plasma exchange (200-250 ml/kg over 5 sessions) is an effective alternative when IVIg is contraindicated, not tolerated, or unavailable 3, 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, 2
Monitoring and Respiratory Support
- Regular assessment of respiratory function is essential using the "20/30/40 rule": patient is at risk of respiratory failure if vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 3, 1
- Monitor for single breath count (a single breath count of ≤19 predicts a requirement for mechanical ventilation), use of accessory respiratory muscles, and ability to cough 3, 1
- Swallowing and coughing difficulties should be assessed to prevent aspiration 3, 1
- Autonomic dysfunction should be monitored via electrocardiography, heart rate, blood pressure, and bowel/bladder function 3, 1
Management of Disease Progression
- Approximately 40% of patients do not show improvement in the first 4 weeks following treatment, which doesn't necessarily indicate treatment ineffectiveness 1, 5
- Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement 1, 5
- 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 1, 5
- 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 1, 5
Supportive Care and Complication Management
- Multidisciplinary supportive care is crucial and should include pain management, as pain is common in GBS patients 3, 1
- Prevention of pressure ulcers, hospital-acquired infections (pneumonia, urinary tract infections), and deep vein thrombosis is essential 3, 1
- Psychological support for anxiety, depression, and hallucinations which are frequent in GBS patients is crucial 3, 1
- 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, 1
- Early recognition and management of limb contractures, corneal ulceration in patients with facial palsy, and pressure palsies in patients with limb weakness is important 3
Special Considerations for Children
- Young children (<6 years) may present with nonspecific or atypical clinical features such as poorly localized pain, refusal to bear weight, irritability, meningism, or unsteady gait, which can delay diagnosis 3
- Children with GBS treated with IVIg have shown shorter PICU stays compared to those treated with plasma exchange 6
- Early treatment with IVIg in children with severe GBS may reduce mortality and the need for intubation and mechanical ventilation 4
- Clinical improvement typically occurs within 2-3 days of beginning IVIg treatment in children 6, 7