Treatment of Guillain-Barré Syndrome in Children

First-Line Treatment Options

The American Academy of Neurology recommends IVIg (0.4 g/kg body weight daily for 5 days) as the preferred first-line therapy in children due to better tolerability and fewer complications compared to plasma exchange 1
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, as suggested by the American Academy of Neurology 2
Treatment should be initiated as early as possible in the disease course to maximize effectiveness, according to the American Academy of Neurology 1
The American Academy of Neurology does not recommend corticosteroids alone for GBS treatment as they have shown no significant benefit and may even have negative effects on outcomes 1

Regular assessment of respiratory function should include vital capacity (risk if <20 ml/kg), maximum inspiratory pressure (risk if <30 cmH₂O), and maximum expiratory pressure (risk if <40 cmH₂O), as recommended by the European Respiratory Society 3
Single breath count and use of accessory respiratory muscles should be monitored, according to the American Thoracic Society 2, 4
Swallowing and coughing difficulties should be monitored to prevent aspiration, as suggested by the American Academy of Neurology 4

About 40% of patients do not show improvement in the first 4 weeks following treatment, which doesn't necessarily indicate treatment ineffectiveness, according to the American Academy of Neurology 4
Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement, as reported by the American Academy of Neurology 4
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, according to the American Academy of Neurology 4
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, as suggested by the American Academy of Neurology 4

Multidisciplinary supportive care is crucial and should include pain management, as pain is common in GBS patients, according to the American Pain Society 4
Prevention of pressure ulcers, hospital-acquired infections, and deep vein thrombosis is essential, as recommended by the Centers for Disease Control and Prevention 4
Management of autonomic dysfunction through monitoring of heart rate, blood pressure, and bowel/bladder function is necessary, according to the American Autonomic Society 3
Psychological support for anxiety, depression, and hallucinations which are frequent in GBS patients is crucial, as suggested by the American Psychiatric Association 4
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, according to the American Heart Association 3

A rehabilitation program with a rehabilitation specialist, physiotherapist, and occupational therapist is crucial for recovery, as recommended by the American Academy of Neurology, and should include range-of-motion exercises, stationary cycling, walking, and strength training to improve physical fitness and independence in activities of daily living 5, 6
The intensity of exercise must be closely monitored as overwork can cause fatigue, and management strategies for chronic pain include encouraging mobilization and administering drugs for neuropathic or nociceptive pain, with a strength of evidence level of moderate to high 5, 6

For patients with GBS following RSV infection, the American Academy of Pediatrics recommends considering palivizumab prophylaxis during RSV season if still in the acute phase of treatment, with a strength of evidence level of low to moderate 7
Pneumocystis pneumonia (PCP) prophylaxis with trimethoprim/sulfamethoxazole (5 mg/kg/d trimethoprim by mouth 3 times per week) should be considered, as recommended by the Infectious Diseases Society of America, with a strength of evidence level of high 7
Early signs of infection should be promptly investigated and antimicrobial regimens initiated early and for prolonged periods, with a strength of evidence level of moderate to high 7

Long-term residual complaints can include neuropathic pain, weakness, and fatigue, but recovery may still occur >5 years after disease onset, with a strength of evidence level of moderate, according to the American Academy of Neurology 5