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Immune globulins are effective in severe pediatric Guillain-Barré syndrome
E Shahar1, Z Shorer, C M Roifman
1Child Neurology Unit; Rambam Medical Center; Haifa, Israel.
Insights
High-dose intravenous immune globulins (IVIG) effectively treated severe childhood Guillain-Barré syndrome. Most children showed rapid improvement, walking independently within weeks, demonstrating IVIG
Area of Science:
- Pediatric Neurology
- Immunology
- Clinical Therapeutics
Background:
- Severe Guillain-Barré syndrome in children can lead to significant disability, including respiratory failure.
- Traditional recovery courses for severe cases are often prolonged.
Purpose of the Study:
- To evaluate the efficacy and safety of high-dose intravenous immune globulins (IVIG) in children with severe Guillain-Barré syndrome.
- To determine if IVIG can accelerate recovery and improve outcomes in this population.
Main Methods:
- An open, prospective, multicenter study involving 26 children with severe Guillain-Barré syndrome.
- Administration of high-dose IVIG (2 gm/kg) over two consecutive days.
- Assessment of clinical improvement using the Disability Grading Scale.
Main Results:
- Rapid and marked improvement was observed in 25 out of 26 children within two weeks of IVIG infusion.
- Twenty children regained independent ambulation within one week; one patient was weaned from mechanical ventilation.
- No adverse effects requiring discontinuation of IVIG therapy were reported.
Conclusions:
- High-dose IVIG is an effective and safe treatment for severe childhood-onset Guillain-Barré syndrome.
- IVIG may serve as the initial treatment of choice, offering a faster recovery compared to natural disease progression.
- The study highlights the potential of IVIG to significantly alter the recovery trajectory in pediatric Guillain-Barré syndrome.
Abstract:
The effect of high-dose intravenous immune globulins was evaluated in an open prospective multicenter study of 26 children with severe Guillain-Barré syndrome. They presented with mild to moderate flaccid weakness of extremities, with cranial nerve involvement (20) and sensory impairment (22). All children rapidly deteriorated in 2-16 days (mean 6) to become bedridden, and 2 children also developed respiratory failure requiring artificial ventilation (Disability Grading Scale 4-5). Immune globulins were then administered at a total dose of 2 gm/kg, on 2 consecutive days, without adverse effects requiring discontinuation of therapy. Marked and rapid improvement was noted in 25 children, who improved by 1 to 2 Disability Grade Scales < or = 2 weeks after the infusion. Twenty were able to walk independently by 1 week, and 1 could be weaned off a ventilator. Eighteen children recovered by 2 weeks. The rest recuperated in a period of four months, including a child who was artificially ventilated for 4 weeks. The uniform rapid improvement and recovery associated with immune globulins contrasts with the slow recovery course in severe natural cases. We conclude that immune globulins are effective and safe in severe childhood-onset Guillain-Barré syndrome and therefore may serve as the initial treatment of choice.