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.