High-dose immunoglobulin therapy for Guillain-Barré syndrome in Japanese children.

Background: Guillain-Barré syndrome (GBS) is an acute acquired demyelinating polyneuropathy, presumed to be immune-mediated. Intravenous immunoglobulin (IVIg) has been used to treat GBS and was found to be effective. However, a well-controlled study of pediatric GBS has not been conducted in Japan. Therefore, to evaluate the efficacy of IVIg in the treatment of GBS, an open-labeled study was performed in pediatric patients.

Methods: Participants in the study were required to be younger than 15 years old, and diagnosed as having moderate or severe GBS. IVIg (400 mg/kg per day) was administered to patients for five consecutive days. Predefined outcome measures were defined on a seven-point scale of motor function (Hughes' functional grade [FG]).

Results: Eleven patients were treated with IVIg. The median time taken to improve by one grade on the FG scale was 10.0 days after initial treatment. Two weeks after initial treatment, 72.7% of patients treated with IVIg improved by one or more grades, and 36.4% improved by two or more grades, measured on the FG scale. After 4 weeks an improvement by one or more grades was observed in 81.8% of patients, and two or more grades in 63.6% of patients. These improvement rates were markedly greater than would occur with the natural course of GBS1. Adverse events (subjective symptoms or abnormal laboratory findings) were observed in four patients, although all were temporary and mild.

Conclusions: The authors conclude that IVIg is a safe and effective treatment for childhood GBS, which shortens the time to recovery.