Intravenous immunoglobulin as adjunctive therapy for juvenile spasms

Intravenous immunoglobulin has been reported to be an effective treatment for infantile spasms. Juvenile spasms are electrically and clinically similar to infantile spasms but occur in a later age group. We retrospectively reviewed the charts of five children (aged 4.5-11.5 years) at our institution. Their primary seizure type was juvenile spasms and they were treated with a single inpatient course of intravenous immunoglobulin (400 mg/kg/day intravenously for 5 consecutive days) on an adjunctive basis. Seizure frequency was determined from parental reports. By 3 months after treatment, improvement (a 50-92% reduction in seizure frequency) was noted in four patients; sustained benefit was seen in three patients for up to 12 months. One patient showed no response at 3 and 6 months and had an increase in seizure frequency at 1 year. We conclude that single-course intravenous immunoglobulin can be effective as adjunctive therapy for juvenile spasms and that benefit can persist for many months. However, larger controlled, prospective clinical trials are needed to validate this unconventional treatment modality for specific seizure types such as juvenile spasms.