Abstracts

RATIONALE: Epileptic spasms (ES) is a rare seizure type, similar electroclinically to infantile spasms, but occurring in older children. They are defined by brief axial flexion/extension jerks (often in clusters) which are time-locked to a generalized decrement in EEG activity. None of the conventional anticonvulsants, alone or in combination, has proven effective in adequately controlling these unusual seizures. Several studies have demonstrated efficacy of intravenous immunoglobulin (IVIG) in refractory epilepsies, including infantile spasms. We report our initial experience with IVIG in the treatment of ES. METHODS: Five patients (range: 4 to 11 years, mean 7.6) with a confirmed diagnosis of epileptic spasms were treated with IVIG at a dose of 400 mg/kg/d for 5 days. We retrospectively reviewed their medical records to determine frequency and severity of seizure activity, as well as results of video-EEG recordings before and after (up to 12 months) IVIG administration. RESULTS: Four of five children showed initial improvement in seizure control within several weeks after treatment based on parental report and video-EEG evaluations; at 3 months, these four children had at least a 50% reduction in seizure frequency (range 50% to >90%). Sustained improvement was seen in 3 patients for up to 12 months. None of the patients became seizure-free, but one experienced a seizure-free interval of 1 month. No significant adverse effects were noted in any of the patients during either IVIG administration or the follow-up period. CONCLUSIONS: Our preliminary data suggest that IVIG may be a useful adjunct in the treatment of intractable epileptic spasms. Larger prospective studies are warranted to further evaluate the efficacy of this treatment modality.