Abstracts

Rationale: Treatment strategies in patients with the electroencephalographic (EEG) pattern of electrical status epilepticus in sleep (ESES) are based on expert opinions and small case series. The objective of this study was to review our long-term experience with high-dose diazepam treatment. Methods: We retrospectively reviewed patients who were monitored by overnight inpatient video-EEG at Boston Children's Hospital with: 1) presence of ESES considered as at least 50% of the non-REM sleep tracing occupied by spike-waves, 2) on high-dose diazepam treatment (1mg/Kg the first night, followed by 0.5 mg/Kg/day for a variable time subsequently and eventually a tapering schedule), 3) during the period from 2000 to 2007, and 4) had at least 4 years of clinical and EEG follow-up data after the last treatment with high-dose diazepam. We investigated their electro-clinical features and recorded the age of occurrence of the significant clinical events. Per patient, we recorded the number and timing of treatments with high-dose diazepam, any adverse effects associated with this treatment protocol, and the timing of any other anti-epileptic treatments needed after the last high-dose diazepam treatment. Results: 25 patients were identified. Demographic features are presented in Table 1. Treatment with high-dose diazepam led to improvement in clinical (seizure frequency and severity, cognitive and behavioral function) and EEG features in all patients, but its effect slowly wore off over time. Five patients received two trials of high-dose diazepam treatment and four patients received three trials of high-dose diazepam (Table 2). 20/25 patients required other antiepileptic treatments after high-dose diazepam, including epilepsy surgery (functional hemispherectomy and corpus callosotomy) in two cases. Adverse effects of the high-dose diazepam treatment protocol included daytime somnolence (two patients), agitation (two patients), and ataxia (two patients); these adverse effects led to treatment discontinuation in one patient. There were no respiratory complications related to the treatment administration. Conclusions: Children with ESES experienced improvement in clinical and EEG features after high-dose diazepam treatment. Beneficial effects dissipated and further trials of high-dose diazepam and/or other treatments were needed. Infrequent and mild adverse effects were found with the high-dose diazepam treatment protocol. No patients suffered from respiratory complications related to the treatment.