Abstracts

Rationale: Electrical status epilepticus during sleep (ESES) is an electroencephalographic pattern demonstrating significant activation of epileptiform discharges in sleep. The standard of treatment for ESES is high-dose oral diazepam. However, there have been only a few studies in the literature with the largest series being twenty-nine children. We examined the safety and efficacy of treatment with high-dose diazepam for children diagnosed with ESES. Methods: We conducted an IRB approved retrospective review of 35 children diagnosed with ESES and subsequently treated with high-dose oral diazepam at Children's Hospital Colorado from 2010 to 2011. Response to treatment as evidenced by decreased epileptiform discharges during sleep, and clinical improvement in development or language skills was assessed. Complications associated with treatment were evaluated. Results: Thirty-five children ages 2 to 12 years (mean=6.97, median=7) were diagnosed with ESES and treated with high-dose oral diazepam at Children's Hospital Colorado from 2010 to 2011. Standard treatment dose initiated during hospitalization was 1mg/kg/dose with a maximum dose of 60mg. Eleven subjects (31%) had a history of regular benzodiazepine use as part of their seizure treatment or sleep regimen prior to diagnosis of ESES. Two (6%) had a history of respiratory issues prior to treatment with high-dose diazepam. Thirty-four (97%) had a history of seizures prior to diagnosis of ESES with all but one having clinical seizures. Twenty-nine (83%) had developmental delay, and nineteen (54%) had language regression prior to diagnosis of ESES. Twenty-one (60%) showed short term of three months improvement after treatment with high-dose diazepam manifested by reduction in epileptiform discharges during sleep or clinical improvement in language or other developmental skills. Only two children (6%) experienced complications with high-dose diazepam, both of which were respiratory in nature. One had a brief drop in oxygen saturations to 84% requiring blow-by oxygen after administration of the first dose but was able to tolerate continued treatment. One patient on scheduled clonazepam at baseline had a history of significant medication related sedation prior to treatment with high-dose diazepam; he experienced significant respiratory depression with increasing oxygen requirement within two hours of first dose administration, followed by development of an aspiration pneumonia and persistent fatigue resulting in prolonged hospitalization. Treatment with high-dose diazepam was discontinued with subsequent improvement in mental status and oxygen requirement. Conclusions: 1) High dose diazepam has few side effects with respiratory depression occurring in only two patients, one of which had a previous history of severe sedation with medications. 2) 60% of patients showed short term improvement after treatment. 3) High-dose oral diazepam is a safe and effective treatment for patients with ESES although a prospective study with more patients would be helpful to verify these findings long term.