Diazepam for outpatient treatment of nonconvulsive status epilepticus in pediatric patients with Angelman syndrome

Publication date: May 2018 Source:Epilepsy & Behavior, Volume 82 Author(s): Lila Worden, Olivia Grocott, Amanda Tourjee, Fonda Chan, Ronald Thibert Nonconvulsive status epilepticus (NCSE) is present in multiple pediatric neurogenetic syndromes with epileptic encephalopathies. While intravenous (IV) medications are used inpatient for treatment of critical illness-related NCSE, there is no consensus on treatment of ambulatory NCSE. Up to 50% of patients with Angelman syndrome (AS) have NCSE with myoclonic or atypical absence status. Here we report our experience in pediatric patients with AS and NCSE treated outpatient with a tapering course of oral diazepam. We conducted a chart review of 104 patients seen in the Angelman Syndrome Clinic at Massachusetts General Hospital from January 2008 to March 2017, who met the criteria. Response to treatment was defined as cessation of NCSE symptoms with electroencephalogram (EEG) confirmation when possible. Twenty-one patients with NCSE were identified, and 13 patients (9 male) with 25 episodes of NCSE were included. Mean age at NCSE episode was 5years 4months (15months–12years). Six patients had one episode of NCSE, and 7 patients had recurrent episodes (mean: 2.7; range: 2–4). Median diazepam treatment was 6days (4–12days), with a mean dose of 0.32mg/kg/day divided over 2–3 administrations, decreased every 2days. Nine episodes required multiple courses; however, oral diazepam alone was ultimately successful in 80...
Source: Epilepsy and Behavior - Category: Neurology Source Type: research