Abstracts

RATIONALE: Continuous spikes and waves during slow sleep (CSWS) is described as nearly continuous spike and slow wave discharges during slow wave sleep. CSWS can be seen with a variety of medical conditions including Landau-Kleffner Syndrome (LKS) and epilepsy. Patients usually have associated neurobehavioral abnormalities ranging from aphasia to cognitive decline. CSWS is often refractory to standard anticonvulsant therapy. In an attempt to help eliminate CSWS and improve both seizures and neurobehavioral abnormalities we have treated 14 patients with CSWS with high dose diazepam (DZP).
METHODS: Patients with a diagnosis of CSWS were admitted to the long term monitoring unit at Children[ssquote]s Hospital, Boston. After CSWS was confirmed with a 24 hour EEG recording patients were treated with 1mg/kg/dose of DZP. Patients were hospitalized for the initial treatment to monitor for side effects including respiratory suppression and lethargy. Patients who responded with an improvement in the EEG abnormalities were treated with 0.5mg/kg/day DZP for three weeks. At the end of the treatment period patients were evaluated both clinically and electrographically.
RESULTS: Patients were divided into two groups for analysis: LKS (n=3) and CSWS with epilepsy with (n=11). The mean age was 7 years with a mean follow-up of 5 months. All three patients with LKS responded both electrographically with a normalization of the EEG and clinically with a significant improvement in language. Two out of three patients who had experienced seizures prior to treatment were seizure free following treatment. In the group of patients with CSWS and epilepsy there was an initial EEG and clinical response seen in 72% (8/11). Following the completion of treatment, 50% of patients had a continued improvement electrographically; however, only 25% had a prolonged reduction in seizure frequency. Side effects occurred in 28% (4/14) of patients and included behavioral problems and lethargy. No serious adverse effects necessitating the discontinuation of treatment occurred.
CONCLUSIONS: High dose valium is a safe and effective treatment for CSWS. Patients with LKS responded the best with all three experiencing an improvement in language as well as a normalization of their EEGs. Patients with underlying epilepsy also benefited; however, while the EEG response persisted, seizures often returned following the completion of treatment. High dose valium should be considered as a treatment option in patients with CSWS.