Abstracts

Rationale: Background: The impact of continuous spikes and waves during slow wave sleep (CSWS) on the developing brain has been well documented, and patients may present with cognitive regression prior to diagnosis. Disease-related variables such as etiology (lesional versus nonlesional), duration of abnormal EEG, age of diagnosis, and response to pharmacotherapy have been reported as potential contributors to long-term outcome. Objective: This is a prospective evaluation of the neurobehavioral status and neuropsychological profile of children with CSWS at time of diagnosis, and re-evaluation following 3 months of high-dose clobazam. Preliminary report of short-term outcome on two subjects is presented, and additional follow up data on further subjects will be available at the time of presentation. Methods: Methods: We prospectively evaluated patients with CSWS before and after 3 months of treatment with high-dose Clobazam. The battery of psychological tests was performed by the same clinical pediatric neuropsychologist. Evaluations included the Differential Ability Scales- Second Edition (DAS-2) (Elliott 2007) to measure intelligence, Peabody Picture Vocabulary Test-Fourth Edition (PPVT-4) (Dunn 2007) and Word Generation, NEPSY: A Developmental Neuropsychological Assessment-Second Edition (NEPSY-2) (Korkman, et al. 2007) for language, the Behavior Rating Inventory of Executive Function (BRIEF) (Gioia, et al.) for executive functions, the Scales of Independent Behaviors Revised (SIB-R) (Bruininks 1996) for adaptive skills, and the Child Behavior Checklist (CBCL) (Achenbach & Rescorla 2000) for psychosocial status (emotional, behavioral, and social development).Results: Results: One of the patients presented with CSWS at the age of 9 secondary to perinatal stroke (lesional); the second patient is 6 years old and had no evidence of brain abnormality on imaging (nonlesional). Decreased epileptiform discharges on EEG were observed in one patient (nonlesional), and no adverse effects were reported by either. Notable improvements (> 1 standard deviation) on adaptive skills were identified in both subjects. No significant changes in overall intelligence were noted, although both patients demonstrated improvement on verbal intellectual skills. Changes to language skills were inconsistent. No change in executive functions was observed after treatment. Psychosocial status, including symptoms of anxiety, depression, aggression, conduct disorder and ADHD remained at pre-treatment status. Conclusions: Conclusion: Preliminary findings on two patients with CSWS treated with high dose clobazam show select improvements on adaptive (independent) skills and verbal intelligence. No other neurocognitive, emotional, behavioral or social changes were observed after 3 months of treatment. These findings suggest that high-dose clobazam is a safe option for CSWS and treatment appears to have short-term benefits on neurobehavioral status. Long-term follow up will be necessary to determine the extent and/or maintenance of improvements, as well as impact on other skills.