OUTCOMES IN PATIENTS WITH DEE-SWAS TREATED WITH HORMONAL THERAPY IN COMBINATION WITH CLOBAZAM: PROSPECTIVE STUDY DATA

Аннотация

Developmental and epileptic encephalopathies with spike–wave activation in sleep (DEE-SWAS, formerly known as CSWS/ESES) represent a severe form of epilepsy characterized by neurodevelopmental regression, behavioral disturbances, speech impairment, and cognitive deficits. The key neurophysiological hallmark of the syndrome is pronounced epileptiform activity during slow-wave sleep with a high spike–wave index (SWI). Higher SWI values correlate with more severe clinical regression, and the duration of untreated sleep-activated epileptiform activity is directly associated with cognitive decline. To date, hormonal therapy (corticosteroids or ACTH) is considered the most effective treatment option; however, optimal treatment regimens and therapeutic combinations require further investigation. Clobazam, a benzodiazepine anticonvulsant with proven efficacy in DEE-SWAS, is frequently used as part of polytherapy. Nevertheless, data on combined treatment with hormonal therapy and clobazam in Central Asian populations are scarce.