Rebuilding the Tower of Babel: The Current Landscape and Emerging Opportunities in DEE-SWAS

Abstract

Developmental/epileptic encephalopathy with spike-wave activation in sleep (DEE-SWAS) is newly proposed nomenclature put forth by the International League Against Epilepsy (ILAE) to replace the problematic electrographic and/or clinical phenotypes of electrical status epilepticus of sleep (ESES) and continuous spike-wave in sleep (CSWS). The nomenclature update represents a noble effort to minimize the confusion of how to define and appropriately utilize this alphabet soup of acronyms, thereby aiding in future clinical and research efforts. The name change fails to capture greater challenges within the field, which still plague diagnosis and treatment and stagnates substantive research advancements. Through a directed literature review of DEE-SWAS with emphasis on the new ILAE nomenclature and the RESCUE-ESES trial, we will highlight major persistent quandaries in the field. These include inadequate or insufficient diagnostic biomarkers (ie, the spike wave index), the highly variable clinical manifestations, ranging from dubious associations to profound developmental regression, presumptively caused by spike-wave activation in sleep, and variable and often ineffective treatment paradigms. We will also review the broader diagnostic evaluation of DEE-SWAS. By doing so, we aim to shed light on crucial research and clinical questions that could advance our understanding of diagnosing and treating children with DEE-SWAS, as well as addressing the uncertainty surrounding the neurological effects of sleep-activated epileptiform discharges.