Stowe Robert C, Kelley Sarah A, Bhatia Sonal, McLaren John R, Rao Lekha M, Patel Shital H, Fine Anthony L, Baumer Fiona, Duong Priscilla, Pavuluri Spriha, Liu Virginia B, Phillips Donald J
Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
Johns Hopkins Hospital, Baltimore, MD, USA.
Epilepsy Curr. 2025 Jun 9:15357597251344191. doi: 10.1177/15357597251344191.
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.
睡眠期棘波激活型发育性/癫痫性脑病（DEE-SWAS）是国际抗癫痫联盟（ILAE）新提出的命名，用以取代睡眠期癫痫性电持续状态（ESES）和睡眠期持续性棘慢波（CSWS）存在问题的脑电图和/或临床表型。这一命名更新是一项崇高的努力，旨在尽量减少如何定义和恰当使用这一堆首字母缩略词所造成的混乱，从而有助于未来的临床和研究工作。然而，名称的改变未能解决该领域内更大的挑战，这些挑战仍困扰着诊断和治疗，并阻碍了实质性的研究进展。通过对DEE-SWAS进行有针对性的文献综述，重点关注新的ILAE命名和RESCUE-ESES试验，我们将突出该领域主要的持续性难题。这些难题包括诊断生物标志物不足或缺乏（即棘波指数）、临床表现高度可变，从可疑关联到严重发育倒退，推测是由睡眠期棘波激活所致，以及治疗模式多变且往往无效。我们还将回顾DEE-SWAS更广泛的诊断评估。通过这样做，我们旨在阐明关键的研究和临床问题，这些问题可能会增进我们对DEE-SWAS患儿的诊断和治疗的理解，以及解决围绕睡眠激活型癫痫样放电的神经学影响的不确定性。
