From the Schulich School of Medicine and Dentistry (T.E.G.), Western University, London, Canada; Service de Neurologie (N.G.), Université Libre de Bruxelles-Hôpital Erasme, Brussels, Belgium; Division of Critical Care Neurology, Department of Neurology (S.E.H.), Mayo Clinic, Rochester, MN; Division of Epilepsy and Clinical Neurophysiology, Department of Neurology (T.L.), Boston Children's Hospital & Harvard Medical School, MA; and Comprehensive Epilepsy Center, Department of Neurology (L.G.H.), Yale University, New Haven, CT.
Neurology. 2019 Apr 23;92(17):802-810. doi: 10.1212/WNL.0000000000007322. Epub 2019 Mar 20.
New-onset refractory status epilepticus (NORSE) is a clinical presentation, not a specific diagnosis, in a patient without active epilepsy or other preexisting relevant neurologic disorder, with new onset of refractory status epilepticus (RSE) that does not resolve after 2 or more rescue medications, without a clear acute or active structural, toxic, or metabolic cause. Febrile infection-related epilepsy syndrome is a subset of NORSE in which fever began at least 24 hours prior to the RSE. Both terms apply to all age groups. Until recently, NORSE was a poorly recognized entity without a consistent definition or approach to care. We review the current state of knowledge in NORSE and propose a roadmap for future collaborative research. Research investigating NORSE should prioritize the following 4 domains: (1) clinical features, etiology, and pathophysiology; (2) treatment; (3) adult and pediatric evaluation and management approaches; and (4) public advocacy, professional education, and family support. We consider international collaboration and multicenter research crucial in achieving these goals.
新起难治性癫痫持续状态（NORSE）是指在无活动性癫痫或其他先前存在的相关神经障碍的患者中出现的一种临床表现，而非特定诊断，其特点为新发难治性癫痫持续状态（RSE），在使用 2 种或更多种抢救药物后仍未缓解，且无明确的急性或活动性结构、毒性或代谢原因。发热相关感染性癫痫综合征是 NORSE 的一个亚型，其特征为发热发生在 RSE 之前至少 24 小时。这两个术语适用于所有年龄段。直到最近，NORSE 还是一种认识不足的实体，缺乏一致的定义或治疗方法。我们回顾了 NORSE 的现有知识状况，并为未来的合作研究提出了路线图。针对 NORSE 的研究应优先考虑以下 4 个领域：（1）临床特征、病因和发病机制；（2）治疗；（3）成人和儿科评估和管理方法；（4）公众宣传、专业教育和家庭支持。我们认为国际合作和多中心研究对于实现这些目标至关重要。
