From the Department of Neurology (E.M., N.M., R.L., K.D., J.C., K.T.T.), Columbia University, New York, NY; and Department of Neurology (A.A.), University of Miami, FL.
Neurology. 2020 Oct 20;95(16):e2280-e2285. doi: 10.1212/WNL.0000000000010787. Epub 2020 Sep 17.
To better understand the heterogeneous population of patients with new-onset refractory status epilepticus (NORSE), we studied the most severe cases in patients who presented with new-onset super-refractory status epilepticus (NOSRSE).
We report a retrospective case series of 26 adults admitted to the Columbia University Irving Medical Center neurologic intensive care unit (NICU) from February 2009 to February 2016 with NOSRSE. We evaluated demographics, diagnostic studies, and treatment course. Outcomes were modified Rankin Scale score (mRS) at hospital discharge and most recent follow-up visit (minimum of 2 months post discharge), NICU and hospital length of stay, and long-term antiepileptic drug use.
Of the 252 patients with refractory status epilepticus, 27/252 had NORSE and 26/27 of those had NOSRSE. Age was bimodally distributed with peaks at 27 and 63 years. The majority (96%) had an infectious or psychiatric prodrome. Etiology was cryptogenic in 73%, autoimmune in 19%, and infectious in 8%. Seven patients (27%) underwent brain biopsy, autopsy, or both; 3 (12%) were diagnostic (herpes simplex encephalitis, candida encephalitis, and acute demyelinating encephalomyelitis). On discharge, 6 patients (23%) had good or fair outcome (mRS 0-3). Of the patients with long-term follow-up data (median 9 months, interquartile range 2-22 months), 12 patients (71%) had mRS 0-3.
Among our cohort, nearly all patients with NORSE had NOSRSE. The majority were cryptogenic with few antibody-positive cases identified. Neuropathology was diagnostic in 12% of cases. Although only 23% of patients had good or fair outcome on discharge, 71% met these criteria at follow-up.
为了更好地了解新发难治性癫痫持续状态（NORSE）患者的异质人群，我们研究了新发生超难治性癫痫持续状态（NOSRSE）患者中最严重的病例。
我们报告了 2009 年 2 月至 2016 年 2 月期间，26 名成人因 NOSRSE 入住哥伦比亚大学欧文医学中心神经重症监护病房（NICU）的回顾性病例系列。我们评估了人口统计学、诊断研究和治疗过程。结果是出院时和最近随访时（出院后至少 2 个月）的改良 Rankin 量表评分（mRS）、NICU 和住院时间以及长期抗癫痫药物使用情况。
在 252 名难治性癫痫持续状态患者中，27/252 例为 NORSE，其中 26/27 例为 NOSRSE。年龄呈双峰分布，峰值分别为 27 岁和 63 岁。大多数（96%）患者有感染或精神科前驱症状。病因在 73%的患者中为特发性，19%为自身免疫性，8%为感染性。7 例（27%）患者进行了脑活检、尸检或两者兼有；3 例（12%）有明确诊断（单纯疱疹脑炎、念珠菌性脑炎和急性脱髓鞘性脑脊髓炎）。出院时，6 例（23%）患者有良好或尚可结局（mRS 0-3）。在有长期随访数据的患者中（中位数 9 个月，四分位距 2-22 个月），12 例（71%）患者的 mRS 为 0-3。
在我们的队列中，几乎所有 NORSE 患者均为 NOSRSE。大多数患者为特发性，很少有抗体阳性病例。神经病理学诊断率为 12%。尽管出院时只有 23%的患者有良好或尚可结局，但71%的患者在随访时达到了这些标准。
