Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
Department of Neurology, Erasmus Hospital, Free University of Brussels, Brussels, Belgium.
Epilepsia. 2021 Jul;62(7):1629-1642. doi: 10.1111/epi.16950. Epub 2021 Jun 6.
We aimed to characterize the clinical profile and outcomes of new onset refractory status epilepticus (NORSE) in children, and investigated the relationship between fever onset and status epilepticus (SE).
Patients with refractory SE (RSE) between June 1, 2011 and October 1, 2016 were prospectively enrolled in the pSERG (Pediatric Status Epilepticus Research Group) cohort. Cases meeting the definition of NORSE were classified as "NORSE of known etiology" or "NORSE of unknown etiology." Subgroup analysis of NORSE of unknown etiology was completed based on the presence and time of fever occurrence relative to RSE onset: fever at onset (≤24 h), previous fever (2 weeks-24 h), and without fever.
Of 279 patients with RSE, 46 patients met the criteria for NORSE. The median age was 2.4 years, and 25 (54%) were female. Forty (87%) patients had NORSE of unknown etiology. Nineteen (48%) presented with fever at SE onset, 16 (40%) had a previous fever, and five (12%) had no fever. The patients with preceding fever had more prolonged SE and worse outcomes, and 25% recovered baseline neurological function. The patients with fever at onset were younger and had shorter SE episodes, and 89% recovered baseline function.
Among pediatric patients with RSE, 16% met diagnostic criteria for NORSE, including the subcategory of febrile infection-related epilepsy syndrome (FIRES). Pediatric NORSE cases may also overlap with refractory febrile SE (FSE). FIRES occurs more frequently in older children, the course is usually prolonged, and outcomes are worse, as compared to refractory FSE. Fever occurring more than 24 h before the onset of seizures differentiates a subgroup of NORSE patients with distinctive clinical characteristics and worse outcomes.
我们旨在描述儿童新发难治性癫痫持续状态（NORSE）的临床特征和结局，并探讨发热与癫痫持续状态（SE）之间的关系。
2011 年 6 月 1 日至 2016 年 10 月 1 日期间，前瞻性纳入难治性 SE（RSE）患者进入 pSERG（儿科癫痫持续状态研究组）队列。符合 NORSE 定义的病例被归类为“已知病因的 NORSE”或“未知病因的 NORSE”。根据 RSE 发作时发热的出现和时间，对未知病因的 NORSE 进行亚组分析：发作时发热（≤24 小时）、发热前（2 周-24 小时）和无发热。
在 279 例 RSE 患者中，46 例符合 NORSE 标准。中位年龄为 2.4 岁，女性 25 例（54%）。40 例（87%）为未知病因的 NORSE。19 例（48%）在 SE 发作时出现发热，16 例（40%）发热前有发热，5 例（12%）无发热。有发热前发热的患者 SE 持续时间更长，结局更差，25%恢复基线神经功能。发作时发热的患者年龄更小，SE 发作时间更短，89%恢复基线功能。
在 RSE 的儿科患者中，16%符合 NORSE 的诊断标准，包括发热感染相关癫痫综合征（FIRES）的亚类。儿科 NORSE 病例也可能与难治性发热性 SE（FSE）重叠。FIRES 多见于年长儿，病程通常较长，预后较难治性 FSE 差。与癫痫发作前 24 小时以上出现发热可区分具有独特临床特征和较差结局的 NORSE 患者亚组。
Zotero 插件