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费比洛芬酯治疗肌阵挛失神发作(EMAtS)患者的疗效。

Efficacy of felbamate in a cohort of patients with epilepsy with myoclonic atonic seizures (EMAtS).

机构信息

University of Michigan, C.S. Mott Children's Hospital, 1500 E. Hospital Dr., SPC 4279, Ann Arbor, MI 48109-4279, USA.

University of Iowa, 200 Hawkins Dr, Iowa City, IA 52242, USA.

出版信息

Epilepsy Res. 2024 Mar;201:107314. doi: 10.1016/j.eplepsyres.2024.107314. Epub 2024 Feb 2.

Abstract

Epilepsy with myoclonic atonic seizures (EMAtS) is a rare childhood onset developmental and epileptic encephalopathy which is frequently refractory to medical therapy. The optimal antiseizure medication remains unknown. This study reports the efficacy of felbamate in children with EMAtS. Six large pediatric epilepsy centers performed a retrospective chart review on patients diagnosed with EMAtS at their institutions and collected data on felbamate usage and efficacy. Responders were classified as patients who had a 50% or greater reduction in seizures with a given therapy. Out of 259 patients, 37 (14%) were treated with felbamate. The efficacy of felbamate was 62%, which was greater than that of either levetiracetam or valproic acid (15%, p < 0.001% and 32%, p = 0.001 respectively) and similar to that of the ketogenic diet (69%, p = 0.8). Felbamate appears to be an effective treatment for EMAtS and should be strongly considered in the treatment course of this disease.

摘要

肌阵挛失神癫痫(EMAtS)是一种罕见的儿童期起病的发育性和癫痫性脑病,常对药物治疗产生耐药性。最佳的抗癫痫药物仍不清楚。本研究报告了氨己烯酸在 EMAtS 患儿中的疗效。6 家大型儿科癫痫中心对在其机构诊断为 EMAtS 的患者进行了回顾性图表审查,并收集了氨己烯酸使用和疗效的数据。应答者被定义为在特定治疗下癫痫发作减少 50%或以上的患者。在 259 名患者中,37 名(14%)接受了氨己烯酸治疗。氨己烯酸的疗效为 62%,高于左乙拉西坦或丙戊酸(15%,p<0.001%和 32%,p=0.001),与生酮饮食(69%,p=0.8)相似。氨己烯酸似乎是治疗 EMAtS 的有效药物,应在该疾病的治疗过程中强烈考虑。

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