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成功使用吡仑帕奈治疗部分连续性癫痫发作:两例儿科病例。

Successful treatment of epilepsia partialis continua with perampanel: two pediatric cases.

机构信息

Department of Pediatric Neurology, King Fahad Specialist Hospital, Dammam, Saudi Arabia.

出版信息

Epileptic Disord. 2021 Apr 1;23(2):385-391. doi: 10.1684/epd.2021.1262.

Abstract

Epilepsia partialis continua (EPC) is a form of focal motor status epilepticus, associated with multiple etiologies. Etiology-specific treatments, such as hemispherotomy for Rasmussen encephalitis, lesionectomy for focal cortical dysplasia, and metabolic correction for non-ketotic hyperglycemia, have proven to be efficacious in treating EPC, but, in general, EPC is difficult to treat and often drug-resistant, and there is little evidence to guide therapy. We report the successful treatment of EPC with perampanel in two pediatric patients. The first patient was a 12-year-old boy with neuronal ceroid lipofuscinosis (NCL) who started to have EPC around the age of 10 years, characterized by left hemifacial myoclonic twitches and hemi-body jerks that were almost continuous throughout the day and disappeared during sleep. He had failed several antiepileptic drugs (AEDs). The EPC stopped within three days of initiating perampanel. The second patient was a six-year-old boy with POLG-related mitochondrial disease who presented to the emergency room with continuous jerky movements of the right arm and face after a trivial head injury. After failing several AEDs, including a midazolam drip, the EPC was controlled with perampanel. Both patients showed dramatic improvement and continue to show sustained efficacy after around five months of follow-up. Based on our observations, perampanel, which has a unique mechanism of action, appears to be a promising therapeutic option for treating EPC. [Published with video sequence].

摘要

部分连续性癫痫(EPC)是一种局灶性运动性癫痫持续状态,与多种病因有关。针对病因的治疗方法,如针对 Rasmussen 脑炎的半脑切除术、针对局灶性皮质发育不良的病灶切除术,以及针对非酮性高血糖的代谢纠正,已被证明对治疗 EPC 有效,但总的来说,EPC 难以治疗且往往耐药,而且很少有证据指导治疗。我们报告了用吡仑帕奈成功治疗两例儿科 EPC 患者。第一例患者是一名 12 岁男孩,患有神经元蜡样脂褐质沉积症(NCL),他在 10 岁左右开始出现 EPC,表现为左侧半面肌阵挛抽搐和半身抽搐,几乎整天持续发作,在睡眠中消失。他已经尝试了几种抗癫痫药物(AEDs)但均失败。开始使用吡仑帕奈后三天内 EPC 停止。第二例患者是一名 6 岁男孩,患有 POLG 相关线粒体疾病,在轻微头部外伤后急诊出现右臂和面部持续抽搐。在尝试了几种 AEDs 包括咪达唑仑滴注后,EPC 被吡仑帕奈控制。两名患者在观察后都有明显改善,在随访约 5 个月后仍继续显示出持续疗效。根据我们的观察,吡仑帕奈具有独特的作用机制,似乎是治疗 EPC 的一种有前途的治疗选择。[附有视频序列]。

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