加用塞诺巴马特治疗拉斯穆森脑炎引起的难治性癫痫的疗效。

IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Epilepsia Open Pub Date : 2024-10-10 DOI:10.1002/epi4.13060
Andres Schulze-Bonhage, Bernhard Steinhoff, Mercedes Garcés, Martin Hirsch, Vicente Villanueva
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引用次数: 0

摘要

目的评估仙诺巴马特(一种至少具有两种作用机制的新型抗癫痫药物)对罕见、高度耐药且与拉斯穆森脑炎相关的进展性癫痫综合征的抗癫痫效果:方法:报告了来自弗莱堡、科克和巴伦西亚癫痫中心的三名患者,他们患有局灶性癫痫,对之前超过 10 种治疗方案产生了耐药性。评估包括使用西诺巴马酯后至少一年的随访,内容包括癫痫发作频率、发作严重程度(尤其是癫痫状态)以及联合用药的变化:在这三名患者中,西诺巴马特的附加治疗效果优于之前单独使用的所有抗癫痫和免疫调节治疗。不仅完全控制了局灶性至双侧强直阵挛发作,而且不再出现局灶性运动状态癫痫。所有患者都减少了联合用药:这组罕见且高度耐药的癫痫综合征病例表明,西诺巴马酯附加治疗对控制癫痫发作具有很高的疗效。这对与拉斯穆森脑炎相关的癫痫患者来说可能是一个有价值的信息,与之前的钠通道阻滞剂和苯二氮卓类药物等治疗方法相比,该药对癫痫发作的控制效果更佳,因此需要进一步阐明其机制:拉斯穆森脑炎是一种罕见的癫痫类型,它会随着时间的推移而恶化,并且对大多数癫痫发作药物反应不佳。我们描述了三位患者的情况,他们尝试了多种治疗方法,但都没有取得很大成功,但当他们在治疗中加入仙蟾毒酯后,效果比其他药物更好。这也让他们减少了服用药物的总量。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Efficacy of add-on Cenobamate treatment in refractory epilepsy due to Rasmussen's encephalitis

Objective

To assess antiseizure effects of cenobamate, a new antiseizure medication with at least two mechanisms of action, in the rare, highly pharmacoresistant and progressive epilepsy syndrome related to Rasmussen's encephalitis.

Methods

Three patients from the epilepsy centers in Freiburg, Kork, and Valencia are reported with focal epilepsy which had been pharmacoresistant to more than 10 prior treatment regimens. Assessment included at least 1 year of follow-up after cenobamate introduction and included seizure frequency, seizure severity (in particular status epilepticus) and changes in co-medication.

Results

In the three patients, cenobamate add on treatment proved superior to all prior antiseizure and immunomodulatory treatments which had been individually applied. Not only were focal to bilateral tonic–clonic seizure completely controlled, but also focal motor status epilepticus no longer occurred. Co-medication could be reduced in all patients.

Significance

This case series in a rare and highly pharmacoresistant epilepsy syndrome suggests high efficacy of cenobamate add-on treatment for seizure control. This may be a valuable information in epilepsy related to Rasmussen encephalitis and calls for further elucidation of the mechanism involved in superior seizure control also compared to prior treatments including sodium channel blockers and benzodiazepines.

Plain Language Summary

Rasmussen's encephalitis is a rare type of epilepsy that gets worse over time and doesn't respond well to most seizure medications. We describe three patients who tried many treatments without much success, but when they added cenobamate to their treatment, it worked better than the other medications. This also let them lower the overall amount of medication they were taking.

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来源期刊
Epilepsia Open
Epilepsia Open Medicine-Neurology (clinical)
CiteScore
4.40
自引率
6.70%
发文量
104
审稿时长
8 weeks
期刊最新文献
Efficacy and tolerability of low versus standard daily doses of antiseizure medications in newly diagnosed focal epilepsy. A multicenter, randomized, single-blind, non-inferiority trial (STANDLOW). Three cases of atypical Rasmussen's encephalitis with delayed-onset seizures. GATAD2B-related developmental and epileptic encephalopathy (DEE): Extending the epilepsy phenotype and a literature appraisal. Intrinsic brain network stability during kainic acid-induced epileptogenesis. Diagnostic yield of utilizing 24-72-hour video electroencephalographic monitoring in the diagnosis of seizures presenting as paroxysmal events in resource-limited settings.
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