Schulze-Bonhage Andres, Steinhoff Bernhard, Garcés Mercedes, Hirsch Martin, Villanueva Vicente
Epilepsy Center, University Medical Center, University of Freiburg, Freiburg, Germany.
European Reference Network EpiCare, Europe.
Epilepsia Open. 2024 Dec;9(6):2537-2545. doi: 10.1002/epi4.13060. Epub 2024 Oct 10.
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.
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.
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.
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.
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.
评估司替戊醇（cenobamate）的抗癫痫作用，司替戊醇是一种具有至少两种作用机制的新型抗癫痫药物，用于治疗与拉斯穆森脑炎相关的罕见、高度耐药且进行性的癫痫综合征。
报告了来自弗赖堡、科尔克和巴伦西亚癫痫中心的3例局灶性癫痫患者，这些患者对10多种先前的治疗方案均耐药。评估包括引入司替戊醇后至少1年的随访，内容包括癫痫发作频率、发作严重程度（尤其是癫痫持续状态）以及联合用药的变化。
在这3例患者中，司替戊醇添加治疗被证明优于所有先前单独应用的抗癫痫和免疫调节治疗。不仅局灶性至双侧强直阵挛发作得到完全控制，而且局灶性运动性癫痫持续状态也不再发生。所有患者的联合用药均可减少。
这个针对罕见且高度耐药的癫痫综合征的病例系列表明，司替戊醇添加治疗对控制癫痫发作具有高效性。这对于与拉斯穆森脑炎相关的癫痫可能是有价值的信息，并且需要进一步阐明与先前包括钠通道阻滞剂和苯二氮䓬类药物在内的治疗相比，司替戊醇在卓越癫痫控制中所涉及的机制。
拉斯穆森脑炎是一种罕见的癫痫类型，会随着时间推移而恶化，并且对大多数抗癫痫药物反应不佳。我们描述了3例尝试了多种治疗但效果不佳的患者，但当他们在治疗中添加司替戊醇后，其效果优于其他药物。这也使他们能够减少总体用药量。
