Stiripentol for the treatment of refractory status epilepticus.

Leona Möller, Ole J Simon, Clara Jünemann, Meike Austermann-Menche, Marc-Philipp Bergmann, Lena Habermehl, Katja Menzler, Lars Timmermann, Adam Strzelczyk, Susanne Knake
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Abstract

Background: Status epilepticus (SE) is one of the most common neurological emergencies and an acutely life-threatening condition characterized by high mortality and morbidity. Despite the well-established staged therapy of status epilepticus, especially stages 1 and 2, more than one third of patients develop (super-) refractory SE. Despite a large variety of potential treatment options for super-refractory SE, there is an unmet clinical need of potential new treatment ideas in this often desperate clinical situation. A number of studies have demonstrated the safety and efficacy of stiripentol (STP) in patients with Dravet syndrome (DS) and in children with focal epilepsy and generalized epilepsies. Some smaller series and case reports have documented the use of STP in the treatment of status epilepticus in adult patients.

Methods: We retrospectively analyzed all patients who were admitted to the Department of Neurology at Marburg University Hospital between 2013 and 2023 with a diagnosis of (super)-refractory status epilepticus and who received additional treatment of SE with STP. All patients who received STP during the SE were included, regardless of previous medication.

Results: SE ceased in 64% of 25 patients (13 female and 12 male). The mean age was 58.6 ± 21.9 years (mean ± SD). 72% had a structural epilepsy. In 20% of patients, SE was terminated by the administration of STP alone in 32% of cases, while in a further 32% of patients, the simultaneous administration of multiple anti-seizure medications (ASMs) including STP was potentially responsible for the cessation of the SE, with valproic acid (VPA), benzodiazepines and STP, being the most frequently implicated ASMs. In 12% of patients, there was at least a temporary improvement in the electroencephalogram (EEG). Stiripentol had to be discontinued in three cases due to a reduction in vigilance or hypercalcemia.

Conclusions: Stiripentol may represent a promising additional treatment option for refractory and super-refractory status epilepticus. The tolerability of this treatment has already been demonstrated in previous studies, and was also reflected in these data. Further prospective investigation in larger patient populations are necessary to ascertain the efficacy of stiripentol in SE.

Trial registration: NCT06540378, retrospectively registered.

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治疗难治性癫痫状态的斯利潘托。
背景:癫痫状态(SE)是最常见的神经系统急症之一,也是一种危及生命的急性疾病,死亡率和发病率都很高。尽管癫痫状态的分期治疗(尤其是第 1 期和第 2 期)已得到广泛认可,但仍有三分之一以上的患者发展为(超级)难治性癫痫。尽管针对超级难治性 SE 的潜在治疗方案种类繁多,但在这种往往令人绝望的临床情况下,潜在的新治疗理念仍未满足临床需求。许多研究已经证明,斯奇潘托(STP)对德拉维特综合征(Dravet Syndrome,DS)患者以及局灶性癫痫和全身性癫痫患儿具有安全性和有效性。一些较小规模的系列研究和病例报告也记录了 STP 用于治疗成年患者的癫痫状态:我们回顾性分析了 2013 年至 2023 年期间马尔堡大学医院神经内科收治的所有诊断为(超)难治性癫痫状态并接受 STP 额外 SE 治疗的患者。所有在癫痫发作期间接受过 STP 治疗的患者均被纳入研究范围,与之前的用药情况无关:结果:25 名患者(13 名女性和 12 名男性)中有 64% 的 SE 停止。平均年龄为 58.6 ± 21.9 岁(平均 ± SD)。72%的患者患有结构性癫痫。在 20% 的患者中,32% 的病例仅因服用 STP 而终止癫痫发作,而在另外 32% 的患者中,包括 STP 在内的多种抗癫痫药物(ASMs)的同时服用可能是导致癫痫发作停止的原因,其中丙戊酸(VPA)、苯二氮卓类药物和 STP 是最常涉及的 ASMs。在 12% 的患者中,脑电图(EEG)至少出现了暂时性改善。有三例患者因警觉性下降或高钙血症而不得不停用斯利潘托:结论:对于难治性和超难治性癫痫状态,斯利潘托可能是一种很有前景的额外治疗选择。这种治疗方法的耐受性已在之前的研究中得到证实,这些数据也反映了这一点。有必要在更大的患者群体中开展进一步的前瞻性研究,以确定斯奇潘托对SE的疗效:试验注册:NCT06540378,回顾性注册。
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来源期刊
CiteScore
7.40
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审稿时长
14 weeks
期刊最新文献
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