The Use of Perampanel in the Treatment of Lance-Adams Syndrome.

Journal of epilepsy research Pub Date : 2024-12-10 eCollection Date: 2024-12-01 DOI:10.14581/jer.24016
Vishal Pandya, Khalil S Husari
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Abstract

Lance Adams syndrome (LAS) is characterized by chronic action or intention myoclonus resulting from cerebral hypoxia. Perampanel, a non-competitive antagonist of aamino-3-hydroxy-5methyl-4 isooxazoleproprionic acid glutamate receptor, has demonstrated some efficacy in myoclonic epilepsy and other types of myoclonus. We report significant benefit in a patient with LAS treated with add on perampanel and provide a review of the relevant literature. In our case, a male patient in his 30s was found pulseless with unknown down time. The patient developed post anoxic myoclonus within 1 week from cardiac arrest. Patient continued to suffer from intractable myoclonus despite being treated with brivaracetam, valproic acid, and clonazepam. Perampanel was added to his medication regimen and up-titrated to 12 mg daily over 1-2 weeks. This resulted in significant improvement in frequency and severity of myoclonus for about 6 months. Growing evidence exists for perampanel as an adjunctive treatment in patients with post hypoxic myoclonus or LAS. A review of the available literature, comprised of case reports and case series, and suggests a potential role for perampanel in patients with LAS. Further study is warranted including controlled trials of perampanel use in post hypoxic myoclonus.

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Perampanel在Lance-Adams综合征治疗中的应用。
兰斯·亚当斯综合征(LAS)以脑缺氧引起的慢性动作性或意向性肌阵挛为特征。Perampanel是一种氨基-3-羟基-5甲基-4异唑丙酸谷氨酸受体的非竞争性拮抗剂,已证明对肌阵挛性癫痫和其他类型的肌阵挛有一定疗效。我们报告了一名LAS患者接受加药治疗的显著获益,并对相关文献进行了回顾。在我们的病例中,一名30多岁的男性患者被发现无脉搏,停搏时间不详。患者在心脏骤停后1周内出现缺氧后肌阵挛。尽管接受了布瓦西坦、丙戊酸和氯硝西泮治疗,患者仍然患有顽固性肌阵挛。将Perampanel添加到他的药物治疗方案中,并在1-2周内将剂量增加到每天12毫克。这导致肌阵挛的频率和严重程度在大约6个月内显著改善。越来越多的证据表明,perampanel可作为缺氧后肌阵挛或LAS患者的辅助治疗。对现有文献的回顾,包括病例报告和病例系列,并建议perampanel在LAS患者中的潜在作用。进一步的研究是有必要的,包括perampanel在缺氧后肌阵挛中的应用的对照试验。
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