Pandya Vishal, Husari Khalil S
Department of Neurology, Comprehensive Epilepsy Center, Medical College of Wisconsin, Milwaukee, WI, USA.
Department of Neurology, Comprehensive Epilepsy Center, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
J Epilepsy Res. 2024 Dec 10;14(2):97-101. doi: 10.14581/jer.24016. eCollection 2024 Dec.
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.
兰斯·亚当斯综合征(LAS)的特征是由脑缺氧导致的慢性动作性或意向性肌阵挛。吡仑帕奈是一种α-氨基-3-羟基-5-甲基-4-异恶唑丙酸谷氨酸受体的非竞争性拮抗剂,已在肌阵挛性癫痫和其他类型的肌阵挛中显示出一定疗效。我们报告了一名接受吡仑帕奈辅助治疗的LAS患者取得显著疗效,并对相关文献进行了综述。在我们的病例中,一名30多岁的男性患者被发现无脉搏,心跳停止时间不明。该患者在心脏骤停后1周内出现缺氧后肌阵挛。尽管接受了溴吡斯的明、丙戊酸和氯硝西泮治疗,患者仍持续遭受难治性肌阵挛。吡仑帕奈被添加到他的药物治疗方案中,并在1-2周内逐渐滴定至每日12毫克。这导致肌阵挛的频率和严重程度在大约6个月内有显著改善。越来越多的证据表明,吡仑帕奈可作为缺氧后肌阵挛或LAS患者的辅助治疗药物。对现有文献(包括病例报告和病例系列)的综述表明,吡仑帕奈在LAS患者中可能发挥作用。有必要进行进一步研究,包括对吡仑帕奈用于缺氧后肌阵挛的对照试验。