特发性全身性癫痫(IGE)和进行性肌阵挛性癫痫(PME)可发生癫痫肌阵挛性或肌阵挛性癫痫发作。然而,在PME和Lance-Adams综合征中常见的症状性肌阵挛症是刺激敏感的,由运动引起。症状性肌阵鸣并不总是与脑电图上的癫痫样放电有关。治疗性干预措施,如抗癫痫药物(ASM),生酮饮食和迷走神经刺激并不总是有效的。有新的证据表明,perampanel(PER),α-氨基-3-羟基-5-甲基-4-异恶唑丙酸(AMPA)受体拮抗剂,可能是有效的治疗肌阵挛性癫痫和症状性肌阵挛症。我们对文献进行了系统回顾,以评估PER治疗肌阵挛性癫痫和症状性肌阵挛症的疗效。纳入了27项研究,总样本量为260名患者。分别分析了PER对肌阵挛性癫痫发作和症状性肌阵挛症的疗效。在肌阵挛性癫痫发作的组中,50%的响应者,75%的反应者和癫痫发作自由率报告为74.3%(101/136),60.3%(82/136)和57.4%(78/136),分别,随访6-12个月。然而,在对IGE患者数据的事后分析中,与安慰剂相比,在双盲期,PER治疗肌阵挛性癫痫发作的疗效无显著差异.在总共119例患者中报告了PER对症状性肌阵挛症的疗效。四项研究(n=88例患者)报道了PER的疗效,表现为肌阵鸣评分/量表的降低。在其余31名患者中,有症状的肌阵鸣在三名患者中得到解决,21例患者下降,7例患者无改善。我们还分析了在开始PER时已经使用左乙拉西坦(LEV)或丙戊酸(VPA)的患者人数;这些数据可用于153名患者。其中,当开始PER时,56.8%的人在LEV上,75.1%的人在VPA上。这项系统评价表明,PER可能可有效治疗耐药性肌阵挛性癫痫发作和症状性肌阵挛症。它对已经对LEV和VPA没有反应的患者也可能有效。这些发现是初步的,但令人鼓舞。这项研究有几个局限性,特别是考虑到缺乏高质量的随机对照试验以及研究类型和结果的明显异质性.因此,这项审查的结果应该有相当大的保留。
Epileptic myoclonus or myoclonic seizures can occur in idiopathic generalized epilepsy (IGE) and progressive myoclonus epilepsy (PME). However, symptomatic myoclonus which is stimulus-sensitive and provoked by movement is typically seen in PME and Lance-Adams syndrome. Symptomatic myoclonus is not always associated with epileptiform discharges on the electroencephalogram. Therapeutic interventions such as anti-seizure medications (ASMs), the ketogenic diet and vagus nerve stimulation are not always effective. There is emerging evidence that perampanel (PER), an α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor antagonist, may be effective for the treatment of myoclonic seizures and symptomatic myoclonus. We performed a systematic
review of the literature to assess the efficacy of PER as treatment for myoclonic seizures and symptomatic myoclonus. Twenty-seven studies with a total sample size of 260 patients were included. The efficacy of PER was analysed separately for myoclonic seizures and symptomatic myoclonus. In the group with myoclonic seizures, 50% responder, 75% responder and seizure freedom rates were reported as 74.3% (101/ 136), 60.3% (82/136) and 57.4% (78/136), respectively, with a follow-up duration of 6-12 months. However, in one post-hoc analysis of data from patients with IGE, the efficacy of PER as treatment for myoclonic seizures during the double-blind phase showed no significant difference compared to placebo. The efficacy of PER for symptomatic myoclonus was reported in a total of 119 patients. Four studies (n=88 patients) reported the efficacy of PER as a decrease in myoclonus score/scale. In the remaining 31 patients, symptomatic myoclonus resolved in three patients, decreased in 21 patients and seven patients showed no improvement. We also analysed the number of patients who were already on levetiracetam (LEV) or valproic acid (VPA) at the time of PER initiation; these data were available for 153 patients. Of these, 56.8% were on LEV and 75.1% were on VPA when PER was initiated. This systematic
review suggests that PER maybe effective as treatment for drug-resistant myoclonic seizures and symptomatic myoclonus. It may also be effective in patients who have already failed to respond to LEV and VPA. These findings are preliminary yet encouraging. This study has several limitations, particularly given the scarcity of high-quality randomized controlled trials and marked heterogeneity regarding the type and results of the studies. Hence, the findings of this
review should be viewed with considerable reservation.