Yamagishi Hirokazu, Osaka Hitoshi, Muramatsu Kazuhiro, Kojima Karin, Monden Yukifumi, Mitani Tadahiro, Asakura Yuta, Wakae Keizo, Nagai Kohei, Tajima Toshihiro
Department of Pediatrics, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke City, Tochigi, 329-0498, Japan.
Sci Rep. 2024 Dec 3;14(1):30051. doi: 10.1038/s41598-024-82014-5.
Seizures in patients with developmental and epileptic encephalopathies (DEEs) are often highly resistant to various antiseizure medications. Perampanel (PER) is a novel antiseizure medication that non-competitively inhibits the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor and is expected to reduce seizure frequency not only for focal seizures and generalized tonic-clonic seizures (GTCS) but also for other seizure types. This study aimed to clarify the long-term therapeutic efficacy and tolerability of PER in patients with DEEs. We analyzed data regarding patients' background characteristics, medication retention, trends in seizure frequency, and adverse events obtained from 24 patients with DEEs who had been on PER treatment for 60 months. The retention rates were 62.5% and 46.9% at 12 and 60 months, respectively. At 60 months after PER initiation, the rate of patients with > 50% seizure reduction was 33.3%, 33.3%, 38.5%, 54.5%, 54.5%, and 36.4% among patients with atypical absence seizures, tonic seizures, focal seizures, GTCS, myoclonic seizures, and atonic seizures, respectively. The frequency of adverse events was 70.8%. PER showed long-term efficacy in various seizure types. PER is a promising treatment option for patients with DEEs.
发育性和癫痫性脑病(DEE)患者的癫痫发作通常对各种抗癫痫药物具有高度耐药性。吡仑帕奈(PER)是一种新型抗癫痫药物,它非竞争性抑制α-氨基-3-羟基-5-甲基-4-异恶唑丙酸受体,不仅有望降低局灶性癫痫发作和全身强直阵挛性发作(GTCS)的发作频率,还能降低其他癫痫发作类型的频率。本研究旨在阐明PER对DEE患者的长期治疗效果和耐受性。我们分析了24例接受PER治疗60个月的DEE患者的背景特征、药物留存情况、癫痫发作频率趋势及不良事件的数据。12个月和60个月时的留存率分别为62.5%和46.9%。在开始使用PER后60个月时,非典型失神发作、强直发作、局灶性发作、GTCS、肌阵挛发作和失张力发作患者中癫痫发作减少>50%的比例分别为33.3%、33.3%、38.5%、54.5%、54.5%和36.4%。不良事件的发生率为70.8%。PER对各种癫痫发作类型均显示出长期疗效。对于DEE患者,PER是一种有前景的治疗选择。