Koichihara Reiko, Hamano Shin-Ichiro, Daida Atsuro, Nonoyama Hazuki, Ikemoto Satoru, Hirata Yuko, Matsuura Ryuki
Division of Neurology, Saitama Children's Medical Center,, Department for Child Health and Human Development, Saitama Children's Medical Center 2-1 Shin-toshin, Chuo-ku, Saitama-city, Saitama, Japan.
Division of Neurology, Saitama Children's Medical Center.
Epileptic Disord. 2020 Feb 1;22(1):73-82. doi: 10.1684/epd.2020.1130.
This study aimed to investigate the general presentation of epileptic spasms without hypsarrhythmia (ESwoH) and retrospectively determine whether there are differences in treatment effects related to ACTH therapy, long-term seizure outcome, and evolution of EEG features according to pre-treatment EEG patterns. According to the pattern of background activity, we divided our cohort into two groups: Group 1: normal background activity or with localized intermittent slow waves; Group 2: intermittent slow waves appearing generalized or in two or more lobes. Subjects included 22 children (Group 1: n=10; Group 2: n=12) diagnosed with ESwoH who received treatment from 2007 to 2017. The median age at onset of epileptic spasms was 5.5 months and the follow-up period lasted for 40.5 months. ACTH therapy was performed for seven patients from Group 1 and eight patients from Group 2. Only one patient from Group 2 responded to ACTH. Patients receiving effective treatments at early stages had excellent seizure outcome. Refractory cases included six patients in Group 1 and eight patients in Group 2; subsequent follow-up EEGs indicated hypsarrhythmia in one patient in Group 1 (17%) and six patients (75%) in Group 2, including one patient whose EEG pattern indicated progression to Lennox-Gastaut syndrome. Overall, ACTH is ineffective for patients with epileptic spasms without hypsarrhythmia. The EEG may indicate possible future development of hypsarrhythmia if epileptic spasms are resistant to treatment, especially in patients with diffuse slow waves on pre-treatment EEG. The efficacy of treatment introduced at early stages from onset may predict long-term seizure outcome.
本研究旨在调查无高峰失律的癫痫性痉挛（ESwoH）的一般表现，并回顾性确定根据治疗前脑电图模式，在促肾上腺皮质激素（ACTH）治疗的疗效、长期癫痫发作结局以及脑电图特征演变方面是否存在差异。根据背景活动模式，我们将队列分为两组：第1组：背景活动正常或伴有局限性间歇性慢波；第2组：间歇性慢波呈广泛性出现或在两个或更多脑叶出现。研究对象包括22例在2007年至2017年期间接受治疗的被诊断为ESwoH的儿童（第1组：n = 10；第2组：n = 12）。癫痫性痉挛发作的中位年龄为5.5个月，随访期持续40.5个月。第1组7例患者和第2组8例患者接受了ACTH治疗。第2组仅1例患者对ACTH有反应。早期接受有效治疗的患者癫痫发作结局良好。难治性病例包括第1组6例患者和第2组8例患者；随后的随访脑电图显示，第1组1例患者（17%）和第2组6例患者（75%）出现高峰失律，其中1例患者的脑电图模式显示进展为Lennox-Gastaut综合征。总体而言，ACTH对无高峰失律的癫痫性痉挛患者无效。如果癫痫性痉挛对治疗耐药，脑电图可能提示高峰失律未来可能的发展，尤其是在治疗前脑电图有弥漫性慢波的患者中。从发作起早期引入的治疗疗效可能预测长期癫痫发作结局。
