van den Munckhof Bart, van Dee Violet, Sagi Liora, Caraballo Roberto H, Veggiotti Pierangelo, Liukkonen Elina, Loddenkemper Tobias, Sánchez Fernández Iván, Buzatu Marga, Bulteau Christine, Braun Kees P J, Jansen Floor E
Department of Pediatric Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, The Netherlands.
Pediatric Neurology Unit, Dana Children's Hospital, Tel Aviv Medical Center, Tel Aviv, Israel.
Epilepsia. 2015 Nov;56(11):1738-46. doi: 10.1111/epi.13128. Epub 2015 Sep 4.
Epileptic encephalopathy with electrical status epilepticus in sleep (ESES) is a pediatric epilepsy syndrome with sleep-induced epileptic discharges and acquired impairment of cognition or behavior. Treatment of ESES is assumed to improve cognitive outcome. The aim of this study is to create an overview of the current evidence for different treatment regimens in children with ESES syndrome.
A literature search using PubMed and Embase was performed. Articles were selected that contain original treatment data of patients with ESES syndrome. Authors were contacted for additional information. Individual patient data were collected, coded, and analyzed using logistic regression analysis. The three predefined main outcome measures were improvement in cognitive function, electroencephalography (EEG) pattern, and any improvement (cognition or EEG).
The literature search yielded 1,766 articles. After applying inclusion and exclusion criteria, 112 articles and 950 treatments in 575 patients could be analyzed. Antiepileptic drugs (AEDs, n = 495) were associated with improvement (i.e., cognition or EEG) in 49% of patients, benzodiazepines (n = 171) in 68%, and steroids (n = 166) in 81%. Surgery (n = 62) resulted in improvement in 90% of patients. In a subgroup analysis of patients who were consecutively reported (585 treatments in 282 patients), we found improvement in a smaller proportion treated with AEDs (34%), benzodiazepines (59%), and steroids (75%), whereas the improvement percentage after surgery was preserved (93%). Possible predictors of improved outcome were treatment category, normal development before ESES onset, and the absence of structural abnormalities.
Although most included studies were small and retrospective and their heterogeneity allowed analysis of only qualitative outcome data, this pooled analysis suggests superior efficacy of steroids and surgery in encephalopathy with ESES.
睡眠中癫痫性电持续状态（ESES）相关的癫痫性脑病是一种小儿癫痫综合征，具有睡眠诱发的癫痫放电以及认知或行为获得性损害。ESES的治疗被认为可改善认知结局。本研究的目的是概述目前关于ESES综合征患儿不同治疗方案的证据。
使用PubMed和Embase进行文献检索。选取包含ESES综合征患者原始治疗数据的文章。与作者联系以获取更多信息。收集个体患者数据，进行编码，并使用逻辑回归分析进行分析。三个预定义的主要结局指标为认知功能改善、脑电图（EEG）模式以及任何改善（认知或EEG）。
文献检索共得到1766篇文章。应用纳入和排除标准后，可对来自112篇文章的575例患者的950次治疗进行分析。抗癫痫药物（AEDs，n = 495）使49%的患者获得改善（即认知或EEG），苯二氮䓬类药物（n = 171）使68%的患者获得改善，类固醇（n = 166）使81%的患者获得改善。手术（n = 62）使患者改善率达90%。在对连续报道患者的亚组分析中（282例患者的585次治疗），我们发现接受AEDs治疗的患者改善比例较小（34%），苯二氮䓬类药物治疗的患者改善比例为59%，类固醇治疗的患者改善比例为75%，而手术治疗后的改善比例保持不变（93%）。改善结局可能的预测因素为治疗类别、ESES发作前发育正常以及无结构异常。
尽管大多数纳入研究规模较小且为回顾性研究，且其异质性使得仅能分析定性结局数据，但这项汇总分析表明类固醇和手术在ESES相关脑病中疗效更佳。
