Sulthiame use in children with pharmacoresistant epilepsies: A retrospective study

IF 16.4 1区化学 Q1 CHEMISTRY, MULTIDISCIPLINARY

Accounts of Chemical Research Pub Date : 2024-05-31 DOI:10.1002/epd2.20250

Alexandra Laliberté, Saoussen Berrahmoune, Kenneth A. Myers

{"title":"Sulthiame use in children with pharmacoresistant epilepsies: A retrospective study","authors":"Alexandra Laliberté, Saoussen Berrahmoune, Kenneth A. Myers","doi":"10.1002/epd2.20250","DOIUrl":null,"url":null,"abstract":"<div>\n \n \n <section>\n \n <h3> Objective</h3>\n \n <p>This retrospective study aimed to assess the efficacy and tolerability of sulthiame as an add-on treatment in children with pharmacoresistant epilepsies.</p>\n </section>\n \n <section>\n \n <h3> Methods</h3>\n \n <p>All patients with epilepsy who received sulthiame at Montreal Children's Hospital over an 11-year period were included. Medical charts were reviewed, and extracted data included patient age and sex, seizure types, epilepsy syndrome, electroencephalography (EEG) reports, brain imaging reports, antiseizure treatments trialed, starting and final dose of sulthiame, duration of sulthiame treatment, adverse events attributed to sulthiame, and seizure frequency before and after sulthiame treatment. EEG studies were also analyzed and spike–wave index (SWI) in the first 10 min of sleep was calculated.</p>\n </section>\n \n <section>\n \n <h3> Results</h3>\n \n <p>Sixteen patients were included, all of whom had pharmacoresistant epilepsies (mean of 9.9 trials of other antiseizure treatments). Six had genetic diagnoses, four had in utero/perinatal acquired brain injury, one had a suspected focal cortical dysplasia, and five were idiopathic. Ten patients had developmental and epileptic encephalopathy with spike–wave activation in sleep, three had Lennox–Gastaut syndrome, and one each had sleep-related hyperkinetic epilepsy, self-limited epilepsy with centrotemporal spikes, and mixed generalized and multifocal epilepsy. Of the 12 patients with uncontrolled seizures at the time of sulthiame initiation, 4 had improvement in seizure frequency, including 2 who became seizure free. Eight patients had EEG data available that allowed calculation of sleep SWI; from this group, SWI decreased from 81.1% +/− 17.6% to 45.1% +/− 36.5% (<i>p</i> = .007). The most common adverse events reported were somnolence/drowsiness, aggression, and increased seizure frequency. Of the patients with genetic etiologies, significant positive responses were seen in patients with pathogenic variants in <i>NDUFS1</i> and <i>SATB1</i>.</p>\n </section>\n \n <section>\n \n <h3> Significance</h3>\n \n <p>These data demonstrate the therapeutic potential of sulthiame, even in patients with highly pharmacoresistant epilepsy. Improvements may be seen in both seizure frequency and sleep SWI.</p>\n </section>\n </div>","PeriodicalId":1,"journal":{"name":"Accounts of Chemical Research","volume":null,"pages":null},"PeriodicalIF":16.4000,"publicationDate":"2024-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epd2.20250","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Accounts of Chemical Research","FirstCategoryId":"3","ListUrlMain":"https://onlinelibrary.wiley.com/doi/10.1002/epd2.20250","RegionNum":1,"RegionCategory":"化学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CHEMISTRY, MULTIDISCIPLINARY","Score":null,"Total":0}

引用次数: 0

Abstract

Objective

This retrospective study aimed to assess the efficacy and tolerability of sulthiame as an add-on treatment in children with pharmacoresistant epilepsies.

Methods

All patients with epilepsy who received sulthiame at Montreal Children's Hospital over an 11-year period were included. Medical charts were reviewed, and extracted data included patient age and sex, seizure types, epilepsy syndrome, electroencephalography (EEG) reports, brain imaging reports, antiseizure treatments trialed, starting and final dose of sulthiame, duration of sulthiame treatment, adverse events attributed to sulthiame, and seizure frequency before and after sulthiame treatment. EEG studies were also analyzed and spike–wave index (SWI) in the first 10 min of sleep was calculated.

Results

Sixteen patients were included, all of whom had pharmacoresistant epilepsies (mean of 9.9 trials of other antiseizure treatments). Six had genetic diagnoses, four had in utero/perinatal acquired brain injury, one had a suspected focal cortical dysplasia, and five were idiopathic. Ten patients had developmental and epileptic encephalopathy with spike–wave activation in sleep, three had Lennox–Gastaut syndrome, and one each had sleep-related hyperkinetic epilepsy, self-limited epilepsy with centrotemporal spikes, and mixed generalized and multifocal epilepsy. Of the 12 patients with uncontrolled seizures at the time of sulthiame initiation, 4 had improvement in seizure frequency, including 2 who became seizure free. Eight patients had EEG data available that allowed calculation of sleep SWI; from this group, SWI decreased from 81.1% +/− 17.6% to 45.1% +/− 36.5% (p = .007). The most common adverse events reported were somnolence/drowsiness, aggression, and increased seizure frequency. Of the patients with genetic etiologies, significant positive responses were seen in patients with pathogenic variants in NDUFS1 and SATB1.

Significance

These data demonstrate the therapeutic potential of sulthiame, even in patients with highly pharmacoresistant epilepsy. Improvements may be seen in both seizure frequency and sleep SWI.

Abstract Image

查看原文本刊更多论文

药物耐药性癫痫患儿使用舒利迭的情况：一项回顾性研究。

研究目的这项回顾性研究旨在评估舒利迭作为药物耐药性癫痫患儿的附加治疗药物的疗效和耐受性：方法：纳入蒙特利尔儿童医院在 11 年间接受舒利迭治疗的所有癫痫患者。对病历进行了审查，提取的数据包括患者的年龄和性别、癫痫发作类型、癫痫综合征、脑电图（EEG）报告、脑成像报告、试用过的抗癫痫治疗方法、舒硫安的起始剂量和最终剂量、舒硫安治疗的持续时间、舒硫安引起的不良事件以及舒硫安治疗前后的癫痫发作频率。此外，还对脑电图研究进行了分析，并计算了睡眠前 10 分钟的尖波指数（SWI）：共纳入 16 名患者，他们都患有药物耐药性癫痫（平均接受过 9.9 次其他抗癫痫治疗）。其中六人有遗传诊断，四人有子宫内/围产期获得性脑损伤，一人有疑似局灶性皮质发育不良，五人是特发性癫痫。10名患者患有发育性癫痫性脑病并伴有睡眠中的尖波激活，3名患者患有伦诺克斯-加斯塔特综合征，与睡眠相关的高运动性癫痫、伴有中心颞区尖波的自限性癫痫以及全身性和多灶性混合癫痫各1名。在开始接受舒思安治疗时发作未受控制的 12 名患者中，有 4 人的发作频率有所改善，其中 2 人已不再发作。八名患者的脑电图数据可用于计算睡眠SWI；该组患者的SWI从81.1% +/- 17.6%降至45.1% +/- 36.5%（p = .007）。最常见的不良反应是嗜睡、攻击性和癫痫发作频率增加。在有遗传病因的患者中，NDUFS1和SATB1致病变体患者出现了显著的阳性反应：这些数据证明了舒利迭的治疗潜力，即使是对药物高度耐药的癫痫患者也不例外。癫痫发作频率和睡眠SWI均可得到改善。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

求助全文

约1分钟内获得全文求助全文

来源期刊

Accounts of Chemical Research 化学-化学综合

CiteScore

31.40

自引率

1.10%

发文量

312

审稿时长

2 months

期刊介绍： Accounts of Chemical Research presents short, concise and critical articles offering easy-to-read overviews of basic research and applications in all areas of chemistry and biochemistry. These short reviews focus on research from the author’s own laboratory and are designed to teach the reader about a research project. In addition, Accounts of Chemical Research publishes commentaries that give an informed opinion on a current research problem. Special Issues online are devoted to a single topic of unusual activity and significance. Accounts of Chemical Research replaces the traditional article abstract with an article "Conspectus." These entries synopsize the research affording the reader a closer look at the content and significance of an article. Through this provision of a more detailed description of the article contents, the Conspectus enhances the article's discoverability by search engines and the exposure for the research.