Management of and prophylaxis against status epilepticus in children with severe myoclonic epilepsy in infancy (SMEI; Dravet syndrome) - A nationwide questionnaire survey in Japan
Management of and prophylaxis against status epilepticus in children with severe myoclonic epilepsy in infancy (SMEI; Dravet syndrome) - A nationwide questionnaire survey in Japan
复制标题
DOI：
10.1016/j.braindev.2008.03.002
复制
发表时间：
2008-11-01
影响因子：
1.7
通讯作者：
Maekawa, Kihei
中科院分区：
文献类型：
--
作者：
Tanabe, Takuya;Awaya, Yutaka;Maekawa, Kihei
本研究的目的是建立策略，以预防癫痫持续状态（SE）与高热和持续SE的管理与婴儿期严重肌阵挛性癫痫（SMEI）的儿童。研究方法：这项调查是使用问卷进行回顾性调查的，问卷询问了药物，并分发给日本各地的癫痫专家。所有受访者都是日本癫痫学会（JES）和/或日本儿童神经病学学会（JSCN）的成员。本研究使用了109例SMEI患者（51例男性和58例女性）的数据，年龄为1-37（M +/- SD，10.7 +/- 6.53）岁。在这109例患者中，10例因未发生SE而被排除，因此分析了99例患者的数据。结果如下：在每日使用的抗癫痫药物（AED）中，以下药物对SE进展的疗效极佳：溴化钾（KBr）（41.7%）、唑尼沙胺（ZNS）（13.5%）、氯巴占（CLB）（10.0%）、丙戊酸盐（VPA）（8.0%）、苯巴比妥（PB）（6.7%）和苯妥英（PHT）（2.6%）。氯硝西泮（CZP）或卡马西平（CBZ）均未获得良好疗效。地西泮栓剂是预防发热所致SE最常用的药物，但仅有2例（2.4%）效果良好。以下药物在终止持续SE方面获得了极好的疗效：静脉注射巴比妥类药物（75-100%）、静脉注射咪达唑仑（MDZ）（68.8%）、静脉注射DZP（54.3%）、静脉注射利多卡因（Lid）（21.4%）和静脉注射PHT（15.4%）。结论：每日KBr对控制SMEI患者的癫痫发作最有效。早期静脉注射巴比妥类药物是阻止部分患者SE的最有效策略。预防性DZP、静脉注射苯二氮卓类药物（BZPs）、PHT和Lid在SE中未获得可靠疗效。(C)2008 Elsevier B. V.保留所有权利。
The aim of this study was to establish strategies for prophylaxis against status epilepticus (SE) associated with high fever and for management of ongoing SE in children with severe myoclonic epilepsy in infancy (SMEI). Methods: The investigation was performed retrospectively using a questionnaire, asking about medications, which was distributed to epilepsy specialists throughout Japan. All respondents were members of the Japan Epilepsy Society (JES) and/or the Japanese Society of Child Neurology (JSCN). Data from 109 SMEI patients (51 mates and 58 females), 1-37 (M +/- SD, 10.7 +/- 6.53) years old, were used for this study. Of these 109 patients, 10 were excluded because they had not experienced SE, such that data from 99 patients were analyzed. Results: Among the anti-epileptic drugs (AEDs) used daily, excellent efficacy against SE evolution was obtained with the following: potassium bromide (KBr) (41.7%,), zonisamide (ZNS) (13.5%), clobazam (CLB) (10.0%), valproate (VPA) (8.0%), phenobarbital (PB) (6.7%), and phenytoin (PHT)(2.6%). Excellent efficacy was not obtained with either clonazepam (CZP) or carbamazepine (CBZ). The diazepam (DZP) suppository was the most frequently given drug for prophylaxis against SE triggered by fever, but only 2 (2.4%) cases showed excellent results. Excellent efficacy in terminating ongoing SE was obtained with the following medications; intravenous barbiturates (75-100%), intravenous midazolam (MDZ) (68.8%), intravenous DZP (54.3%), intravenous lidocaine (Lid) (21.4%), and intravenous PHT (15.4%). Conclusions: Daily KBr was most efficacious for controlling seizures in SMEI patients. Early use of intravenous barbiturates is the most effective strategy in stopping SE in a subset of patients. Reliable efficacy in SE was not obtained with prophylactic DZP, intravenous benzodiazepines (BZPs), PHT and Lid. (C) 2008 Elsevier B.V. All rights reserved.