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伦诺克斯-加斯托综合征。管理更新。

Lennox-Gastaut syndrome. Management update.

作者信息

Al-Banji Muradi H, Zahr Doaa K, Jan Mohammed M

机构信息

Department of Pediatrics, King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia.

出版信息

Neurosciences (Riyadh). 2015 Jul;20(3):207-12. doi: 10.17712/nsj.2015.3.20140677.

Abstract

Lennox-Gastaut syndrome (LGS) is a severe pediatric epilepsy syndrome characterized by mixed seizures, cognitive decline, and generalized slow (<3 Hz) spike wave discharges on electroencephalography. Atonic seizures result in dangerous drop attacks with risks of injury and impairment of the quality of life. The seizures are frequently resistant to multiple antiepileptic (AED) drugs. Newer AEDs, such as rufinamide, are now available. When multiple AED trials fail, non-pharmacological treatments such as the ketogenic diet, vagus nerve stimulation, and epilepsy surgery, should be considered. The aim of this review is to present an updated outline of LGS and the available treatments. Although the prognosis for complete seizure control remains poor, the addition of newer therapies provides an improved hope for some of these patients and their families. Further long term randomized controlled trials are required to compare different therapeutic interventions in terms of efficacy and tolerability.

摘要

伦诺克斯 - 加斯托综合征(LGS)是一种严重的儿童癫痫综合征,其特征为混合性发作、认知功能下降以及脑电图上的广泛性慢波(<3Hz)棘波放电。失张力发作会导致危险的跌倒发作,存在受伤风险并损害生活质量。这些发作常常对多种抗癫痫药物(AED)耐药。新型抗癫痫药物,如卢非酰胺,现已可用。当多次抗癫痫药物试验失败时,应考虑采用生酮饮食、迷走神经刺激和癫痫手术等非药物治疗方法。本综述的目的是呈现LGS及现有治疗方法的最新概述。尽管完全控制癫痫发作的预后仍然很差,但新疗法的加入为部分此类患者及其家庭带来了更大的希望。需要进一步进行长期随机对照试验,以比较不同治疗干预措施在疗效和耐受性方面的差异。

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