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儿童失神性癫痫的最新进展。

Current advances in childhood absence epilepsy.

机构信息

Department of Pediatrics, University "G. D'Annunzio" of Chieti, Chieti, Italy.

Department of Pediatrics, University of Perugia, Perugia, Italy.

出版信息

Pediatr Neurol. 2014 Mar;50(3):205-12. doi: 10.1016/j.pediatrneurol.2013.10.009. Epub 2013 Oct 23.

Abstract

BACKGROUND

Childhood absence epilepsy is an age-dependent, idiopathic, generalized epilepsy with a characteristic seizure appearance. The disorder is likely to be multifactorial, resulting from interactions between genetic and acquired factors, but the debate is still open. We review recent studies on different aspects of childhood absence epilepsy and also to describe new concepts.

METHODS

Data for this review were identified using Medline and PubMed survey to locate studies dealing with childhood absence epilepsy. Searches included articles published between 1924 and 2013.

RESULTS

The diagnosis comprises predominant and associated seizure types associated with other clinical and electroencephalographic characteristics. Many studies have challenged the prevailing concepts, particularly with respect to the pathophysiological mechanisms underlying the electroencephalographic seizure discharges. Childhood absence epilepsy fits the definition of system epilepsy as a condition resulting from the persisting susceptibility of the thalamocortical system as a whole to generate seizures. This syndrome, if properly defined using strict diagnostic criteria, has a good prognosis. In some cases, it may affect multiple cognitive functions determining risk for academic and functional difficulties; the detection of children at risk allows tailored interventions. Childhood absence epilepsy is usually treated with ethosuximide, valproate, lamotrigine, or levetiracetam, but the most efficacious and tolerable initial empirical treatment has not been well defined.

CONCLUSIONS

We review recent studies and new concepts on the electroclinical features and pathophysiological findings of childhood absence epilepsy in order to highlight areas of consensus as well as areas of uncertainty that indicate directions for future research.

摘要

背景

儿童失神癫痫是一种年龄依赖性、特发性、全面性癫痫,具有特征性发作表现。该疾病可能是多因素的,是遗传和获得性因素相互作用的结果,但争议仍未解决。我们回顾了儿童失神癫痫的不同方面的最新研究,并描述了新概念。

方法

本综述的数据通过 Medline 和 PubMed 检索来确定,以找到涉及儿童失神癫痫的研究。检索包括 1924 年至 2013 年发表的文章。

结果

该诊断包括主要和相关的发作类型,以及其他临床和脑电图特征。许多研究对流行的概念提出了挑战,特别是在脑电图发作放电的病理生理机制方面。儿童失神癫痫符合系统癫痫的定义,是整个丘脑皮质系统持续易感性导致癫痫发作的一种状态。如果使用严格的诊断标准来正确定义这种综合征,其预后良好。在某些情况下,它可能会影响多种认知功能,从而导致学习和功能困难的风险;对高危儿童的检测可以进行针对性的干预。儿童失神癫痫通常用乙琥胺、丙戊酸钠、拉莫三嗪或左乙拉西坦治疗,但尚未明确最有效和耐受的初始经验性治疗方法。

结论

我们回顾了儿童失神癫痫的电临床特征和病理生理学研究的最新进展和新概念,以突出共识领域以及表明未来研究方向的不确定领域。

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