Pediatric epilepsy syndromes.

Pediatric epilepsy syndromes.
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小儿癫痫综合征。

DOI:
10.1097/00008480-199512000-00003
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发表时间:
1995
影响因子:
3.6
通讯作者:
Glauser,TA
Glauser,TA
中科院分区:
医学3区
文献类型:
--
作者:
Glauser,TA

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癫痫和癫痫综合征国际分类的发展使人们对癫痫患者的认识和治疗取得了实质性进展。癫痫和癫痫综合征首先按癫痫发作类型,然后按病因划分。本文介绍了六个主要的小儿癫痫综合征的最新进展。前三个讨论(良性儿童癫痫与中央颞区棘波,青少年肌阵挛性癫痫,儿童失神癫痫)是三个最常见的癫痫综合征,每一个都是重点的重要研究。良性儿童癫痫伴中央颞区棘波(良性罗兰癫痫)患者可能不需要抗惊厥治疗,并且他们的癫痫发作通常在青春期中期缓解。青少年肌阵挛性癫痫是第一个发现基因位点的主要癫痫综合征,儿童失神癫痫的广泛失神发作的神经通路和细胞机制已被很好地阐明。最后讨论的三种综合征(West综合征、Lennox-Gastaut综合征和获得性癫痫性失语症)是毁灭性的灾难性癫痫综合征。West综合征(婴儿痉挛)的研究重点是开发更好的治疗干预措施(包括医疗和手术),并提高对其发病机制的理解。新的有效药物可用于治疗Lennox-Gastaut综合征(儿童癫痫性脑病)患者,但其中一种药物(非氨酯)与之前未被认识到的严重副作用有关。最后,获得性癫痫性失语症是一个例子,一个鲜为人知的癫痫综合征。一个多中心的小组已经组织起来重新审查这种综合征,并产生更好的了解其发病机制和治疗。
: The development of an international classification for epilepsies and epileptic syndromes has led to substantial progress in the understanding and treatment of patients with epilepsy. Epilepsies and epileptic syndromes are partitioned first by seizure type and then by etiology. This manuscript describes recent advances in six major pediatric epilepsy syndromes. The first three discussed (benign childhood epilepsy with centrotemporal spikes, juvenile myoclonic epilepsy, and childhood absence epilepsy) are the three most common epilepsy syndromes, and each has been the focus of significant research. Patients with benign childhood epilepsy with centrotemporal spikes (benign rolandic epilepsy) may not require anticonvulsant therapy, and their seizures always remit by midadolescence. Juvenile myoclonic epilepsy was the first major epilepsy syndrome for which a gene locus was found. The underlying neuronal pathways and cellular mechanisms of the generalized absence seizures of childhood absence epilepsy are well delineated. The last three syndromes discussed (West syndrome, Lennox-Gastaut syndrome, and acquired epileptic aphasia) are devastating catastrophic epileptic syndromes. The focus of research in West syndrome (infantile spasms) has been to develop better therapeutic interventions (both medical and surgical) and an improved understanding of its pathogenesis. New effective medications are available for patients with Lennox-Gastaut syndrome (childhood epileptic encephalopathy), but one of them (felbamate) has been associated with previously unrecognized serious side effects. Lastly, acquired epileptic aphasia is an example of a poorly understood epilepsy syndrome. A multicenter group has been organized to reexamine this syndrome and produce a better understanding of its pathogenesis and treatment.
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