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从伴中央颞区棘波的儿童良性癫痫到Landau-Kleffner综合征的谱系:中央颞区脑电图特征对认知的影响。

The spectrum from BCECTS to LKS: The Rolandic EEG trait-impact on cognition.

作者信息

Stephani Ulrich, Carlsson Goran

机构信息

University Children's Hospital, Department of Neuropediatrics, Kiel, Germany.

出版信息

Epilepsia. 2006;47 Suppl 2:67-70. doi: 10.1111/j.1528-1167.2006.00694.x.

Abstract

The laboratory hallmark of BCECTS is the rolandic discharge (RD) in the EEG of patients, occurring in a characteristic topographical, vigilance-related, event-related, and age-related pattern, disappearing during puberty. RDs are present in 2% of healthy children. About 8% of children with RDs have epilepsy. An increased prevalence rate of RDs is found in children with cognitive and behavioral disorders, with headaches and some genetic syndromes. In some patients, the cognitive disorders are transient but in others they are progressive, resulting in stable mental retardation after puberty. A recent study of 36 BCECTS patients addressed the following questions. (1) the possible relationship between the severity of RDs and the neuropsychological deficits; (2) the profile of neuropsychological deficits; (3) changes of cognition related to EEG changes; and (4) effects of therapy. No correlation was found between global IQ and the severity of the RDs. All the children had at least one specific learning disorder (sometimes long-lasting). When the children were treated, a correlation between cognitive and EEG improvement could not be demonstrated. Recently, 21 patients without epilepsy but with attention deficit and hyperactivity and/or learning disorders were studied: an open treatment trial with sulthiame resulted in improved sustained and selective attention. The neurobiology of RDs and their relationship to cognitive dysfunction and epilepsy requires further study.

摘要

儿童良性中央颞区癫痫(BCECTS)的实验室特征是患者脑电图中的中央颞区放电(RD),其出现具有特征性的地形、与警觉性相关、与事件相关以及与年龄相关的模式,在青春期消失。RDs在2%的健康儿童中存在。约8%有RDs的儿童患有癫痫。在患有认知和行为障碍、头痛以及一些遗传综合征的儿童中,RDs的患病率有所增加。在一些患者中,认知障碍是短暂的,但在另一些患者中则是进行性的,导致青春期后出现稳定的智力迟钝。最近一项针对36例BCECTS患者的研究探讨了以下问题:(1)RDs的严重程度与神经心理缺陷之间的可能关系;(2)神经心理缺陷的概况;(3)与脑电图变化相关的认知变化;以及(4)治疗效果。未发现总体智商与RDs的严重程度之间存在相关性。所有儿童至少有一种特定的学习障碍(有时持续时间较长)。当对儿童进行治疗时,无法证明认知改善与脑电图改善之间存在相关性。最近,对21例无癫痫但有注意力缺陷多动障碍和/或学习障碍的患者进行了研究:一项关于舒噻美(sulthiame)的开放治疗试验使持续和选择性注意力得到改善。RDs的神经生物学及其与认知功能障碍和癫痫的关系需要进一步研究。

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