Autism in Dravet syndrome: Prevalence, features, and relationship to the clinical characteristics of epilepsy and mental retardation
Autism in Dravet syndrome: Prevalence, features, and relationship to the clinical characteristics of epilepsy and mental retardation
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DOI：
10.1016/j.yebeh.2011.04.060
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发表时间：
2011-07-01
影响因子：
2.6
通讯作者：
Liao, Wei-Ping
中科院分区：
文献类型：
--
作者：
Li, Bing-Mei;Liu, Xiao-Rong;Liao, Wei-Ping
自闭症是一种普遍存在的发育障碍，经常与癫痫同时发生。德拉韦综合征是一种严重的癫痫脑病，与精神运动发育迟缓有关。然而，德拉维综合征中的自闭症很少被研究。本研究旨在探讨自闭症患者的患病率、自闭症的特点、自闭症与精神发育迟滞的关系以及癫痫的临床特点。收集37例Drave氏综合征患者的临床资料，并对其进行自闭症和智力低下评定。9名患者(24.3%)符合自闭症标准。所有自闭症患者均表现为言语延迟、无情感互惠、兴趣狭隘，而非自闭症患者言语延迟、脾气暴躁、兴趣狭窄的比例分别为89.3%、46.4%和39.9%。94.6%的Dravet综合征患者存在精神发育迟滞，其中重度或重度智力发育迟滞在自闭症患者中更为常见。自闭症患者与非自闭症患者癫痫的临床特征差异无统计学意义。(C)2011 Elsevier Inc.保留所有权利。
Autism is a pervasive developmental disorder that frequently co-occurs with epilepsy. Dravet syndrome is a severe epileptic encephalopathy associated with psychomotor developmental delay. Autism in Dravet syndrome, however, has rarely been studied. In this study, the prevalence and features of autism in patients with Dravet syndrome, their potential association with mental retardation, and the clinical characteristics of epilepsy were investigated. Clinical data of 37 patients with Dravet syndrome were collected, and evaluations of autism and mental retardation were performed. Nine patients (24.3%) met the criteria for autism. All patients with autism showed speech delay, no emotional reciprocity, and narrow interests, whereas 89.3, 46.4, and 39.9% of patients without autism had speech delay, short temper, and narrow interests, respectively. Mental retardation was observed in 94.6% of patients with Dravet syndrome, with more frequent severe or profound mental retardation in those with autism. The clinical features of epilepsy did not statistically differ between the patients with autism and the patients without autism. (C) 2011 Elsevier Inc. All rights reserved.