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Research Article| Volume 21, ISSUE 3, P291-295, July 2011

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Autism in Dravet syndrome: Prevalence, features, and relationship to the clinical characteristics of epilepsy and mental retardation

  • Author Footnotes
    1 Co-first author.
    Bing-Mei Li
    Footnotes
    1 Co-first author.
    Affiliations
    Institute of Neuroscience and the Second Affiliated Hospital of Guangzhou Medical University and Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China, Guangzhou 510260, China
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  • Author Footnotes
    1 Co-first author.
    Xiao-Rong Liu
    Footnotes
    1 Co-first author.
    Affiliations
    Institute of Neuroscience and the Second Affiliated Hospital of Guangzhou Medical University and Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China, Guangzhou 510260, China
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  • Yong-Hong Yi
    Affiliations
    Institute of Neuroscience and the Second Affiliated Hospital of Guangzhou Medical University and Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China, Guangzhou 510260, China
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  • Yu-Hong Deng
    Affiliations
    Institute of Neuroscience and the Second Affiliated Hospital of Guangzhou Medical University and Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China, Guangzhou 510260, China
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  • Tao Su
    Affiliations
    Institute of Neuroscience and the Second Affiliated Hospital of Guangzhou Medical University and Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China, Guangzhou 510260, China
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  • Xin Zou
    Affiliations
    Institute of Neuroscience and the Second Affiliated Hospital of Guangzhou Medical University and Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China, Guangzhou 510260, China
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  • Wei-Ping Liao
    Correspondence
    Corresponding author. Fax: +86 20 34153378.
    Affiliations
    Institute of Neuroscience and the Second Affiliated Hospital of Guangzhou Medical University and Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China, Guangzhou 510260, China
    Search for articles by this author
  • Author Footnotes
    1 Co-first author.

      Abstract

      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.

      Research highlights

      • The prevalence and features of autism in patients with Dravet syndrome are assessed.
      • The majority of patients with Dravet syndrome exhibit some symptoms of autistic spectrum disorder.
      • Patients with Dravet syndrome with autism exhibit more severe mental retardation.
      • This is the first clinical study demonstrating autism in Dravet syndrome.

      Keywords

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