title: Autism in Dravet syndrome: prevalence, features, and relationship to the clinical characteristics of epilepsy and mental retardation.
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.
authors.affiliation: Institute of Neuroscience and Second Affiliated Hospital of Guangzhou Medical University and Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and Ministry of Education of China, Guangzhou 510260, China.
authors.forename: Bing-Mei
authors.initials: BM
authors.lastname: Li
authors.forename: Xiao-Rong
authors.initials: XR
authors.lastname: Liu
authors.forename: Yong-Hong
authors.initials: YH
authors.lastname: Yi
authors.forename: Yu-Hong
authors.initials: YH
authors.lastname: Deng
authors.forename: Tao
authors.initials: T
authors.lastname: Su
authors.forename: Xin
authors.initials: X
authors.lastname: Zou
authors.forename: Wei-Ping
authors.initials: WP
authors.lastname: Liao
mesh_terms: D000293:Adolescent; D001321:Autistic Disorder / Q000453:epidemiology*; D001921:Brain / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D004831:Epilepsies, Myoclonic / Q000150:complications* / Q000453:epidemiology*; D005260:Female; D006801:Humans; D007361:Intelligence Tests; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D003294:Seizures, Febrile / Q000150:complications* / Q000453:epidemiology*; D012720:Severity of Illness Index