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.
- 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.
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- Autism and epilepsy: cause, consequence, comorbidity, or coincidence?.Epilepsy Behav. 2005; 7: 652-656
- Epilepsy in autism.Lancet Neurol. 2002; 1: 352-358
- Frequent association of autism spectrum disorder in patients with childhood onset epilepsy.Brain Dev. 2010; 32: 759-763
- Autism and tuberous sclerosis.J Child Neurol. 2004; 19: 675-679
- Proposal for revised classification of epilepsies and epileptic syndromes.Epilepsia. 1989; 30: 389-399
- Epidemiology of severe myoclonic epilepsy of infancy.Epilepsia. 1990; 31: 397-400
- Severe myoclonic epilepsy in infancy: a systematic review and a meta-analysis of individual patient data.Epilepsia. 2008; 49: 343-348
- Severe myoclonic epilepsy of infants (Dravet syndrome): natural history and neuropsychological findings.Epilepsia. 2006; 47: 45-48
- Fever, genes, and epilepsy.Lancet Neurol. 2004; 3: 421-430
- Severe myoclonic epilepsy in infancy (Dravet syndorme).in: Roger J. Bureau M. Dravet C. Genton P. Tassinari C.A. Wolf P. Epileptic syndromes in infancy, childhood and adolescence. John Libbey Eurotext, Montrouge2005: 89-113
- The spectrum of SCNIA-related infantile epileptic encephalopathies.Brain. 2007; 130: 843-852
- Regression in pervasive developmental disorders: seizures and epileptiform electroencephalogram correlates.Pediatrics. 1997; 99: 560-566
- Benefits of reducing prenatal exposure to coal-burning pollutants to children's neurodevelopment in China.Environ Health Perspect. 2008; 116: 1396-1400
- Children's Neuropsychological Tests.2nd ed. Shanghai Scientific and Technological Publ. Co., Shanghai1987
- Epilepsy in young adults with autism: a prospective population-based follow-up study of 120 individuals diagnosed in childhood.Epilepsia. 2005; 46: 918-923
- Convulsing toward the pathophysiology of autism.Brain Dev. 2009; 31: 95-103
- Childhood autism and associated comorbidities.Brain Dev. 2007; 29: 257-272
- CDKL5 and ARX mutations are not responsible for early onset severe myoclonic epilepsy in infancy.Epilepsy Res. 2009; 87: 25-30
- Clinical features of epilepsy with pervasive developmental disorder.Brain Dev. 2010; 32: 764-768
- New trends in developmental disorders:etiology, clinical features and rehabilitation.in: Presented at the 378th meeting of the Fukuoka Pediatric Society. July 23 2008
- Epilepsy in autism is associated with intellectual disability and gender: evidence from a meta-analysis.Biol Psychiatry. 2008; 64: 577-582
- Psychiatric disorders in children and adolescents with mental retardation and active epilepsy.Arch Neurol. 1996; 53: 904-912
- Autistic and dysphasic children: II: Epilepsy.Pediatrics. 1991; 88: 1219-1225
- The genetics of autistic disorders and its clinical relevance: a review of the literature.Mol Psychiatry. 2007; 12: 2-22
- Sodium channels SCN1A, SCN2A and SCN3A in familial autism.Mol Psychiatry. 2003; 8: 186-194
- Epilepsy and autism spectrum disorders: are there common developmental mechanisms?.Brain Dev. 2010; 32: 731-738
Accepted: April 21, 2011
Received in revised form: April 20, 2011
Received: January 22, 2011
© 2011 Elsevier Inc. Published by Elsevier Inc. All rights reserved.