{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T15:20:09Z","timestamp":1790522409341,"version":"4.1.0"},"reference-count":42,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2018,12,20]],"date-time":"2018-12-20T00:00:00Z","timestamp":1545264000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia Open"],"published-print":{"date-parts":[[2019,3]]},"abstract":"Summary<\/jats:title>\n \n Objective<\/jats:title>\n \n We aimed to assess a cohort of young patients with Dravet syndrome (\n DS<\/jats:styled-content>\n ) for intellectual disability (\n ID<\/jats:styled-content>\n ) and autism spectrum disorder (\n ASD<\/jats:styled-content>\n ) using standardized tools and parental questionnaires to delineate their specific profiles.\n <\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n We included 35 patients with\n DS<\/jats:styled-content>\n aged 24\u00a0months to 7\u00a0years, excluding patients with a developmental age (\n DA<\/jats:styled-content>\n )\u00a0<18\u00a0months (n\u00a0=\u00a05). We performed specific tests adapted for\n ID<\/jats:styled-content>\n (Psychoeducational Profile, Third Edition [\n PEP<\/jats:styled-content>\n \u20103]), in addition to the Child Development Inventory (\n CDI<\/jats:styled-content>\n ) and Vineland Adaptive Behavior Scales, Second Edition (\n VABS<\/jats:styled-content>\n \u2010\n II<\/jats:styled-content>\n ) questionnaires. We used 2 standardized tools for\n ASD<\/jats:styled-content>\n : the Autism Diagnostic Observation Schedule, Second Edition (\n ADOS<\/jats:styled-content>\n \u20102) and the Autism Diagnostic Interview\u2010Revised (\n ADI<\/jats:styled-content>\n \u2010R). We compared the with parental questionnaires and the\n VABS<\/jats:styled-content>\n \u2010\n II<\/jats:styled-content>\n , and with\n ASD<\/jats:styled-content>\n characteristics.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n PEP<\/jats:styled-content>\n \u20103 subscales showed pathologic development in all but one patient (97%):\n ID<\/jats:styled-content>\n in 23 of 30 (77%), and borderline cognitive functioning in 6 of 30 (22%). Eleven patients (39%) had\n ASD<\/jats:styled-content>\n and 2 (7%) had a Social Communication Disorder (\n SCD<\/jats:styled-content>\n ) diagnosis. We found no difference between\n PEP<\/jats:styled-content>\n \u20103 and\n CDI<\/jats:styled-content>\n categorization except for fine motor skills. We found significant negative correlations between\n ADOS<\/jats:styled-content>\n \u20102 and\n PEP<\/jats:styled-content>\n \u20103 for the majority of scores. For patients aged older than 50\u00a0months, 2 groups emerged (\n ASD<\/jats:styled-content>\n \/no\n ASD<\/jats:styled-content>\n ) with significant difference in\n DA<\/jats:styled-content>\n . The logistic regression for\n ASD<\/jats:styled-content>\n diagnosis explained by\n VABS<\/jats:styled-content>\n \u2010\n II<\/jats:styled-content>\n showed a significant effect for Socialization, Motor Skills, and Adaptive Behavior.\n <\/jats:p>\n <\/jats:sec>\n \n Significance<\/jats:title>\n \n We found a high prevalence of\n ID<\/jats:styled-content>\n in patients with\n DS<\/jats:styled-content>\n .\n ID<\/jats:styled-content>\n is characterized by expressive and comprehensive communication deficits in addition to visuospatial difficulties.\n ASD<\/jats:styled-content>\n showed a specific profile with a relative preservation of social skills, emphasizing a possible underdiagnosis. Parental questionnaires can provide a good assessment of cognitive profile and might allow the difficulty of addressing cognitive scales in\n DS to be overcome<\/jats:styled-content>\n . The profile of\n ID<\/jats:styled-content>\n and\n ASD<\/jats:styled-content>\n should help to establish early adapted rehabilitation programs and emphasizes the global need for care beyond seizures in\n DS<\/jats:styled-content>\n and other developmental epileptic encephalopathies.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1002\/epi4.12281","type":"journal-article","created":{"date-parts":[[2018,10,29]],"date-time":"2018-10-29T15:20:10Z","timestamp":1540826410000},"page":"40-53","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":69,"title":["Autism spectrum disorder and cognitive profile in children with Dravet syndrome: Delineation of a specific phenotype"],"prefix":"10.1002","volume":"4","author":[{"given":"Lisa","family":"Ouss","sequence":"first","affiliation":[{"name":"Reference Centre for Rare Epilepsies Department of Pediatric Neurology Necker Enfants Malades Hospital APHP Paris Descartes University Imagine Institute Paris France"},{"name":"Child Psychiatry Unit Necker Enfants Malades Hospital Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dorothee","family":"Leunen","sequence":"additional","affiliation":[{"name":"Reference Centre for Rare Epilepsies Department of Pediatric Neurology Necker Enfants Malades Hospital APHP Paris Descartes University Imagine Institute Paris France"},{"name":"Child Psychiatry Unit Necker Enfants Malades Hospital Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacques","family":"Laschet","sequence":"additional","affiliation":[{"name":"Reference Centre for Rare Epilepsies Department of Pediatric Neurology Necker Enfants Malades Hospital APHP Paris Descartes University Imagine Institute Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Chemaly","sequence":"additional","affiliation":[{"name":"Reference Centre for Rare Epilepsies Department of Pediatric Neurology Necker Enfants Malades Hospital APHP Paris Descartes University Imagine Institute Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Barcia","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics Necker Enfants Malades Hospital APHP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emma M.","family":"Losito","sequence":"additional","affiliation":[{"name":"Reference Centre for Rare Epilepsies Department of Pediatric Neurology Necker Enfants Malades Hospital APHP Paris Descartes University Imagine Institute Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aveline","family":"Aouidad","sequence":"additional","affiliation":[{"name":"Department of Child and Adolescent Psychiatry APHP Piti\u00e9\u2010Salp\u00eatri\u00e8re Hospital Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zoe","family":"Barrault","sequence":"additional","affiliation":[{"name":"Reference Centre for Rare Epilepsies Department of Pediatric Neurology Necker Enfants Malades Hospital APHP Paris Descartes University Imagine Institute Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Desguerre","sequence":"additional","affiliation":[{"name":"Reference Centre for Rare Epilepsies Department of Pediatric Neurology Necker Enfants Malades Hospital APHP Paris Descartes University Imagine Institute Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Delphine","family":"Breuillard","sequence":"additional","affiliation":[{"name":"Reference Centre for Rare Epilepsies Department of Pediatric Neurology Necker Enfants Malades Hospital APHP Paris Descartes University Imagine Institute Paris 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