{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T00:08:07Z","timestamp":1792195687349,"version":"4.4.0"},"reference-count":51,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T00:00:00Z","timestamp":1769990400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T00:00:00Z","timestamp":1769990400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Dravet syndrome (DS) is the prototypic developmental and epileptic encephalopathy, characterized by drug\u2010resistant seizures, developmental slowing, and many other morbidities. Detailed characterization of behavioral phenotypes and social\u2013emotional skill development are limited.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      We prospectively assessed behavioral and social\u2013emotional problems in the ENVISION natural history study (NCT04537832) of children with DS associated with\n                      <jats:italic>SCN1A<\/jats:italic>\n                      pathogenic variants (\n                      <jats:italic>SCN1A<\/jats:italic>\n                      + DS) enrolled at &lt;5\u2009years of age. Assessments every 3\u2009months for up to 2\u2009years included the Child Behavior Checklist (CBCL), Strengths and Difficulties Questionnaire (SDQ), Brief Infant\u2010Toddler Social and Emotional Assessment (BITSEA), and Vineland Adaptive Behavior Scales, 3rd Edition (VABS\u20103).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Fifty\u2010eight children with DS enrolled at 16 sites worldwide. Problematic behaviors\u2014inattention, aggressive or oppositional behaviors, and withdrawn and autistic behaviors\u2014began before age 3\u2009years, became more evident with age, and were clinically significant in many children by age 3\u20134\u2009years. CBCL Total, Externalizing, and Internalizing Problems T\u2010scores rose by approximately 3 points per year, and BITSEA Problem scale scores increased by 2.3 points annually (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u2009.002). SDQ Hyperactivity scores worsened over time (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u2009.013), whereas emotional difficulties remained stable. VABS\u20103 Socialization domain scores, which were already more than 1 SD below the normative mean at baseline, decreased further, particularly in younger participants. Correlation analyses showed that poorer communication abilities were associated with increased problematic behaviors (\n                      <jats:italic>R<\/jats:italic>\n                      \u2009=\u2009\u2212.55 for CBCL Total Problems and VABS\u20103 Communication scores). Mixed\u2010effects modeling identified age as the strongest predictor of worsening behavioral outcomes.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Significance<\/jats:title>\n                    <jats:p>We found that behavioral and social\u2013emotional problems are inherent components of DS that present in toddlerhood and worsen throughout early childhood. This highlights the need to diagnose and manage these issues early. Targeted therapy may alleviate the wide\u2010ranging morbidities that are intrinsic to DS, including the social\u2013emotional and behavior problems that frequently emerge.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/epi.70127","type":"journal-article","created":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T17:14:16Z","timestamp":1770052456000},"page":"2463-2479","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Characterizing early behavioral and social\u2013emotional problems in young children with\n                    <scp>\n                      <i>SCN1A<\/i>\n                    <\/scp>\n                    + Dravet syndrome: Findings from the\n                    <scp>ENVISION<\/scp>\n                    prospective natural history study"],"prefix":"10.1002","volume":"67","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2311-2174","authenticated-orcid":false,"given":"Ingrid E.","family":"Scheffer","sequence":"first","affiliation":[{"name":"Epilepsy Research Centre University of Melbourne, Austin Health  Heidelberg Victoria Australia"},{"name":"Florey and Murdoch Children's Research Institutes  Melbourne Victoria Australia"},{"name":"Department of Paediatrics University of Melbourne, Royal Children's Hospital  Parkville Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1825-846X","authenticated-orcid":false,"given":"M. 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