{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,5]],"date-time":"2026-11-05T21:34:02Z","timestamp":1793914442210,"version":"4.8.0"},"reference-count":22,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2022,5,12]],"date-time":"2022-05-12T00:00:00Z","timestamp":1652313600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100009712","name":"Dravet Syndrome Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100009712","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2022,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>This study was undertaken to gain consensus from experienced physicians and caregivers regarding optimal diagnosis and management of Dravet syndrome (DS), in the context of recently approved, DS\u2010specific therapies and emerging disease\u2010modifying treatments.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A core working group was convened consisting of six physicians with recognized expertise in DS and two representatives of the Dravet Syndrome Foundation. This core group summarized the current literature (focused on clinical presentation, comorbidities, maintenance and rescue therapies, and evolving disease\u2010modifying therapies) and nominated the 31\u2010member expert panel (ensuring international representation), which participated in two rounds of a Delphi process to gain consensus on diagnosis and management of DS.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>There was strong consensus that infants 2\u201315\u00a0months old, presenting with either a first prolonged hemiclonic seizure or first convulsive status epilepticus with fever or following vaccination, in the absence of another cause, should undergo genetic testing for DS. Panelists agreed on evolution of specific comorbidities with time, but less agreement was achieved on optimal management. There was also agreement on appropriate first\u2010 to third\u2010line maintenance therapies, which included the newly approved agents. Whereas there was agreement for recommendation of disease\u2010modifying therapies, if they are proven safe and efficacious for seizures and\/or reduction of comorbidities, there was less consensus for when these should be started, with caregivers being more conservative than physicians.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Significance<\/jats:title>\n                    <jats:p>This International DS Consensus, informed by both experienced global caregiver and physician voices, provides a strong overview of the impact of DS, therapeutic goals and optimal management strategies incorporating the recent therapeutic advances in DS, and evolving disease\u2010modifying therapies.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/epi.17274","type":"journal-article","created":{"date-parts":[[2022,5,1]],"date-time":"2022-05-01T13:35:16Z","timestamp":1651412116000},"page":"1761-1777","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":256,"title":["International consensus on diagnosis and management of Dravet syndrome"],"prefix":"10.1111","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3015-8282","authenticated-orcid":false,"given":"Elaine C.","family":"Wirrell","sequence":"first","affiliation":[{"name":"Divisions of Child and Adolescent Medicine and Epilepsy Department of Neurology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1988-4980","authenticated-orcid":false,"given":"Veronica","family":"Hood","sequence":"additional","affiliation":[{"name":"Dravet Syndrome Foundation  Cherry Hill New Jersey USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1967-0827","authenticated-orcid":false,"given":"Kelly G.","family":"Knupp","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics and Neurology University of Colorado, Anschutz Campus  Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary Anne","family":"Meskis","sequence":"additional","affiliation":[{"name":"Dravet Syndrome Foundation  Cherry Hill New Jersey USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5877-4074","authenticated-orcid":false,"given":"Rima","family":"Nabbout","sequence":"additional","affiliation":[{"name":"Reference Center for Rare Epilepsies Department of Pediatric Neurology Necker\u2013Enfants Malades Hospital, Member of European Reference Network EpiCARE University of Paris  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2311-2174","authenticated-orcid":false,"given":"Ingrid E.","family":"Scheffer","sequence":"additional","affiliation":[{"name":"Austin Health and Royal Children\u2019s Hospital, Florey Institute of Neuroscience and Mental Health, Murdoch Children\u2019s Research Institute University of Melbourne  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7328-1796","authenticated-orcid":false,"given":"Jo","family":"Wilmshurst","sequence":"additional","affiliation":[{"name":"Department of Paediatric Neurology Red Cross War Memorial Children\u2019s Hospital, Neuroscience Institute University of Cape Town  Cape Town South Africa"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Sullivan","sequence":"additional","affiliation":[{"name":"Departments of Neurology and Pediatrics Benioff Children\u2019s Hospital University of California, San Francisco  San Francisco California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,5,12]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000003716"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2017.01.025"},{"issue":"3","key":"e_1_2_8_4_1","first-page":"S39","article-title":"Dravet syndrome: treatment options and management of prolonged seizures","volume":"60","author":"Cross JH","year":"2019","journal-title":"Epilepsia"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1611618"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(19)32500-0"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2019.4113"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(00)03157-3"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.aaz6100"},{"key":"e_1_2_8_10_1","unstructured":"YoungAN TanenhausA BelleA McLaughlinJ LiJ LinW.A GABA\u2010selective AAV vector upregulates endogenous SCN1A expression and reverses multiple phenotypes in a mouse model of Dravet syndrome. Paper presented at: American Epilepsy Society Annual Meeting; December2019; Baltimore Maryland."},{"key":"e_1_2_8_11_1","unstructured":"BelleA McLaughlinJ LiJ LuceyG ChouT.ETX101 a GABAergic interneuron selective AAV\u2010mediated gene therapy for the treatment of SCN1A+ Dravet syndrome: biodistribution and safety in non\u2010human primates. 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