{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T04:17:58Z","timestamp":1789100278665,"version":"build-2803163510"},"reference-count":23,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,11,30]],"date-time":"2025-11-30T00:00:00Z","timestamp":1764460800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2025,11,30]],"date-time":"2025-11-30T00:00:00Z","timestamp":1764460800000},"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 Open"],"published-print":{"date-parts":[[2026,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Dravet syndrome (DS) is a severe developmental and epileptic encephalopathy with a high seizure burden and mortality risk. Stiripentol, one of the first DS\u2010specific therapies, received FDA approval in 2018 but its real\u2010world use and impact post\u2010approval in the USA remain insufficiently characterized. The STIRUS study aimed to assess treatment patterns, clinical efficacy, and quality of life outcomes associated with stiripentol in a contemporary US cohort.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>STIRUS was a retrospective, multicenter chart review of 98 DS patients who initiated stiripentol after FDA approval and were treated for at least 3\u2009months. Data were collected from 10 USA epilepsy centers on seizure types and frequency, status epilepticus (SE), rescue medication use, emergency room visits, and quality of life, at baseline and during the first and final 3\u2009months on stiripentol. Changes over time were assessed using generalized estimating equation models.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Initiation of stiripentol was associated with a significant reduction in bilateral convulsive seizure frequency (OR\u2009=\u20092.16,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.006) and a nearly 50% decrease in SE episodes (OR\u2009=\u20092.9,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.003). Importantly, there was a marked decline in rescue medication use and seizure\u2010related hospital visits: the proportion of patients requiring no rescue medications increased from 23% to over 53%, and those needing frequent emergency interventions dropped from 39% at baseline to 14% during the final 3\u2009months. Quality of life improved in more than half of patients and caregivers.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Significance<\/jats:title>\n                    <jats:p>The STIRUS study supports the real\u2010world efficacy of stiripentol in reducing seizure burden and healthcare utilization in DS. Despite its demonstrated benefits and approval for use in infants, stiripentol remains underutilized and often introduced late in the treatment course. Early and broader adoption may help improve clinical outcomes and quality of life in this vulnerable population.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Plain Language Summary<\/jats:title>\n                    <jats:p>The STIRUS study found that stiripentol, an anti\u2010seizure medication specifically approved for Dravet Syndrome, helped reduce seizures and hospital visits for children living with this severe form of epilepsy. Patients had fewer convulsive seizures, needed fewer rescue medicines, and reported better quality of life. Despite these benefits, stiripentol is still not widely used and often started too late in treatment, suggesting earlier use could further improve outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/epi4.70191","type":"journal-article","created":{"date-parts":[[2025,12,1]],"date-time":"2025-12-01T08:00:26Z","timestamp":1764576026000},"page":"200-210","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Stiripentol use in Dravet syndrome patients in the\n                    <scp>USA<\/scp>\n                    : Results of a real\u2010world study"],"prefix":"10.1002","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3015-8282","authenticated-orcid":false,"given":"Elaine","family":"Wirrell","sequence":"first","affiliation":[{"name":"Divisions of Child and Adolescent Neurology and Epilepsy, Department of Neurology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4735-3431","authenticated-orcid":false,"given":"James","family":"Wheless","sequence":"additional","affiliation":[{"name":"Pediatric Neurology University of Tennessee Health Science Center  Memphis Tennessee USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1825-846X","authenticated-orcid":false,"given":"Michael Scott","family":"Perry","sequence":"additional","affiliation":[{"name":"Cook Children's Medical Center Jane and John Justin Institute for Mind Health  Fort Worth Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda","family":"Laux","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Pediatrics Ann &amp; Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine  Chicago Illinois USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-3585-5529","authenticated-orcid":false,"given":"Karen C.","family":"Keough","sequence":"additional","affiliation":[{"name":"Austin Dell Children's Medical Center  Austin Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie","family":"Ziobro","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Michigan Medicine  Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1235-0708","authenticated-orcid":false,"given":"Aimee F.","family":"Luat","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Central Michigan University  Mt Pleasant Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gewalin","family":"Aungaroon","sequence":"additional","affiliation":[{"name":"Cincinnati Children's Hospital Medical Center University of Cincinnati  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael A.","family":"Ciliberto","sequence":"additional","affiliation":[{"name":"University of Iowa Hospitals and Clinics  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roxane","family":"Noel","sequence":"additional","affiliation":[{"name":"Biocodex Research Center  Compi\u00e8gne France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-3771-9451","authenticated-orcid":false,"given":"Laurent","family":"Chancharme","sequence":"additional","affiliation":[{"name":"Biocodex Research Center  Compi\u00e8gne France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Sullivan","sequence":"additional","affiliation":[{"name":"Department of Neurology University of California San Francisco  San Francisco California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,11,30]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.17239"},{"issue":"2","key":"e_1_2_11_3_1","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1111\/j.1528-1167.2011.03011.x","article-title":"Comprehensive care of children with Dravet syndrome","volume":"52","author":"Granata 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