{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T00:49:29Z","timestamp":1787014169281,"version":"build-2736575974"},"reference-count":36,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2018,8,22]],"date-time":"2018-08-22T00:00:00Z","timestamp":1534896000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2018,9]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>\n                      To evaluate continuing stiripentol treatment in adulthood in Dravet syndrome (\n                      <jats:styled-content style=\"fixed-case\">DS<\/jats:styled-content>\n                      ).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Method<\/jats:title>\n                    <jats:p>\n                      Longitudinal data were collected from the last visit prior to age 15\u00a0years (V\n                      <jats:sub>15\u00a0y<\/jats:sub>\n                      ) to the last visit in adulthood (V\n                      <jats:sub>adult<\/jats:sub>\n                      ) in the 40\n                      <jats:styled-content style=\"fixed-case\">DS<\/jats:styled-content>\n                      patients (32 typical, eight atypical) of a French historical cohort (Paris) of subjects who continued stiripentol from childhood or adolescence to adulthood.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      At V\n                      <jats:sub>adult<\/jats:sub>\n                      (18\u201040\u00a0years, median = 23\u00a0years), all the patients were still receiving stiripentol (exposure = 3\u201024\u00a0years, median = 18\u00a0years), associated with clobazam (40\/40), valproate (39\/40), and topiramate (21\/40). Between V\n                      <jats:sub>15\u00a0y<\/jats:sub>\n                      and V\n                      <jats:sub>adult<\/jats:sub>\n                      , stiripentol was interrupted in five patients (two for adverse events) but reintroduced following seizure aggravation. Loss of appetite affected 15 of 40 patients but resolved after reducing the dose of stiripentol or valproate; no other new stiripentol\u2010related adverse events were reported. Mean stiripentol dose was progressively decreased from 39 to 25\u00a0mg\/kg\/d (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.0002), whereas clobazam (0.27\u00a0mg\/kg\/d) and valproate (14\u00a0mg\/kg\/d) remained stable. At V\n                      <jats:sub>adult<\/jats:sub>\n                      , 37 of 40 patients still had generalized tonic\u2013clonic seizures, but none still had status epilepticus (vs three at V\n                      <jats:sub>15\u00a0y<\/jats:sub>\n                      ) and only one had myoclonia. During adulthood, generalized tonic\u2013clonic seizure frequency and duration continued to decrease (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.02,\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.008) and 10 patients experienced seizure\u2010free periods\u00a0\u2265 1\u00a0years (up to 5\u00a0years). All patients already had intellectual disability at V\n                      <jats:sub>15\u00a0y<\/jats:sub>\n                      , but retardation was more severe at V\n                      <jats:sub>adult<\/jats:sub>\n                      (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.03). Furthermore, neurological\/gait condition had declined (two patients became bedridden) and behavior had worsened (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0&lt;\u00a00.0002). Nevertheless, the 33 patients on stiripentol from infancy\/childhood (&lt;15\u00a0years) tended to have better seizure outcome in midadulthood than the seven treated from adolescence (&gt;15\u00a0years) and the\n                      <jats:styled-content style=\"fixed-case\">DS<\/jats:styled-content>\n                      patients treated from adult age or stiripentol\u2010naive subjects reported in the literature.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Significance<\/jats:title>\n                    <jats:p>The efficacy and safety of the stiripentol\/valproate\/clobazam combination started at pediatric age are maintained at very long term during adulthood. Such prolonged stiripentol therapy tends to positively impact the late prognosis of epilepsy, especially when initiated before adolescence.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/epi.14536","type":"journal-article","created":{"date-parts":[[2018,8,22]],"date-time":"2018-08-22T12:32:08Z","timestamp":1534941128000},"page":"1705-1717","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":53,"title":["Do children with Dravet syndrome continue to benefit from stiripentol for long through adulthood?"],"prefix":"10.1111","volume":"59","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9096-1694","authenticated-orcid":false,"given":"Catherine","family":"Chiron","sequence":"first","affiliation":[{"name":"Department of Pediatric Neurology Inserm U1129 Necker\u2010Enfants Malades Hospital Paris France"},{"name":"Reference Center for Rare Epilepsies APHP Necker\u2010Enfants Malades Hospital Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Helias","sequence":"additional","affiliation":[{"name":"ESAT le Val (Center of Readaptation for Adults) Mortagne\u2010au\u2010Perche France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Kaminska","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology Inserm U1129 Necker\u2010Enfants Malades Hospital Paris France"},{"name":"Reference Center for Rare Epilepsies APHP Necker\u2010Enfants Malades Hospital Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cecile","family":"Laroche","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Mother and Child Hospital Limoges France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2006-631X","authenticated-orcid":false,"given":"Bertrand","family":"de Toffol","sequence":"additional","affiliation":[{"name":"Department of Neurology Bretonneau Hospital Tours France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Dulac","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology Inserm U1129 Necker\u2010Enfants Malades Hospital Paris France"},{"name":"Reference Center for Rare Epilepsies APHP Necker\u2010Enfants Malades Hospital Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rima","family":"Nabbout","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology Inserm U1129 Necker\u2010Enfants Malades Hospital Paris France"},{"name":"Reference Center for Rare Epilepsies APHP Necker\u2010Enfants Malades Hospital Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"An","sequence":"additional","affiliation":[{"name":"Reference Center for Rare Epilepsies APHP Necker\u2010Enfants Malades Hospital Paris France"},{"name":"Department of Neurology APHP Pitie\u2010Salpetriere Hospital Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,8,22]]},"reference":[{"key":"e_1_2_9_2_1","unstructured":"Diacomit. 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