{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T12:51:58Z","timestamp":1788871918807,"version":"build-2803163510"},"reference-count":23,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2013,7,12]],"date-time":"2013-07-12T00:00:00Z","timestamp":1373587200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Biocodex"},{"DOI":"10.13039\/100004336","name":"Novartis","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100004336","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Cyberonics"},{"name":"Glaxo"},{"name":"UCB"},{"DOI":"10.13039\/100004319","name":"Pfizer","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100004319","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Cubist"},{"name":"Athena"},{"DOI":"10.13039\/501100013327","name":"Lundbeck","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100013327","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2013,9]]},"abstract":"Summary<\/jats:title>\n \n Purpose<\/jats:title>\n \n To review the efficacy and tolerability of stiripentol in the treatment of\n U<\/jats:styled-content>\n .\n S<\/jats:styled-content>\n . children with\n D<\/jats:styled-content>\n ravet syndrome.\n <\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n U<\/jats:styled-content>\n .\n S<\/jats:styled-content>\n . clinicians who had prescribed stiripentol for two or more children with\n D<\/jats:styled-content>\n ravet syndrome between March 2005 and 2012 were contacted to request participation in this retrospective study. Data collected included overall seizure frequency, frequency of prolonged seizures, and use of rescue medications and emergency room (\n ER<\/jats:styled-content>\n )\/hospital visits in the year preceding stiripentol initiation, and with stiripentol therapy. We separately assessed efficacy in the following treatment groups:\n g<\/jats:styled-content>\n roup\n A<\/jats:styled-content>\n , stiripentol without clobazam or valproate;\n g<\/jats:styled-content>\n roup\n B<\/jats:styled-content>\n , stiripentol with clobazam but without valproate;\n g<\/jats:styled-content>\n roup\n C<\/jats:styled-content>\n , stiripentol with valproate but without clobazam; and\n g<\/jats:styled-content>\n roup\n D<\/jats:styled-content>\n , stiripentol with clobazam and valproate. In addition, adverse effects were recorded.\n <\/jats:p>\n <\/jats:sec>\n \n Key Findings<\/jats:title>\n \n Thirteen of 16 clinicians contacted for study participated and provided data on 82 children. Stiripentol was initiated a median of 6.0\u00a0years after seizure onset and 1.2\u00a0years after diagnosis of\n D<\/jats:styled-content>\n ravet syndrome. Compared to baseline, overall seizure frequency was reduced in 2\/6 in\n g<\/jats:styled-content>\n roup\n A<\/jats:styled-content>\n , 28\/35 in\n g<\/jats:styled-content>\n roup\n B<\/jats:styled-content>\n , 8\/14 in\n g<\/jats:styled-content>\n roup\n C<\/jats:styled-content>\n , and 30\/48 in\n g<\/jats:styled-content>\n roup\n D<\/jats:styled-content>\n . All children with prolonged seizure frequency greater than quarterly during the baseline period experienced a reduction in this frequency on the various treatment arms with stiripentol. Similarly, 2\/4 patients in\n g<\/jats:styled-content>\n roup\n A<\/jats:styled-content>\n , 25\/25 in\n g<\/jats:styled-content>\n roup\n B<\/jats:styled-content>\n , 5\/10 in\n g<\/jats:styled-content>\n roup\n C<\/jats:styled-content>\n , and 26\/33 in\n g<\/jats:styled-content>\n roup\n D<\/jats:styled-content>\n experienced reduction in frequency of rescue medication use and 1\/1 in\n g<\/jats:styled-content>\n roup\n A<\/jats:styled-content>\n , 12\/12 in\n g<\/jats:styled-content>\n roup\n B<\/jats:styled-content>\n , 3\/5 in\n g<\/jats:styled-content>\n roup\n C<\/jats:styled-content>\n , and 18\/19 in group\n D<\/jats:styled-content>\n had reduction in frequency of\n ER<\/jats:styled-content>\n \/hospital visits. Adverse effects were reported in 38, most commonly sedation and reduced appetite. Four patients (5%) discontinued stiripentol for adverse effects and two (2%) for lack of efficacy.\n <\/jats:p>\n <\/jats:sec>\n \n Significance<\/jats:title>\n \n Stiripentol is an effective and well\u2010tolerated therapy that markedly reduced frequency of prolonged seizures in\n D<\/jats:styled-content>\n ravet syndrome.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1111\/epi.12303","type":"journal-article","created":{"date-parts":[[2013,7,12]],"date-time":"2013-07-12T08:58:55Z","timestamp":1373619535000},"page":"1595-1604","source":"Crossref","is-referenced-by-count":95,"title":["Stiripentol in\n D<\/scp>\n ravet syndrome: Results of a retrospective\n U<\/scp>\n .\n S<\/scp>\n . study"],"prefix":"10.1111","volume":"54","author":[{"given":"Elaine C.","family":"Wirrell","sequence":"first","affiliation":[{"name":"Divisions of Child and Adolescent Neurology Department of Neurology Mayo Clinic Rochester Minnesota U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda","family":"Laux","sequence":"additional","affiliation":[{"name":"Division of Neurology Department of Pediatrics Northwestern University Feinberg School of Medicine Lurie Children's Hospital Chicago Illinois U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David N.","family":"Franz","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics and Neurology University of Cincinnati and Cincinnati Children's Hospital Medical Center Cincinnati Ohio U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Sullivan","sequence":"additional","affiliation":[{"name":"Division of Neurology Department of Pediatrics UCSF San Francisco California U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Russell P.","family":"Saneto","sequence":"additional","affiliation":[{"name":"Division of Pediatric Neurology University of Washington and Seattle Children's Hospital Seattle Washington U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard P.","family":"Morse","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics and Neurology Geisel School of Medicine Dartmouth\u2010Hitchcock Medical Center Lebanon New Hampshire U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Orrin","family":"Devinsky","sequence":"additional","affiliation":[{"name":"Departments of Neurology, Neurosurgery and Psychiatry NYU School of Medicine New York New York U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harry","family":"Chugani","sequence":"additional","affiliation":[{"name":"Children's Hospital of Michigan Wayne State University Detroit Michigan U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angel","family":"Hernandez","sequence":"additional","affiliation":[{"name":"Division of Neurology Cook Children's Health Care System Forth Worth Texas U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorie","family":"Hamiwka","sequence":"additional","affiliation":[{"name":"Division of Neurology Department of Pediatrics Nationwide Children's Hospital and the Ohio State University College of Medicine Columbus Ohio U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohamad A.","family":"Mikati","sequence":"additional","affiliation":[{"name":"Division of Neurology Department of Pediatrics Duke University Medical Center Durham North Carolina U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ignacio","family":"Valencia","sequence":"additional","affiliation":[{"name":"Section of Neurology Departments of Pediatrics and Neurology Drexel University College of Medicine St Christopher's Hospital for Children Philadelphia Pennsylvania U.S.A"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie\u2010Emmanuelle","family":"Le 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