{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T03:04:25Z","timestamp":1790996665958,"version":"4.1.0"},"reference-count":65,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2024,10,3]],"date-time":"2024-10-03T00:00:00Z","timestamp":1727913600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia Open"],"published-print":{"date-parts":[[2024,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:label\/><jats:p>Status epilepticus (SE) is a life\u2010threatening emergency with high morbidity and mortality. In people with epilepsy, the management of SE is focused on early medical treatment. Stiripentol is a third\u2010generation antiseizure medication (ASM) approved for refractory generalized tonic\u2013clonic seizures in Dravet syndrome. The aim of this systematic review was to evaluate the effectiveness and safety of stiripentol in reducing the incidence of SE in patients with Dravet syndrome or any epilepsy characterized by recurrent SE. The PubMed and Cochrane databases were systematically searched, and gray literature was hand\u2010searched. Search results were screened by title and abstract; studies with data on the effect of stiripentol on SE outcomes, including the cessation of SE, reduction in number of SE episodes, or reduction in hospitalizations, were included. Of 66 records identified, 17 studies were eligible for inclusion, of which 15 were human studies (<jats:italic>n<\/jats:italic>\u2009=\u2009474; aged 1.1\u201378\u2009years), and two were animal experiments. Results of retrospective or prospective observational studies showed that stiripentol as add\u2010on therapy to ASMs such as clobazam or valproate reduced the incidence of SE in patients with Dravet syndrome or other developmental and epileptic encephalopathies (DEEs). A mean of 68% of patients (range 41%\u2013100%) had a \u226550% reduction in SE episodes from baseline, and 26%\u2013100% of patients (mean 77%) became SE\u2010free after stiripentol initiation. Moreover, this review found stiripentol, used as acute treatment, may also be effective for the cessation of super\u2010refractory SE, but data are limited to three retrospective case series. Stiripentol was generally well\u2010tolerated. In conclusion, stiripentol reduces the incidence of SE episodes in patients with Dravet syndrome and potentially other DEEs, and it promotes cessation of super\u2010refractory SE in patients with and without a history of seizures.<\/jats:p><\/jats:sec><jats:sec><jats:title>Plain Language Summary<\/jats:title><jats:p>Status epilepticus (SE) is a life\u2010threatening, long\u2010lasting seizure occurring in patients with\/without epilepsy. This article analyzed 15 published studies that investigated the effects and safety of the anti\u2010seizure medication stiripentol for preventing SE in epilepsy patients (prevention) or stopping an SE episode (cessation), and two animal studies that investigated how stiripentol works. In epilepsy patients, stiripentol halved the number of SE episodes in 41\u2013100% of patients, 26\u2013100% of patients became SE\u2010free, and stiripentol was considered to be well tolerated. In patients with\/without epilepsy, stiripentol may stop the SE episode after other drugs like anesthetics have not worked.<\/jats:p><\/jats:sec>","DOI":"10.1002\/epi4.13036","type":"journal-article","created":{"date-parts":[[2024,10,3]],"date-time":"2024-10-03T10:43:31Z","timestamp":1727952211000},"page":"2017-2036","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Efficacy and safety of stiripentol in the prevention and cessation of status epilepticus: A systematic review"],"prefix":"10.1002","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8120-0287","authenticated-orcid":false,"given":"Nicola","family":"Specchio","sequence":"first","affiliation":[{"name":"Neurology, Epilepsy and Movement Disorders Unit, Bambino Ges\u00f9 Children's Hospital IRCCS, Member of ERN EpiCARE  Rome 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Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0928-1577","authenticated-orcid":false,"given":"Francesco","family":"Brigo","sequence":"additional","affiliation":[{"name":"Department of Neurology Hospital of Merano (SABES\u2010ASDAA)  Merano Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2080-8042","authenticated-orcid":false,"given":"Vicente","family":"Villanueva","sequence":"additional","affiliation":[{"name":"Refractory Epilepsy Unit, Neurology Service Hospital Universitario y Polit\u00e9cnico La Fe, Member of ERN EpiCARE  Valencia Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5950-2692","authenticated-orcid":false,"given":"Eugen","family":"Trinka","sequence":"additional","affiliation":[{"name":"Department of Neurology, Christian\u2010Doppler University Hospital, Centre for Cognitive Neuroscience Paracelsus Medical 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