{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,12]],"date-time":"2026-06-12T11:56:41Z","timestamp":1781265401618,"version":"3.54.1"},"reference-count":15,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,10,21]],"date-time":"2024-10-21T00:00:00Z","timestamp":1729468800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,10,21]],"date-time":"2024-10-21T00:00:00Z","timestamp":1729468800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/100008967","name":"Philipps-Universit\u00e4t Marburg","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100008967","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Neurol. Res. Pract."],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Status epilepticus (SE) is one of the most common neurological emergencies and an acutely life-threatening condition characterized by high mortality and morbidity. Despite the well-established staged therapy of status epilepticus, especially stages 1 and 2, more than one third of patients develop (super-) refractory SE. Despite a large variety of potential treatment options for super-refractory SE, there is an unmet clinical need of potential new treatment ideas in this often desperate clinical situation.<\/jats:p>\n                <jats:p>A number of studies have demonstrated the safety and efficacy of stiripentol (STP) in patients with Dravet syndrome (DS) and in children with focal epilepsy and generalized epilepsies. Some smaller series and case reports have documented the use of STP in the treatment of status epilepticus in adult patients.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>We retrospectively analyzed all patients who were admitted to the Department of Neurology at Marburg University Hospital between 2013 and 2023 with a diagnosis of (super)-refractory status epilepticus and who received additional treatment of SE with STP. All patients who received STP during the SE were included, regardless of previous medication.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>SE ceased in 64% of 25 patients (13 female and 12 male). The mean age was 58.6\u2009\u00b1\u200921.9\u00a0years (mean\u2009\u00b1\u2009SD). 72% had a structural epilepsy. In 20% of patients, SE was terminated by the administration of STP alone in 32% of cases, while in a further 32% of patients, the simultaneous administration of multiple anti-seizure medications (ASMs) including STP was potentially responsible for the cessation of the SE, with valproic acid (VPA), benzodiazepines and STP, being the most frequently implicated ASMs. In 12% of patients, there was at least a temporary improvement in the electroencephalogram (EEG). Stiripentol had to be discontinued in three cases due to a reduction in vigilance or hypercalcemia.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>Stiripentol may represent a promising additional treatment option for refractory and super-refractory status epilepticus. The tolerability of this treatment has already been demonstrated in previous studies, and was also reflected in these data. Further prospective investigation in larger patient populations are necessary to ascertain the efficacy of stiripentol in SE.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Trial registration<\/jats:title>\n                <jats:p>NCT06540378, retrospectively registered.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s42466-024-00348-x","type":"journal-article","created":{"date-parts":[[2024,10,21]],"date-time":"2024-10-21T01:01:46Z","timestamp":1729472506000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Stiripentol for the treatment of refractory status epilepticus"],"prefix":"10.1186","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6562-1196","authenticated-orcid":false,"given":"Leona","family":"M\u00f6ller","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ole J.","family":"Simon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clara","family":"J\u00fcnemann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meike","family":"Austermann-Menche","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc-Philipp","family":"Bergmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lena","family":"Habermehl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katja","family":"Menzler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lars","family":"Timmermann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam","family":"Strzelczyk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanne","family":"Knake","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,10,21]]},"reference":[{"issue":"s1","key":"348_CR1","doi-asserted-by":"publisher","first-page":"S37","DOI":"10.1684\/epd.2014.0670","volume":"16","author":"S Auvin","year":"2014","unstructured":"Auvin, S., & Dupuis, N. (2014). Outcome of status epilepticus. What do we learn from animal data? Epileptic Disorders, 16(s1), S37\u2013S43. https:\/\/doi.org\/10.1684\/epd.2014.0670","journal-title":"Epileptic Disorders"},{"issue":"3","key":"348_CR2","doi-asserted-by":"publisher","first-page":"451","DOI":"10.1007\/s40801-022-00305-7","volume":"9","author":"S Balestrini","year":"2022","unstructured":"Balestrini, S., Doccini, V., Boncristiano, A., Lenge, M., De Masi, S., & Guerrini, R. (2022). Efficacy and safety of long-term treatment with stiripentol in children and adults with drug-resistant epilepsies: A retrospective cohort study of 196 patients. Drugs\u2014Real World Outcomes, 9(3), 451\u2013461. https:\/\/doi.org\/10.1007\/s40801-022-00305-7","journal-title":"Drugs\u2014Real World Outcomes"},{"issue":"5","key":"348_CR3","doi-asserted-by":"publisher","first-page":"CD009887","DOI":"10.1002\/14651858.CD009887.pub5","volume":"5","author":"F Brigo","year":"2020","unstructured":"Brigo, F., Igwe, S. C., & Bragazzi, N. L. (2020). Stiripentol add-on therapy for drug-resistant focal epilepsy. Cochrane Database of Systematic Reviews, 5(5), CD009887. https:\/\/doi.org\/10.1002\/14651858.CD009887.pub5","journal-title":"Cochrane Database of Systematic Reviews"},{"issue":"9","key":"348_CR4","doi-asserted-by":"publisher","first-page":"CD009887","DOI":"10.1002\/14651858.CD009887.pub6","volume":"2022","author":"F Brigo","year":"2022","unstructured":"Brigo, F., Igwe, S. C., & Bragazzi, N. L. (2022). Stiripentol add-on therapy for drug-resistant focal epilepsy. Cochrane Database of Systematic Reviews, 2022(9), CD009887. https:\/\/doi.org\/10.1002\/14651858.CD009887.pub6","journal-title":"Cochrane Database of Systematic Reviews"},{"issue":"3","key":"348_CR5","doi-asserted-by":"publisher","first-page":"246","DOI":"10.1016\/S1474-4422(06)70374-X","volume":"5","author":"JW Chen","year":"2006","unstructured":"Chen, J. W., & Wasterlain, C. G. (2006). Status epilepticus: Pathophysiology and management in adults. The Lancet Neurology, 5(3), 246\u2013256. https:\/\/doi.org\/10.1016\/S1474-4422(06)70374-X","journal-title":"The Lancet Neurology"},{"key":"348_CR6","unstructured":"European Medicines Agency (Hg.) (2017): Diacomit. Online verf\u00fcgbar unter https:\/\/www.ema.europa.eu\/en\/medicines\/human\/EPAR\/diacomit."},{"issue":"s6","key":"348_CR7","doi-asserted-by":"publisher","first-page":"S103","DOI":"10.1111\/epi.12291","volume":"54","author":"DK Grosenbaugh","year":"2013","unstructured":"Grosenbaugh, D. K., & Mott, D. D. (2013). Stiripentol in refractory status epilepticus. Epilepsia, 54(s6), S103\u2013S105. https:\/\/doi.org\/10.1111\/epi.12291","journal-title":"Epilepsia"},{"key":"348_CR8","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1016\/j.seizure.2021.03.019","volume":"88","author":"L Habermehl","year":"2021","unstructured":"Habermehl, L., Mross, P. M., Krause, K., Immisch, I., Chiru, D., Zahnert, F., et al. (2021). Stiripentol in the treatment of adults with focal epilepsy- a retrospective analysis. Seizure, 88, 7\u201311. https:\/\/doi.org\/10.1016\/j.seizure.2021.03.019","journal-title":"Seizure"},{"key":"348_CR9","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1016\/j.eplepsyres.2017.03.009","volume":"133","author":"A-M Kantanen","year":"2017","unstructured":"Kantanen, A.-M., Reinikainen, M., Parviainen, I., & K\u00e4lvi\u00e4inen, R. (2017). Long-term outcome of refractory status epilepticus in adults: A retrospective population-based study. Epilepsy research, 133, 13\u201321. https:\/\/doi.org\/10.1016\/j.eplepsyres.2017.03.009","journal-title":"Epilepsy research"},{"issue":"6","key":"348_CR10","doi-asserted-by":"publisher","first-page":"604","DOI":"10.1001\/jamaneurol.2022.0609","volume":"79","author":"LE Roberg","year":"2022","unstructured":"Roberg, L. E., Monsson, O., Kristensen, S. B., Dahl, S. M., Ulvin, L. B., Heuser, K., Taub\u00f8ll, E., Strzelczyk, A., Knake, S., Bechert, L., Rosenow, F., Beier, D., Beniczky, S., Kr\u00f8ig\u00e5rd, T., & Beier, C. P. (2022). Prediction of long-term survival after status epilepticus using the ACD score. JAMA Neurology, 79(6), 604\u2013613. https:\/\/doi.org\/10.1001\/jamaneurol.2022.0609","journal-title":"JAMA Neurology"},{"issue":"11","key":"348_CR11","doi-asserted-by":"publisher","first-page":"2255","DOI":"10.1111\/epi.16363","volume":"60","author":"A Rosati","year":"2019","unstructured":"Rosati, A., Boncristiano, A., Doccini, V., Pugi, A., Pisano, T., Lenge, M., De Masi, S., & Guerrini, R. (2019). Long\u2010term efficacy of add\u2010on stiripentol treatment in children, adolescents, and young adults with refractory epilepsies: A single center prospective observational study. 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