{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T12:47:01Z","timestamp":1793191621359,"version":"4.5.0"},"reference-count":28,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2020,11,23]],"date-time":"2020-11-23T00:00:00Z","timestamp":1606089600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Sciences"],"abstract":"<jats:p>Dravet Syndrome (DS) is burdened by high epilepsy-related premature mortality due to status epilepticus (SE). We surveyed centres within Europe through the Dravet Italia Onlus and EpiCARE network (European Reference Network for Rare and Complex Epilepsies). We collated responses on seven DS SCN1A+ patients who died following refractory SE (mean age 6.9 year, range 1.3\u201323.4 year); six were on valproate, clobazam, and stiripentol. All patients had previous SE. Fatal SE was always triggered by fever: either respiratory infection or one case of hexavalent vaccination. SE lasted between 80 min and 9 h and all patients received IV benzodiazepines. Four patients died during or within hours of SE; in three patients, SE was followed by coma with death occurring after 13\u201360 days. Our survey supports the hypothesis that unresponsive fever is a core characteristic feature of acute encephalopathy. We highlight the need for management protocols for prolonged seizures and SE in DS.<\/jats:p>","DOI":"10.3390\/brainsci10110889","type":"journal-article","created":{"date-parts":[[2020,11,23]],"date-time":"2020-11-23T08:18:23Z","timestamp":1606119503000},"page":"889","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Fatal Status Epilepticus in Dravet Syndrome"],"prefix":"10.3390","volume":"10","author":[{"given":"Paola","family":"De Liso","sequence":"first","affiliation":[{"name":"Department of Neuroscience, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, Full Member of European Reference Network EpiCARE, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Virginia","family":"Pironi","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, Full Member of European Reference Network EpiCARE, 00165 Rome, Italy"},{"name":"Center for Rare Diseases and Birth Defects, Department of Woman and Child Health, Institute of Pediatrics, Policlinico Universitario Gemelli Foundation, Catholic University of Rome, 00168 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Massimo","family":"Mastrangelo","sequence":"additional","affiliation":[{"name":"Paediatric Neurology Unit, V. Buzzi Hospital, A.O. ICP, 20019 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Domenica","family":"Battaglia","sequence":"additional","affiliation":[{"name":"Department of Child Neurology and Psychiatry, Policlinico Universitario Gemelli Foundation, Catholic University of Rome, 00153 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dana","family":"Craiu","sequence":"additional","affiliation":[{"name":"Department of Neurology, Paediatric Neurology, Psychiatry, Neurosurgery, \u201cCarol Davila\u201d University of Medicine of Bucharest, Full Member of European Reference Network EpiCARE, 050474 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marina","family":"Trivisano","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, Full Member of European Reference Network EpiCARE, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8120-0287","authenticated-orcid":false,"given":"Nicola","family":"Specchio","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, Full Member of European Reference Network EpiCARE, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rima","family":"Nabbout","sequence":"additional","affiliation":[{"name":"Centre for Rare Epilepsies, Department of Paediatric Neurology, Necker-Enfants Malades Hospital, Imagine Institute, INSERMU1163, Paris Descartes University, Full Member of European Reference Network EpiCARE, 75006 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federico","family":"Vigevano","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Bambino Ges\u00f9 Children\u2019s Hospital, IRCCS, Full Member of European Reference Network EpiCARE, 00165 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,11,23]]},"reference":[{"key":"ref_1","unstructured":"Dravet, C., Oguni, H., Cokar, O., and Guerrini, R. 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