{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,31]],"date-time":"2025-10-31T05:11:57Z","timestamp":1761887517630,"version":"build-2065373602"},"reference-count":14,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2023,3,1]],"date-time":"2023-03-01T00:00:00Z","timestamp":1677628800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2023,3,1]],"date-time":"2023-03-01T00:00:00Z","timestamp":1677628800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2022,11,12]],"date-time":"2022-11-12T00:00:00Z","timestamp":1668211200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.es","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Neurolog\u00eda (English Edition)"],"published-print":{"date-parts":[[2023,3]]},"DOI":"10.1016\/j.nrleng.2020.04.031","type":"journal-article","created":{"date-parts":[[2022,11,17]],"date-time":"2022-11-17T00:26:14Z","timestamp":1668644774000},"page":"82-86","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":0,"title":["Non-convulsive status epilepticus as the initial manifestation in a family with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)"],"prefix":"10.1016","volume":"38","author":[{"given":"F.","family":"Gonz\u00e1lez","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Bala","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Saucedo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L.","family":"Bandeo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.","family":"Pacio","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Chertcoff","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L.","family":"De Francesco","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L.","family":"Le\u00f3n Cejas","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.S.","family":"Pacha","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Uribe Roca","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"O.","family":"Mart\u00ednez","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Fern\u00e1ndez Pardal","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Reisin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P.","family":"Bonardo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"issue":"11","key":"10.1016\/j.nrleng.2020.04.031_bib0005","first-page":"1287","article-title":"Non-convulsive status epilepticus causing focal neurological deficits in CADASIL","volume":"78","author":"Valko","year":"2009","journal-title":"BMJ Case Rep"},{"issue":"2","key":"10.1016\/j.nrleng.2020.04.031_bib0010","doi-asserted-by":"crossref","first-page":"338","DOI":"10.1086\/381506","article-title":"Pathogenic mutations associated with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy differently affect jagged1 binding and NOTCH3 activity via the RBP\/JK signaling pathway","volume":"74","author":"Joutel","year":"2004","journal-title":"Am J Hum Genet"},{"issue":"5","key":"10.1016\/j.nrleng.2020.04.031_bib0015","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1016\/j.medcli.2009.10.034","article-title":"Fisiopato-log\u0131a del CADASIL","volume":"135","author":"Del R\u00edo Esp\u00ednola","year":"2010","journal-title":"Med Clin (Barc)"},{"issue":"5","key":"10.1016\/j.nrleng.2020.04.031_bib0020","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1136\/jnnp.2004.048207","article-title":"The spectrum of NOTCH3 mutations in 28 Italian CADASIL families","volume":"76","author":"Dotti","year":"2005","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"5","key":"10.1016\/j.nrleng.2020.04.031_bib0025","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1002\/1098-1004(200011)16:5<449::AID-HUMU26>3.0.CO;2-I","article-title":"Identification of a novel mutation C144F in the NOTCH3 gene in an Australian CADASIL pedigree","volume":"16","author":"Grigg","year":"2000","journal-title":"Hum Mutat"},{"issue":"10","key":"10.1016\/j.nrleng.2020.04.031_bib0030","doi-asserted-by":"crossref","first-page":"813","DOI":"10.1038\/sj.ejhg.5201221","article-title":"Detection of the founder effect in Finnish CADASIL families","volume":"12","author":"Mykk\u00e4nen","year":"2004","journal-title":"Eur J Hum Genet"},{"issue":"2","key":"10.1016\/j.nrleng.2020.04.031_bib0035","doi-asserted-by":"crossref","first-page":"176","DOI":"10.1016\/j.neulet.2009.06.087","article-title":"High recurrence of the R1006C NOTCH3 mutation in central Italian patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)","volume":"462","author":"Cappelli","year":"2009","journal-title":"Neurosci Lett"},{"issue":"1","key":"10.1016\/j.nrleng.2020.04.031_bib0040","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1016\/j.neulet.2011.10.062","article-title":"Multi-organ investigation in 16 CADASIL families from central Italy sharing the same R1006C mutation","volume":"506","author":"Ragno","year":"2012","journal-title":"Neurosci Lett"},{"issue":"2","key":"10.1016\/j.nrleng.2020.04.031_bib0045","doi-asserted-by":"crossref","first-page":"104","DOI":"10.14581\/jer.16020","article-title":"CADASIL initially presented with a seizure","volume":"6","author":"Oh","year":"2016","journal-title":"J Epilepsy Res"},{"issue":"2-3","key":"10.1016\/j.nrleng.2020.04.031_bib0050","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1159\/000106518","article-title":"MDF a epilepsy in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy","volume":"24","author":"Joost Haan","year":"2007","journal-title":"Cerebrovasc Dis"},{"key":"10.1016\/j.nrleng.2020.04.031_bib0055","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1016\/j.ebcr.2015.02.004","article-title":"Recurrent status epilepticus as the primary neurological manifestation of CADASIL: a case report","volume":"3","author":"Haddad","year":"2015","journal-title":"Epilepsy Behav Case Rep"},{"key":"10.1016\/j.nrleng.2020.04.031_bib0060","first-page":"36","article-title":"062 Cadasil presenting with focal and generalised epilepsy due to a novel NOTCH3 mutation","volume":"66","author":"Chen","year":"2018","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"2","key":"10.1016\/j.nrleng.2020.04.031_bib0065","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1136\/jnnp.74.2.249","article-title":"\u201cCADASIL coma\u201d: an underdiagnosed acute encephalopathy","volume":"74","author":"Schon","year":"2003","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"5","key":"10.1016\/j.nrleng.2020.04.031_bib0070","first-page":"731","article-title":"The phenotypic spectrum of CADASIL: clinical findings in 102 cases","volume":"44","author":"Oichgans","year":"1999","journal-title":"Headache Q"}],"container-title":["Neurolog\u00eda (English Edition)"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2173580822001729?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2173580822001729?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,31]],"date-time":"2025-10-31T03:35:05Z","timestamp":1761881705000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2173580822001729"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,3]]},"references-count":14,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2023,3]]}},"alternative-id":["S2173580822001729"],"URL":"https:\/\/doi.org\/10.1016\/j.nrleng.2020.04.031","relation":{},"ISSN":["2173-5808"],"issn-type":[{"type":"print","value":"2173-5808"}],"subject":[],"published":{"date-parts":[[2023,3]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Non-convulsive status epilepticus as the initial manifestation in a family with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)","name":"articletitle","label":"Article Title"},{"value":"Neurolog\u00eda (English Edition)","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.nrleng.2020.04.031","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"https:\/\/doi.org\/10.1016\/j.nrl.2020.04.026","name":"associatedlink","label":"CrossRef DOI link to the associated document"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2020 Sociedad Espa\u00f1ola de Neurolog\u00eda. Published by Elsevier Espa\u00f1a, S.L.U.","name":"copyright","label":"Copyright"}]}}