{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,12]],"date-time":"2026-02-12T08:46:32Z","timestamp":1770885992729,"version":"3.50.1"},"reference-count":22,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2024,1,1]],"date-time":"2024-01-01T00:00:00Z","timestamp":1704067200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2024,1,1]],"date-time":"2024-01-01T00:00:00Z","timestamp":1704067200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2024,2,2]],"date-time":"2024-02-02T00:00:00Z","timestamp":1706832000000},"content-version":"vor","delay-in-days":32,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Epilepsy &amp; Behavior Reports"],"published-print":{"date-parts":[[2024]]},"DOI":"10.1016\/j.ebr.2024.100652","type":"journal-article","created":{"date-parts":[[2024,2,6]],"date-time":"2024-02-06T13:37:13Z","timestamp":1707226633000},"page":"100652","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":1,"special_numbering":"C","title":["Recurrent super-refractory status epilepticus and stroke like episode in a patient with Behr syndrome secondary to biallelic variants in OPA1 gene"],"prefix":"10.1016","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8828-0021","authenticated-orcid":false,"given":"Spoorthi","family":"Jagadish","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amy R.U.L.","family":"Calhoun","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sreenath Thati","family":"Ganganna","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"issue":"Pt 3","key":"10.1016\/j.ebr.2024.100652_b0005","doi-asserted-by":"crossref","first-page":"771","DOI":"10.1093\/brain\/awq007","article-title":"Multi-system neurological disease is common in patients with OPA1 mutations","volume":"133","author":"Yu-Wai-Man","year":"2010","journal-title":"Brain"},{"issue":"4","key":"10.1016\/j.ebr.2024.100652_b0010","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1016\/j.ymgme.2011.04.018","article-title":"Early-onset severe neuromuscular phenotype associated with compound heterozygosity for OPA1 mutations","volume":"103","author":"Schaaf","year":"2011","journal-title":"Mol Genet Metab"},{"issue":"10","key":"10.1016\/j.ebr.2024.100652_b0015","first-page":"e301","article-title":"Early-onset Behr syndrome due to compound heterozygous mutations in OPA1. brain: a","volume":"137","author":"Bonneau","year":"2014","journal-title":"J Neurol"},{"issue":"6","key":"10.1016\/j.ebr.2024.100652_b0020","doi-asserted-by":"crossref","first-page":"1005","DOI":"10.3390\/genes13061005","article-title":"Biallelic optic atrophy (OPA1) related disorder- case report and literature review","volume":"13","author":"Othman","year":"2022","journal-title":"Genes (Basel)"},{"issue":"10","key":"10.1016\/j.ebr.2024.100652_b0025","doi-asserted-by":"crossref","first-page":"1866","DOI":"10.1016\/j.biocel.2009.04.013","article-title":"OPA1 functions in mitochondria and dysfunctions in optic nerve","volume":"41","author":"Lenaers","year":"2009","journal-title":"Int J Biochem Cell Biol"},{"issue":"1","key":"10.1016\/j.ebr.2024.100652_b0030","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1186\/s13023-017-0641-1","article-title":"Not only dominant, not only optic atrophy: expanding the clinical spectrum associated with OPA1 mutations","volume":"12","author":"Nasca","year":"2017","journal-title":"Orphanet J Rare Dis"},{"issue":"4","key":"10.1016\/j.ebr.2024.100652_b0035","doi-asserted-by":"crossref","first-page":"1043","DOI":"10.1007\/s11011-019-00415-2","article-title":"Metabolic stroke in a patient with bi-allelic OPA1 mutations","volume":"34","author":"Zerem","year":"2019","journal-title":"Metab Brain Dis"},{"key":"10.1016\/j.ebr.2024.100652_b0040","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1016\/j.ejpn.2019.12.021","article-title":"The mitochondrial epilepsies","volume":"24","author":"Lim","year":"2020","journal-title":"Eur J Paediatr Neurol"},{"issue":"7","key":"10.1016\/j.ebr.2024.100652_b0045","first-page":"532","article-title":"Epilepsy in mitochondrial diseases-current state of knowledge on aetiology and treatment","volume":"8","author":"Wes\u00f3\u0142-Kucharska","year":"2021","journal-title":"Children (Basel)"},{"issue":"11","key":"10.1016\/j.ebr.2024.100652_b0050","article-title":"Metabolic control of epilepsy: a promising therapeutic target for epilepsy","volume":"8","author":"Fei","year":"2020","journal-title":"Front Neurol"},{"issue":"5","key":"10.1016\/j.ebr.2024.100652_b0055","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1111\/epi.12570","article-title":"Seizure semiology and EEG findings in mitochondrial diseases","volume":"55","author":"Chevallier","year":"2014","journal-title":"Epilepsia"},{"issue":"Suppl 4","key":"10.1016\/j.ebr.2024.100652_b0060","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1111\/j.1528-1167.2012.03618.x","article-title":"Mitochondrial diseases and epilepsy","volume":"53","author":"Bindoff","year":"2012","journal-title":"Epilepsia"},{"issue":"4","key":"10.1016\/j.ebr.2024.100652_b0065","doi-asserted-by":"crossref","first-page":"800","DOI":"10.1002\/jimd.12196","article-title":"Safety of drug use in patients with a primary mitochondrial disease: an international delphi-based consensus","volume":"43","author":"De Vries","year":"2020","journal-title":"J Inherit Metab Dis"},{"issue":"1","key":"10.1016\/j.ebr.2024.100652_b0070","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1517\/17425255.2012.644535","article-title":"Mitochondrial toxicity of antiepileptic drugs and their tolerability in mitochondrial disorders","volume":"8","author":"Finsterer","year":"2012","journal-title":"Expert Opin Drug Metab Toxicol"},{"issue":"4","key":"10.1016\/j.ebr.2024.100652_b0075","doi-asserted-by":"crossref","first-page":"685","DOI":"10.1111\/j.1528-1167.2007.01522.x","article-title":"Mitochondrial respiratory chain defects: underlying etiology in various epileptic conditions","volume":"49","author":"Lee","year":"2008","journal-title":"Epilepsia"},{"issue":"4","key":"10.1016\/j.ebr.2024.100652_b0080","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1055\/s-2004-820992","article-title":"Fatal propofol infusion syndrome in association with ketogenic diet","volume":"35","author":"Baumeister","year":"2004","journal-title":"Neuropediatrics"},{"issue":"400","key":"10.1016\/j.ebr.2024.100652_b0085","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1016\/j.jns.2019.03.021","article-title":"Mitochondrial metabolic stroke: phenotype and genetics of stroke-like episodes","volume":"15","author":"Finsterer","year":"2019","journal-title":"J Neurol Sci"},{"issue":"1","key":"10.1016\/j.ebr.2024.100652_b0090","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/j.ymgme.2021.12.014","article-title":"Stroke and stroke-like episodes in inborn errors of metabolism: pathophysiological and clinical implications","volume":"135","author":"Mastrangelo","year":"2022","journal-title":"Mol Genet Metab"},{"issue":"5","key":"10.1016\/j.ebr.2024.100652_b0095","doi-asserted-by":"crossref","first-page":"333","DOI":"10.4330\/wjc.v8.i5.333","article-title":"Mitochondrial vasculopathy","volume":"8","author":"Finsterer","year":"2016","journal-title":"World J Cardiol"},{"issue":"12","key":"10.1016\/j.ebr.2024.100652_b0100","article-title":"Inherited metabolic causes of stroke in children: mechanisms, types, and management","volume":"4","author":"Tabarki","year":"2021","journal-title":"Front Neurol"},{"issue":"3","key":"10.1016\/j.ebr.2024.100652_b0105","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1016\/j.ymgme.2018.01.010","article-title":"8-year retrospective analysis of intravenous arginine therapy for acute metabolic strokes in pediatric mitochondrial disease","volume":"123","author":"Ganetzky","year":"2018","journal-title":"Mol Genet Metab"},{"issue":"3","key":"10.1016\/j.ebr.2024.100652_b0110","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1016\/j.ymgme.2012.06.018","article-title":"Citrulline and arginine utility in treating nitric oxide deficiency in mitochondrial disorders","volume":"107","author":"El-Hattab","year":"2012","journal-title":"Mol Genet Metab"}],"container-title":["Epilepsy &amp; Behavior Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2589986424000091?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2589986424000091?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,11,12]],"date-time":"2025-11-12T00:09:33Z","timestamp":1762906173000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2589986424000091"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024]]},"references-count":22,"alternative-id":["S2589986424000091"],"URL":"https:\/\/doi.org\/10.1016\/j.ebr.2024.100652","relation":{},"ISSN":["2589-9864"],"issn-type":[{"value":"2589-9864","type":"print"}],"subject":[],"published":{"date-parts":[[2024]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Recurrent super-refractory status epilepticus and stroke like episode in a patient with Behr syndrome secondary to biallelic variants in OPA1 gene","name":"articletitle","label":"Article Title"},{"value":"Epilepsy & Behavior Reports","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ebr.2024.100652","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2024 The Authors. Published by Elsevier Inc.","name":"copyright","label":"Copyright"}],"article-number":"100652"}}