{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T21:27:08Z","timestamp":1770067628300,"version":"3.49.0"},"reference-count":25,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2021,6,11]],"date-time":"2021-06-11T00:00:00Z","timestamp":1623369600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Neurol."],"abstract":"<jats:p><jats:bold>Objectives:<\/jats:bold> The present study explored the clinical characteristics and prognostic factors of epilepsy in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS).<\/jats:p><jats:p><jats:bold>Methods:<\/jats:bold> Thirty-four MELAS patients were included in the present study. They were diagnosed by clinical characteristics, genetic testing, muscle biopsy, and retrospective analysis of other clinical data. The patients were divided into three groups according to the effects of treatment after at least 2 years of follow-up.<\/jats:p><jats:p><jats:bold>Results:<\/jats:bold> Epilepsy was more common in male MELAS patients than in females (20\/14). The age of onset ranged from 0.5 to 57 years, with an average of 22.6 years. Patients with epilepsy and MELAS had various forms of seizures. Focal seizures were the most common type affecting 58.82% of patients, and some patients had multiple types of seizures. The abnormal EEG waves were mainly concentrated in the occipital (69.57%), frontal (65.22%) and temporal lobes (47.83%). Overall, the prognosis of patients with epilepsy and MELAS was poor. Poor prognosis was associated with brain atrophy (<jats:italic>P<\/jats:italic> = 0.026), status epilepticus (<jats:italic>P<\/jats:italic> &amp;lt; 0.001), and use of anti-seizure medications with high mitochondrial toxicity (<jats:italic>P<\/jats:italic> = 0.015).<\/jats:p><jats:p><jats:bold>Interpretation:<\/jats:bold> Avoiding the application of anti-seizure medications with high mitochondrial toxicity, controlling seizures more actively and effectively, and delaying the occurrence and progression of brain atrophy as much as possible are particularly important to improve the prognosis of patients with MELAS and epilepsy.<\/jats:p>","DOI":"10.3389\/fneur.2021.675816","type":"journal-article","created":{"date-parts":[[2021,6,11]],"date-time":"2021-06-11T09:31:03Z","timestamp":1623403863000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":16,"title":["Epilepsy Associated With Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Episodes"],"prefix":"10.3389","volume":"12","author":[{"given":"Jiaai","family":"Li","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wuqiong","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhitao","family":"Cui","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhaoran","family":"Li","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ting","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hongmei","family":"Meng","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2021,6,11]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"349","DOI":"10.1023\/B:BOLI.0000031098.41409.55","article-title":"Mitochondrial disorders: prevalence, myths and advances","volume":"27","author":"Thorburn","year":"2004","journal-title":"J Inherit Metab Dis."},{"key":"B2","doi-asserted-by":"publisher","first-page":"147","DOI":"10.1016\/B978-0-12-802395-2.00010-9","article-title":"Mitochondrial diseases","volume":"145","author":"Molnar","year":"2017","journal-title":"Handb Clin Neurol."},{"key":"B3","doi-asserted-by":"publisher","first-page":"59","DOI":"10.1016\/j.pediatrneurol.2016.08.016","article-title":"Epilepsy characteristics and clinical outcome in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS)","volume":"64","author":"Lee","year":"2016","journal-title":"Pediatr Neurol."},{"key":"B4","doi-asserted-by":"publisher","first-page":"60","DOI":"10.1016\/j.ijcard.2008.04.089","article-title":"Manifestations of the mitochondrial A3243G mutation","volume":"137","author":"Finsterer","year":"2009","journal-title":"Int J Cardiol."},{"key":"B5","doi-asserted-by":"publisher","first-page":"956","DOI":"10.1016\/S1474-4422(15)00148-9","article-title":"Mitochondrial dysfunction and seizures: the neuronal energy crisis","volume":"14","author":"Zsurka","year":"2015","journal-title":"Lancet Neurol."},{"key":"B6","doi-asserted-by":"publisher","first-page":"397","DOI":"10.1111\/j.1469-8749.2011.04214.x","article-title":"Mitochondrial disease and epilepsy","volume":"54","author":"Rahman","year":"2012","journal-title":"Dev Med Child Neurol."},{"key":"B7","doi-asserted-by":"publisher","first-page":"270","DOI":"10.1016\/S0242-6498(05)80131-2","article-title":"Mitochondrial diseases: molecular mechanisms, clinical presentations and diagnosis investigations","volume":"25","author":"Aur\u00e9","year":"2005","journal-title":"Annal Pathol."},{"key":"B8","doi-asserted-by":"publisher","first-page":"816","DOI":"10.1212\/WNL.59.6.816","article-title":"Neuronal hyperexcitability in stroke-like episodes of MELAS syndrome","volume":"59","author":"Iizuka","year":"2002","journal-title":"Neurology."},{"key":"B9","first-page":"224","article-title":"Research progress on mitochondrial diseases and epilepsy","volume":"4","author":"Yang","year":"2018","journal-title":"J Epilepsy"},{"key":"B10","doi-asserted-by":"publisher","first-page":"619","DOI":"10.1016\/j.bbagen.2011.03.015","article-title":"MELAS: a nationwide prospective cohort study of 96 patients in Japan","volume":"1820","author":"Yatsuga","year":"2012","journal-title":"Biochim Biophys Acta."},{"key":"B11","doi-asserted-by":"publisher","first-page":"271","DOI":"10.1016\/j.yebeh.2017.03.022","article-title":"An overview of the 2017 ILAE operational classification of seizure types","volume":"70","author":"Fisher","year":"2017","journal-title":"Epilepsy Behav."},{"key":"B12","first-page":"74","article-title":"Clinical features of epileptic seizures in mitochondrial encephalomyopathy with hyperlactic acidemia and stroke-like seizures","volume":"56","author":"Yang","year":"2018","journal-title":"J Shandong Univ"},{"key":"B13","doi-asserted-by":"publisher","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."},{"key":"B14","doi-asserted-by":"publisher","first-page":"504","DOI":"10.1007\/s00415-013-7225-3","article-title":"The m","volume":"261","author":"Mancuso","year":"2014","journal-title":"J Neurol."},{"key":"B15","first-page":"1045","article-title":"Guidelines for the diagnosis and treatment of mitochondrial diseases of the nervous system in China","volume":"48","author":"Yuan","year":"2015","journal-title":"Chin J Neurol"},{"key":"B16","doi-asserted-by":"publisher","first-page":"720","DOI":"10.3969\/j.issn.1002-0152.2016.12.004","article-title":"Clinical features, image, electroencephalogram and genetic study in twelve melas patients","volume":"42","author":"Chen","year":"2016","journal-title":"Chin J Nerv Mental Dis"},{"key":"B17","doi-asserted-by":"publisher","first-page":"161","DOI":"10.1016\/j.yebeh.2015.10.018","article-title":"Ictal epileptic headache in adult life: electroclinical patterns and spectrum of related syndromes","volume":"53","author":"Fanella","year":"2015","journal-title":"Epilepsy Behav."},{"key":"B18","doi-asserted-by":"publisher","first-page":"e161","DOI":"10.1016\/j.ejrad.2009.05.048","article-title":"Diagnostic value of proton MR spectroscopy and diffusion-weighted MR imaging in childhood inherited neurometabolic brain diseases and review of the literature","volume":"74","author":"Cakmakci","year":"2010","journal-title":"Eur J Radiol."},{"key":"B19","doi-asserted-by":"publisher","first-page":"239","DOI":"10.1016\/S1474-4422(05)70043-0","article-title":"Progressive myoclonic epilepsies: a review of genetic and therapeutic aspects","volume":"4","author":"Shahwan","year":"2005","journal-title":"Lancet Neurol."},{"key":"B20","doi-asserted-by":"publisher","first-page":"859","DOI":"10.1007\/s00431-011-1662-8","article-title":"Classical MERRF phenotype associated with mitochondrial tRNA(Leu) (m.3243A>G) mutation","volume":"171","author":"Brackmann","year":"2012","journal-title":"Eur J Pediatr."},{"key":"B21","doi-asserted-by":"publisher","first-page":"467","DOI":"10.1042\/EBC20170114","article-title":"Emerging therapies for mitochondrial diseases","volume":"62","author":"Hirano","year":"2018","journal-title":"Essays Biochem."},{"key":"B22","doi-asserted-by":"publisher","first-page":"195","DOI":"10.1016\/j.spen.2010.06.012","article-title":"Outcome and prognosis of status epilepticus in children","volume":"17","author":"Ostrowsky","year":"2010","journal-title":"Semin Pediatr Neurol."},{"key":"B23","doi-asserted-by":"publisher","first-page":"146","DOI":"10.1016\/j.jcrc.2016.03.003","article-title":"Characteristics and outcome of patients with the ICU Admission diagnosis of status epilepticus in Australia and New Zealand","volume":"34","author":"Hay","year":"2016","journal-title":"J Crit Care."},{"key":"B24","doi-asserted-by":"publisher","first-page":"473","DOI":"10.1007\/164_2016_2","article-title":"Toxicity of antiepileptic drugs to mitochondria","volume":"240","author":"Finsterer","year":"2017","journal-title":"Handb Exp Pharmacol."},{"key":"B25","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1016\/j.eplepsyres.2017.07.003","article-title":"Effects of antiepileptic drugs on mitochondrial functions, morphology, kinetics, biogenesis, and survival","volume":"136","author":"Finsterer","year":"2017","journal-title":"Epilepsy Res."}],"container-title":["Frontiers in Neurology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneur.2021.675816\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,6,11]],"date-time":"2021-06-11T09:31:08Z","timestamp":1623403868000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneur.2021.675816\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,6,11]]},"references-count":25,"alternative-id":["10.3389\/fneur.2021.675816"],"URL":"https:\/\/doi.org\/10.3389\/fneur.2021.675816","relation":{},"ISSN":["1664-2295"],"issn-type":[{"value":"1664-2295","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,6,11]]},"article-number":"675816"}}