{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,15]],"date-time":"2026-07-15T13:39:07Z","timestamp":1784122747434,"version":"3.55.0"},"reference-count":70,"publisher":"Wiley","issue":"S2","license":[{"start":{"date-parts":[[2018,8,29]],"date-time":"2018-08-29T00:00:00Z","timestamp":1535500800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100000765","name":"University College London","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000765","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2018,10]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>This narrative review focuses on the pathophysiology, diagnosis, and management of status epilepticus in the context of primary mitochondrial disease. Epilepsy is common in mitochondrial disease, reported in &gt;20% of adult cases and 40%\u201060% of pediatric cohorts. Status epilepticus is less frequently reported and appears to be associated with particular subgroups of mitochondrial disorders, namely defects of the mitochondrial <jats:styled-content style=\"fixed-case\">DNA<\/jats:styled-content> and its maintenance, and disorders of mitochondrial translation and dynamics. Mechanisms underlying mitochondrial status epilepticus are incompletely understood, and may include bioenergetic failure, oxidative stress, immune dysfunction, and impaired mitochondrial dynamics. Treatments tried in mitochondrial status epilepticus include antiepileptic drugs, anesthetic agents, magnesium, high\u2010dose steroids, immune globulins, vagus nerve stimulation, and surgical procedures, all with variable success. The outcome of mitochondrial status epilepticus is extremely poor, and effective therapeutic options have not been reported. Improved understanding of the mechanisms underpinning mitochondrial status epilepticus is needed in order to develop more effective treatments.<\/jats:p>","DOI":"10.1111\/epi.14485","type":"journal-article","created":{"date-parts":[[2018,8,30]],"date-time":"2018-08-30T10:35:31Z","timestamp":1535625331000},"page":"70-77","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":42,"title":["Mitochondrial diseases and status epilepticus"],"prefix":"10.1111","volume":"59","author":[{"given":"Shamima","family":"Rahman","sequence":"first","affiliation":[{"name":"Mitochondrial Research Group UCL Great Ormond Street Institute of Child Health London UK"},{"name":"Metabolic Unit Great Ormond Street Hospital for Children NHS Foundation Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,8,29]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799603"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.56.10.1340"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2009.02504.x"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.12570"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.13121"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.seizure.2016.10.010"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.46.4.1029"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(06)69043-0"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12028-013-9935-x"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2011.03223.x"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2015.04.013"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2012-303528"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0145500"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.24525"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1157.1998.tb01397.x"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00134-013-2884-1"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000092497.53706.1B"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2010.05.020"},{"key":"e_1_2_11_20_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e31823a0c7f"},{"key":"e_1_2_11_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.25839"},{"key":"e_1_2_11_22_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneurpsyc.1931.02230030027002"},{"key":"e_1_2_11_23_1","doi-asserted-by":"publisher","DOI":"10.1038\/gim.2017.35"},{"key":"e_1_2_11_24_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20079"},{"key":"e_1_2_11_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.13508"},{"key":"e_1_2_11_26_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awp045"},{"key":"e_1_2_11_27_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng2040"},{"key":"e_1_2_11_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mito.2014.10.007"},{"key":"e_1_2_11_29_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-016-0477-0"},{"key":"e_1_2_11_30_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmedgenet-2015-103049"},{"key":"e_1_2_11_31_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/dds294"},{"key":"e_1_2_11_32_1","doi-asserted-by":"publisher","DOI":"10.1038\/jhg.2016.163"},{"key":"e_1_2_11_33_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-011-9413-6"},{"key":"e_1_2_11_34_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.22590"},{"key":"e_1_2_11_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2014.03.003"},{"key":"e_1_2_11_36_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.37721"},{"key":"e_1_2_11_37_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awv182"},{"key":"e_1_2_11_38_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awx002"},{"key":"e_1_2_11_39_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/9.8.1245"},{"key":"e_1_2_11_40_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2013.12.015"},{"key":"e_1_2_11_41_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2008.08.009"},{"key":"e_1_2_11_42_1","doi-asserted-by":"publisher","DOI":"10.1007\/8904_2013_287"},{"key":"e_1_2_11_43_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12519-012-0348-2"},{"key":"e_1_2_11_44_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMc0809100"},{"key":"e_1_2_11_45_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.13003"},{"key":"e_1_2_11_46_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awt328"},{"key":"e_1_2_11_47_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neulet.2016.12.044"},{"key":"e_1_2_11_48_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2015.05.003"},{"key":"e_1_2_11_49_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2009.08.005"},{"key":"e_1_2_11_50_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneurol.2010.36"},{"key":"e_1_2_11_51_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.eplepsyres.2016.03.007"},{"key":"e_1_2_11_52_1","doi-asserted-by":"publisher","DOI":"10.3389\/fncel.2016.00124"},{"key":"e_1_2_11_53_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2008.01877.x"},{"key":"e_1_2_11_54_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.eplepsyres.2008.08.005"},{"key":"e_1_2_11_55_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awr215"},{"key":"e_1_2_11_56_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-010-5721-2"},{"key":"e_1_2_11_57_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2014.01.009"},{"key":"e_1_2_11_58_1","doi-asserted-by":"publisher","DOI":"10.1080\/01443610802048545"},{"key":"e_1_2_11_59_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e31820a0d39"},{"key":"e_1_2_11_60_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.seizure.2012.08.009"},{"key":"e_1_2_11_61_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mito.2007.04.003"},{"key":"e_1_2_11_62_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00381-011-1495-9"},{"key":"e_1_2_11_63_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00381-011-1502-1"},{"key":"e_1_2_11_64_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00381-011-1501-2"},{"key":"e_1_2_11_65_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2015.06.004"},{"key":"e_1_2_11_66_1","doi-asserted-by":"publisher","DOI":"10.1684\/epd.2012.0543"},{"key":"e_1_2_11_67_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuropharm.2012.11.004"},{"key":"e_1_2_11_68_1","doi-asserted-by":"publisher","DOI":"10.1111\/jnc.12646"},{"key":"e_1_2_11_69_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awv325"},{"key":"e_1_2_11_70_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-016-9930-4"},{"key":"e_1_2_11_71_1","first-page":"197","article-title":"Management of mitochondrial disease on an intensive care unit","volume":"88","author":"Howard RS","year":"1995","journal-title":"QJM"}],"container-title":["Epilepsia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fepi.14485","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/epi.14485","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,16]],"date-time":"2023-09-16T18:47:19Z","timestamp":1694890039000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/epi.14485"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,8,29]]},"references-count":70,"journal-issue":{"issue":"S2","published-print":{"date-parts":[[2018,10]]}},"alternative-id":["10.1111\/epi.14485"],"URL":"https:\/\/doi.org\/10.1111\/epi.14485","archive":["Portico"],"relation":{},"ISSN":["0013-9580","1528-1167"],"issn-type":[{"value":"0013-9580","type":"print"},{"value":"1528-1167","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,8,29]]},"assertion":[{"value":"2017-10-26","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-08-29","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}