{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T19:55:27Z","timestamp":1791230127883,"version":"4.1.0"},"reference-count":7,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2015]]},"abstract":"<jats:sec>\n                    <jats:title\/>\n                    <jats:p>Mitochondrial diseases have a special predilection to involve the brain in view of its high metabolic demand and the tendency for the formation of excitatory neurotransmitters when there is deficiency of intracellular ATP. These diseases have a great phenotypic variation and need a high degree of suspicion. However, some specific syndromes are well defined, both genotypically and phenotypically. Some of the drugs are potentially fatal mitochondrial poisons and an insight into that may be lifesaving as well as prevent serious morbidities. We report a typical case of myoclonic epilepsy with ragged red fibers (MERRF) with classical phenotype and genotype. There was rapid multiaxial deterioration with the introduction of sodium valproate which partly reversed on introducing mitochondrial cocktail and withdrawal of the offending drug. Sodium valproate, phenobarbitone, chloramphenicol and many anti-viral agents are mitochondrial poisons that increase the morbidity and mortality in patients with mitochondrial disease. More harm to the patient can be avoided with insight into this information.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.4103\/0022-3859.150905","type":"journal-article","created":{"date-parts":[[2015,2,5]],"date-time":"2015-02-05T07:03:55Z","timestamp":1423119835000},"page":"200-202","source":"Crossref","is-referenced-by-count":13,"title":["A typical case of myoclonic epilepsy with ragged red fibers (MERRF) and the lessons learned"],"prefix":"10.4103","volume":"61","author":[{"given":"SR","family":"Chandra","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"TG","family":"Issac","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N","family":"Gayathri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N","family":"Gupta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"MM","family":"Abbas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-10-20240205","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"},{"key":"R2-10-20240205","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1002\/ana.21217","article-title":"Prevalence of mitochondrial DNA disease in adults","volume":"63","author":"Schaefer","year":"2008","journal-title":"Ann Neurol"},{"key":"R3-10-20240205","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1016\/0022-510X(80)90031-3","article-title":"Myoclonus epilepsy associated with ragged-red fibres (mitochondrial abnormalities): Disease entity or a syndrome? Light-and electron-microscopic studies of two cases and review of literature","volume":"47","author":"Fukuhara","year":"1980","journal-title":"J Neurol Sci"},{"key":"R4-10-20240205","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1007\/BF01962701","article-title":"Metabolism of valproate to hepatotoxic intermediates","volume":"14","author":"Baillie","year":"1992","journal-title":"Pharm Weekblad Scientific edition"},{"key":"R5-10-20240205","doi-asserted-by":"crossref","first-page":"3347","DOI":"10.1002\/jnr.21444","article-title":"The glutamate-glutamine cycle is not stoichiometric: Fates of glutamate in brain","volume":"85","author":"McKenna","year":"2007","journal-title":"J Neurosci Res"},{"key":"R6-10-20240205","doi-asserted-by":"crossref","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":"R8-10-20240205","doi-asserted-by":"crossref","first-page":"1561","DOI":"10.1016\/j.addr.2008.05.001","article-title":"The mitochondrial cocktail: Rationale for combined nutraceutical therapy in mitochondrial cytopathies","volume":"60","author":"Tarnopolsky","year":"2008","journal-title":"Adv Drug Deliv Rev"}],"container-title":["Journal of Postgraduate Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/00005257-201561030-00010","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T19:40:20Z","timestamp":1786650020000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/00005257-201561030-00010"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015]]},"references-count":7,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2015]]}},"URL":"https:\/\/doi.org\/10.4103\/0022-3859.150905","relation":{},"ISSN":["0022-3859"],"issn-type":[{"value":"0022-3859","type":"print"}],"subject":[],"published":{"date-parts":[[2015]]}}}