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The diagnosis of mitochondrial cytopathy was established by the presence of ragged red fibers, cytochrome C oxydase\u2010negative fibers, and a defect of the complex IV of the respiratory chain in muscle biopsy. No mutations in mitochondrial DNA were detected. The choreic movements observed in juvenile forms of mitochondrial cytopathy are rarely observed in adults. Although striatal vulnerability is commonly reported in patients with mitochondrial disorders, the mechanism by which the mitochondrial dysfunction leads to chorea is not known. \u00a9 2004 Movement Disorder Society<\/jats:p>","DOI":"10.1002\/mds.20363","type":"journal-article","created":{"date-parts":[[2004,12,13]],"date-time":"2004-12-13T19:46:25Z","timestamp":1102967185000},"page":"490-492","source":"Crossref","is-referenced-by-count":18,"title":["Adult\u2010onset chorea and mitochondrial cytopathy"],"prefix":"10.1002","volume":"20","author":[{"given":"Morgane","family":"Caer","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karine","family":"Viala","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard","family":"Levy","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thierry","family":"Maisonobe","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Florence","family":"Chochon","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Agn\u00e8s","family":"Lomb\u00e8s","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yves","family":"Agid","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,12,13]]},"reference":[{"key":"e_1_2_4_2_2","doi-asserted-by":"publisher","DOI":"10.1097\/00019052-199708000-00012"},{"key":"e_1_2_4_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.870050204"},{"key":"e_1_2_4_4_2","first-page":"522","article-title":"Bilateral striatal necrosis with a novel point mutation in the mitochondrial ATPase 6 gene","volume":"36","author":"De Meirleir L","year":"1994","journal-title":"Ann Neurol"},{"key":"e_1_2_4_5_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410380321"},{"key":"e_1_2_4_6_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/118.2.339"},{"key":"e_1_2_4_7_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410370317"},{"key":"e_1_2_4_8_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.870090610"},{"key":"e_1_2_4_9_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.36.8.1053"},{"key":"e_1_2_4_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/1531-8257(199905)14:3<488::AID-MDS1017>3.0.CO;2-4"},{"key":"e_1_2_4_11_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.10682"},{"key":"e_1_2_4_12_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410390311"},{"key":"e_1_2_4_13_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1392"},{"key":"e_1_2_4_14_2","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.17-20-07746.1997"}],"container-title":["Movement Disorders"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmds.20363","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mds.20363","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/mds.20363","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mds.20363","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T12:43:29Z","timestamp":1760964209000},"score":1,"resource":{"primary":{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/10.1002\/mds.20363"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,12,13]]},"references-count":13,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2005,4]]}},"alternative-id":["10.1002\/mds.20363"],"URL":"https:\/\/doi.org\/10.1002\/mds.20363","archive":["Portico"],"relation":{},"ISSN":["0885-3185","1531-8257"],"issn-type":[{"type":"print","value":"0885-3185"},{"type":"electronic","value":"1531-8257"}],"subject":[],"published":{"date-parts":[[2004,12,13]]}}}