{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,22]],"date-time":"2026-01-22T06:37:53Z","timestamp":1769063873844,"version":"3.49.0"},"reference-count":46,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2004,10,8]],"date-time":"2004-10-08T00:00:00Z","timestamp":1097193600000},"content-version":"vor","delay-in-days":4117,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[1993,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We reviewed 10 patients (5 males, 5 females) with mitochondrial encephalomyopathy, lactic acidosis, and stroke\u2010like episodes. The age of symptom onset ranged from 3 months to 12 years. All had lactic acidosis, multiple stroke\u2010like events with secondary neurological deficits, radiological changes of progressive brain infarction, and muscle biopsy showing ragged\u2010red fibers. In patients with earlier onset of symptoms (&lt;2 yr), involvement tended to be more diffuse, with failure to thrive and early onset of delayed development. Patients whose symptoms appeared later tended to have focal neurological deficits with migraine\u2010like headache, and a rate of cognitive regression reflecting the rapidity of disease progression. Radiological changes included multiple areas of infarction with initial predilection for parietal occipital areas, progressing to generalized atrophy. Pathological findings in muscle biopsies included type 1 fiber predominance, ragged\u2010red fibers, increased intermyofibrillar lipid deposition, and abnormal mitochondria. Four patients showed mitochondrial DNA tRNA mutation at position 3,243. No difference was noted in clinical, radiological, or pathological findings in patients with and without this mutation, suggesting that multiple sites of point mutation may give rise to mitochondrial encephalomyopathy, lactic acidosis, and stroke\u2010like episodes.<\/jats:p>","DOI":"10.1002\/ana.410340107","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T00:24:42Z","timestamp":1104539082000},"page":"25-32","source":"Crossref","is-referenced-by-count":91,"title":["Mitochondrial encephalomyopathy, lactic acidosis, stroke\u2010like episodes (MELAS): Clinical, radiological, pathological, and genetic observations"],"prefix":"10.1002","volume":"34","author":[{"given":"Betty","family":"Koo","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laurence E.","family":"Becker","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sylvester","family":"Chuang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Frank","family":"Merante","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Brian H.","family":"Robinson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daune","family":"MacGregor","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ingrid","family":"Tein","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vincent B.","family":"Ho","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Douglas A.","family":"McGreal","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John R.","family":"Wherrett","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"William J.","family":"Logan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,10,8]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410160409"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.38.5.751"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.34.1.72"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410290606"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410250611"},{"key":"e_1_2_1_7_2","first-page":"467","article-title":"Clinical syndromes associated with ragged red fibers","volume":"147","author":"Rowland LP","year":"1991","journal-title":"Rev Neurol (Paris)"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4757-9065-8_5"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/0166-2236(91)90081-5"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410170303"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(90)90059-N"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.25.8.528"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(91)90136-D"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0733-8619(18)30832-6"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1038\/348651a0"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410310408"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.3201231"},{"key":"e_1_2_1_18_2","first-page":"852","article-title":"Heteroplasmic mtDNA mutation (T\u2010G) at 8993 can cause Leigh disease when the percentage of abnormal mtDNA is high","volume":"50","author":"Tatuch Y","year":"1992","journal-title":"Am J Hum Genet"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410170602"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.41.10.1663"},{"key":"e_1_2_1_21_2","doi-asserted-by":"publisher","DOI":"10.1016\/0925-4439(91)90042-8"},{"key":"e_1_2_1_22_2","first-page":"99","volume-title":"Definition of pathological changes seen in muscle biopsies","author":"Dubowitz V","year":"1985"},{"key":"e_1_2_1_23_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(73)90023-3"},{"key":"e_1_2_1_24_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.33.9.1179"},{"key":"e_1_2_1_25_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/109.5.915"},{"key":"e_1_2_1_26_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00593950"},{"key":"e_1_2_1_27_2","unstructured":"De VolderA GhilainS de BarsyTh GoffinetAM . Brain metabolism in mitochondrial encephalomyopathy: a PET study.J Comp Assist Tomogr1988:12854\u201312857"},{"key":"e_1_2_1_28_2","doi-asserted-by":"publisher","DOI":"10.1159\/000120365"},{"key":"e_1_2_1_29_2","first-page":"1234","article-title":"CT and MR of MELAS syndrome","volume":"9","author":"Allard JC","year":"1988","journal-title":"AJNR"},{"key":"e_1_2_1_30_2","doi-asserted-by":"publisher","DOI":"10.1097\/00004728-199101000-00010"},{"key":"e_1_2_1_31_2","first-page":"111","volume-title":"Mitochondrial pathology in muscle diseases","author":"DiMauro S","year":"1983"},{"key":"e_1_2_1_32_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410250614"},{"key":"e_1_2_1_33_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410260104"},{"key":"e_1_2_1_34_2","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-197711100-00005"},{"key":"e_1_2_1_35_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1976.00500070017003"},{"key":"e_1_2_1_36_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(72)90070-6"},{"key":"e_1_2_1_37_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(76)90063-0"},{"key":"e_1_2_1_38_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(81)90038-1"},{"key":"e_1_2_1_39_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.34.1.48"},{"key":"e_1_2_1_40_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1970.00480260009002"},{"key":"e_1_2_1_41_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410240609"},{"key":"e_1_2_1_42_2","first-page":"440","volume-title":"The cell","author":"Fawcett DW","year":"1981"},{"key":"e_1_2_1_43_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1977.00500150066013"},{"key":"e_1_2_1_44_2","doi-asserted-by":"publisher","DOI":"10.1016\/0887-8994(90)90116-I"},{"key":"e_1_2_1_45_2","first-page":"599","article-title":"Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke\u2010like episodes. Report of an autopsy","volume":"39","author":"Hamazaki S","year":"1989","journal-title":"Acta Pathol Jpn"},{"key":"e_1_2_1_46_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00688185"},{"key":"e_1_2_1_47_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00442074"}],"container-title":["Annals of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fana.410340107","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ana.410340107","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,25]],"date-time":"2023-10-25T06:44:02Z","timestamp":1698216242000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ana.410340107"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1993,7]]},"references-count":46,"journal-issue":{"issue":"1","published-print":{"date-parts":[[1993,7]]}},"alternative-id":["10.1002\/ana.410340107"],"URL":"https:\/\/doi.org\/10.1002\/ana.410340107","archive":["Portico"],"relation":{},"ISSN":["0364-5134","1531-8249"],"issn-type":[{"value":"0364-5134","type":"print"},{"value":"1531-8249","type":"electronic"}],"subject":[],"published":{"date-parts":[[1993,7]]}}}