{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T06:57:03Z","timestamp":1792047423886,"version":"4.4.0"},"reference-count":26,"publisher":"Wiley","issue":"s1","license":[{"start":{"date-parts":[[2002,1,4]],"date-time":"2002-01-04T00:00:00Z","timestamp":1010102400000},"content-version":"vor","delay-in-days":490,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2000,9]]},"abstract":"<jats:p>Pathology and associated clinical symptoms of hereditary dentatorubral\u2013pallidoluysian atrophy (H\u2010DRPLA) which was established as a new inherited neurodegenerative disease in 1982 are described. Obligatory lesions in the central nervous system combine with degeneration of the dentatorubral and pallidoluysian pathway, and occasional degenerative lesions are found in the cerebral white matter, putamen, Goll's nucleus of the medulla oblongata, and lateral corticospinal and Goll's tract of the spinal cord. The main clinical symptoms are myoclonus, epilepsy, dementia or mental retardation, cerebellar ataxia and choreoathetosis. Furthermore, newly developing aspects in the pathology of H\u2010DRPLA following the discovery of the gene locus of H\u2010DRPLA in 1994 are briefly described.<\/jats:p>","DOI":"10.1046\/j.1440-1789.2000.00307.x","type":"journal-article","created":{"date-parts":[[2003,3,12]],"date-time":"2003-03-12T15:06:53Z","timestamp":1047481613000},"page":"42-46","source":"Crossref","is-referenced-by-count":13,"title":["Hereditary dentatorubral\u2013pallidoluysian atrophy"],"prefix":"10.1111","volume":"20","author":[{"given":"Shinsaku","family":"Oyanagi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2002,1,4]]},"reference":[{"key":"e_1_2_5_2_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.32.8.798"},{"key":"e_1_2_5_3_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng0194-9"},{"key":"e_1_2_5_4_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng0194-14"},{"key":"e_1_2_5_5_2","first-page":"1135","article-title":"Geographical distribution of origins of DRPLA family in Japan and prevalence of DRPLA in Niigata prefecture (in Japanese).","volume":"32","author":"Inazuki G","year":"1990","journal-title":"Seishinigaku"},{"key":"e_1_2_5_6_2","first-page":"871","article-title":"Two families of progressive myoclonus epilepsy with Mendelian dominant heredity (in Japanese with English abstract).","volume":"74","author":"Naito H","year":"1972","journal-title":"Seishin Shinkeigaku Zasshi"},{"key":"e_1_2_5_7_2","first-page":"411","article-title":"A neuropathological study of 8 autopsy cases of degenerative type of myoclonus epilepsy: With special reference to latent combination of degeneration of the pallido\u2010luysian system. (in Japanese with English abstract).","volume":"20","author":"Oyanagi S","year":"1976","journal-title":"Shinkeikenkyu No Shimpo"},{"key":"e_1_2_5_8_2","first-page":"236","article-title":"On the pathology of chorea: With special reference to hereditary pallidal and dentate system atrophy proposed by us (in Japanese).","volume":"33","author":"Oyanagi S.","year":"1978","journal-title":"Saishin Igaku"},{"key":"e_1_2_5_9_2","first-page":"571","article-title":"Clinical picture and type of myoclonus epilepsy with dominant heredity (in Japanese with English abstract).","volume":"81","author":"Naito H","year":"1979","journal-title":"Seishin Shinkeigaku Zasshi"},{"key":"e_1_2_5_10_2","first-page":"13","volume-title":"DRPLA: From Clinical Neurology to Molecular Medicine.","author":"Naito H.","year":"1997"},{"key":"e_1_2_5_11_2","first-page":"519","volume-title":"Handbook of Clinical Neurology, Vol 21, Part 1.","author":"Smith JK.","year":"1975"},{"key":"e_1_2_5_12_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.47.12.1288"},{"key":"e_1_2_5_13_2","first-page":"199","article-title":"Clinico\u2010pathological study of the multiple systemic degeneration involving the dentate fugal system. 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