{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,6]],"date-time":"2025-08-06T13:06:37Z","timestamp":1754485597045,"version":"3.40.5"},"reference-count":24,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2022,5,23]],"date-time":"2022-05-23T00:00:00Z","timestamp":1653264000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2022,8]]},"abstract":"<jats:sec><jats:label\/><jats:p>The presence of polyglutamine\u2010immunoreactive deposits in neurons of the neostriatum has been reported in dentatorubral\u2010pallidoluysian atrophy (DRPLA), Machado\u2010Joseph disease (MJD), and Huntington disease (HD). However, among these diseases, precise quantitative investigations on neurons have been performed only for HD. Changes in the number of neurons and the immunohistological features of polyglutamine deposits in the caudate head and putamen were examined in six patients with DRPLA, three with MJD, and four with HD. In the neostriatum in DRPLA, the numbers of large and small neurons were reduced to 33\u201338% and 48\u201368% relative to controls, respectively, whereas the corresponding figures in MJD were 19\u201326% and 65\u201376%, respectively, and those in HD were 34\u201335% and 12\u201316%, respectively. In DRPLA, 2\u201355% of neurons remaining in the neostriatum showed diffuse nuclear accumulation of polyglutamine, in contrast to 3\u201320% in MJD and a few percent in HD. These findings indicate that, in the neostriatum, a decrease in the number of small neurons is predominant in HD, whereas a decrease in the number of large neurons is predominant in DRPLA and MJD. Thus, it is suggested that disease processs differ among polyglutamine diseases.<\/jats:p><\/jats:sec>","DOI":"10.1111\/neup.12811","type":"journal-article","created":{"date-parts":[[2022,5,24]],"date-time":"2022-05-24T06:11:14Z","timestamp":1653372674000},"page":"274-281","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["The neostriatum in polyglutamine diseases: preferential decreases in large neurons in dentatorubral\u2010pallidoluysian atrophy and <scp>Machado\u2010Joseph<\/scp> disease and in small neurons in Huntington disease"],"prefix":"10.1111","volume":"42","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5196-3489","authenticated-orcid":false,"given":"Kiyomitsu","family":"Oyanagi","sequence":"first","affiliation":[{"name":"Department of Brain Disease Research Shinshu University School of Medicine  Matsumoto 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