{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,12]],"date-time":"2026-05-12T04:47:56Z","timestamp":1778561276634,"version":"3.51.4"},"reference-count":55,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2022,2,23]],"date-time":"2022-02-23T00:00:00Z","timestamp":1645574400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP18dm0107103"],"award-info":[{"award-number":["JP18dm0107103"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP 16H06277"],"award-info":[{"award-number":["JP 16H06277"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2022,4]]},"abstract":"<jats:p>Lewy body\u2010related \u03b1\u2010synucleinopathy (Lewy pathology) has been reported in patients with myotonic dystrophy (DM) type 1 (DM1), but no detailed report has described the prevalence and extent of its occurrence. We studied consecutive full autopsy cases of DM1 at the National Center of Neurology and Psychiatry (NCNP) Brain Bank for intractable psychiatric and neurological disorders. Thirty\u2010two cases, genetically determined to be DM1 (59.0\u2009\u00b1\u20098.7\u2009years), obtained from the NCNP Brain Bank, were compared with control cases obtained from the Brain Bank for Aging Research (BBAR) in Japan. The investigated anatomical sites followed the Dementia with Lewy Bodies Consensus Guideline, expanding to the peripheral autonomic nervous system, temporal pole, and occipital cortex, in addition to the olfactory epithelium and spinal cord. Of the 32 patients, 11 (34.4%) had Lewy pathology, with a significantly higher prevalence than that in the control cases from the BBAR (20.1%). Lewy pathology detected in DM1 was widespread, but no macroscopic depigmentation of the substantia nigra was observed in any DM1 case; this was commensurate with the microscopic paucity of Lewy pathology in the substantia nigra and amygdala. Lewy pathology in DM1 does not appear to follow either Braak's ascending paradigm or the olfactory\u2013amygdala extension. Lewy neurites and dots in DM1 were very sparse in the cerebral cortex and distinct from those observed in BBAR control cases. This study was the first demonstration of unique Lewy pathology in DM1 and may contribute to the understanding of the protein propagation hypothesis of Lewy pathology.<\/jats:p>","DOI":"10.1111\/neup.12790","type":"journal-article","created":{"date-parts":[[2022,2,24]],"date-time":"2022-02-24T04:00:07Z","timestamp":1645675207000},"page":"104-116","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Unique Lewy pathology in myotonic dystrophy type 1"],"prefix":"10.1111","volume":"42","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8967-5984","authenticated-orcid":false,"given":"Terunori","family":"Sano","sequence":"first","affiliation":[{"name":"Department of Laboratory Medicine National Center Hospital, National Center of Neurology and Psychiatry  Tokyo Japan"},{"name":"NCNP Brain Physiology and Pathology, Cognitive and Behavioral Medicine, Graduate School of Medical and Dental Sciences Tokyo Medical and Dental University  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tomoya","family":"Kawazoe","sequence":"additional","affiliation":[{"name":"Department of Neurology, National Center Hospital National Center of Neurology and Psychiatry  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ayako","family":"Shioya","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine National Center Hospital, National Center of Neurology and Psychiatry  Tokyo Japan"},{"name":"Department of Neurology, Mito Kyodo General Hospital Tsukuba University Hospital Mito Area Medical Education Center  Ibaraki Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Madoka","family":"Mori\u2010Yoshimura","sequence":"additional","affiliation":[{"name":"Department of Neurology, National Center Hospital National Center of Neurology and Psychiatry  Tokyo 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