{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,3]],"date-time":"2025-07-03T14:37:54Z","timestamp":1751553474776,"version":"3.35.0"},"reference-count":33,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2024,5,7]],"date-time":"2024-05-07T00:00:00Z","timestamp":1715040000000},"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","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","id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["21K07450","21K06417","18K06506","22H04923"],"award-info":[{"award-number":["21K07450","21K06417","18K06506","22H04923"]}],"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":[[2025,2]]},"abstract":"<jats:p>A 68\u2010year\u2010old woman presented with difficulty finding words and writing characters. Neurological examination led to clinical diagnosis at onset of the logopenic variant of primary progressive aphasia accompanied with ideomotor apraxia, visuospatial agnosia on the right, and Gerstmann syndrome. Bradykinesia and rigidity on the right with shuffling gait developed after one year. Treatment with L\u2010dopa had no effect. The patient was diagnosed with corticobasal syndrome (CBS). Brain magnetic resonance imaging revealed diffuse cortical atrophy dominantly on the left, especially in the temporal, parietal, and occipital lobes. Positron emission tomography did not reveal any significant accumulation of amyloid \u03b2 or tau protein. She died five years later. Neuropathological examination revealed diffuse cortical atrophy with severe neuronal loss and fibrous gliosis in the cortex. Neuronal cytoplasmic inclusions, short dystrophic neurites, and, most notably, neuronal intranuclear inclusions, all immunoreactive for phosphorylated TDP\u201043, were observed. Western blotting revealed a full length and fragments of phosphorylated TDP\u201043 at 45 and 23\u2009kDa, respectively, confirming the pathological diagnosis of type A FTLD\u2010TDP. Whole exome sequencing revealed a pathogenic mutation in <jats:italic>GRN<\/jats:italic> (c.87dupC). FTLD\u2010TDP should be included in the differential diagnosis of CBS.<\/jats:p>","DOI":"10.1111\/neup.12980","type":"journal-article","created":{"date-parts":[[2024,5,8]],"date-time":"2024-05-08T05:54:43Z","timestamp":1715147683000},"page":"38-47","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["An autopsy case of type A <scp>FTLD\u2010TDP<\/scp> with a <scp><i>GRN<\/i><\/scp> mutation presenting with the logopenic variant of primary progressive aphasia at onset and with corticobasal syndrome subsequently"],"prefix":"10.1111","volume":"45","author":[{"given":"Takafumi","family":"Tomenaga","sequence":"first","affiliation":[{"name":"Department of Neurology Osaka Metropolitan University Graduate School of Medicine  Osaka Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shinobu","family":"Minatani","sequence":"additional","affiliation":[{"name":"Department of Neurology Osaka Metropolitan 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