{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T01:46:05Z","timestamp":1747187165645,"version":"3.40.5"},"reference-count":19,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2023,9,17]],"date-time":"2023-09-17T00:00:00Z","timestamp":1694908800000},"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":[[2024,4]]},"abstract":"<jats:p>Progressive nonfluent aphasia (PNFA) is a form of frontotemporal lobar degeneration (FTLD) caused by tau and transactive response DNA\u2010binding protein of 43\u2009kDa (TDP\u201043) accumulation. Here we report the autopsy findings of a 64\u2010year\u2010old right\u2010handed man with an atypical TDP\u201043 proteinopathy who presented with difficulties with speech, verbal paraphasia, and dysphagia that progressed over the 36\u2009months prior to his death. He did not show pyramidal tract signs until his death. At autopsy, macroscopic brain examination revealed atrophy of the left dominant precentral, superior, and middle frontal gyri and discoloration of the putamen. Spongiform change and neuronal loss were severe on the cortical surfaces of the precentral, superior frontal, and middle frontal gyri and the temporal tip. Immunostaining with anti\u2010phosphorylated TDP\u201043 revealed neuronal cytoplasmic inclusions and long and short dystrophic neurites in the frontal cortex, predominantly in layers II, V, and VI of the temporal tip, amygdala, and transentorhinal cortex. Immunoblot analysis of the sarkosyl\u2010insoluble fractions showed hyperphosphorylated TDP\u201043 bands at 45\u2009kDa and phosphorylated C\u2010terminal fragments at approximately 25\u2009kDa. The pathological distribution and immunoblot band pattern differ from the major TDP\u201043 subtype and therefore may represent a new FTLD\u2010TDP phenotype.<\/jats:p>","DOI":"10.1111\/neup.12942","type":"journal-article","created":{"date-parts":[[2023,9,18]],"date-time":"2023-09-18T00:49:50Z","timestamp":1694998190000},"page":"154-160","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Atypical TDP\u201043 proteinopathy clinically presenting with progressive nonfluent aphasia: A case report"],"prefix":"10.1111","volume":"44","author":[{"given":"Yuki","family":"Suzuki","sequence":"first","affiliation":[{"name":"Division of Neurology, Department of Brain and Neurosciences, Faculty of Medicine Tottori University  Yonago Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tadashi","family":"Adachi","sequence":"additional","affiliation":[{"name":"Division of Neuropathology, Department of Brain and Neurosciences, Faculty of Medicine Tottori University  Yonago Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3053-4033","authenticated-orcid":false,"given":"Kentaro","family":"Yoshida","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Brain and Neurosciences, Faculty of Medicine Tottori University  Yonago Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kenta","family":"Taneda","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Brain and Neurosciences, Faculty of Medicine Tottori University  Yonago Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mayuko","family":"Sakuwa","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Brain and Neurosciences, Faculty of Medicine Tottori University  Yonago Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7415-8159","authenticated-orcid":false,"given":"Masato","family":"Hasegawa","sequence":"additional","affiliation":[{"name":"Department of Brain and Neurosciences Tokyo Metropolitan Institute of Medical Science  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ritsuko","family":"Hanajima","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Brain and Neurosciences, Faculty of Medicine Tottori University  Yonago Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,9,17]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e31821103e6"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000000031"},{"key":"e_1_2_9_4_1","first-page":"204","article-title":"Therapeutic and diagnostic challenges for frontotemporal dementia","volume":"6","author":"D'Alton S","year":"2014","journal-title":"Front Aging Neurosci"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-011-0845-8"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-011-0839-6"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.21425"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11910-014-0466-4"},{"key":"e_1_2_9_9_1","first-page":"811","article-title":"Examination of criterion validity about the standard language test of aphasia","volume":"72","author":"Tsutsumiuchi K","year":"2020","journal-title":"Brain Nerve"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/neup.12735"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-006-0127-z"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1093\/jnen\/63.9.911"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-010-5480-0"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1093\/jnen\/nlz132"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/neup.12763"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1789.2011.01253.x"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000436070.28137.7b"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1080\/13554794.2019.1609522"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1080\/13554794.2015.1041534"},{"key":"e_1_2_9_20_1","first-page":"130","article-title":"Neuropathology of neurodegenerative diseases associated with aphasia (in Japanese with English abstract)","volume":"22","author":"Yoshida M","year":"2021","journal-title":"Japan J Cognit Neurosci"}],"container-title":["Neuropathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/neup.12942","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,4,2]],"date-time":"2024-04-02T01:22:57Z","timestamp":1712020977000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/neup.12942"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,9,17]]},"references-count":19,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2024,4]]}},"alternative-id":["10.1111\/neup.12942"],"URL":"https:\/\/doi.org\/10.1111\/neup.12942","archive":["Portico"],"relation":{},"ISSN":["0919-6544","1440-1789"],"issn-type":[{"type":"print","value":"0919-6544"},{"type":"electronic","value":"1440-1789"}],"subject":[],"published":{"date-parts":[[2023,9,17]]},"assertion":[{"value":"2023-06-05","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-08-31","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-09-17","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}