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Corticobasal syndrome and atypical parkinsonism have been occasionally reported, but progressive supranuclear palsy (PSP) syndrome (also known as Richardson syndrome (RS)) has not been reported in patients with FTLD-TDP. In this study we report clinical and pathologic characteristics of FTLD-TDP, clinically diagnosed as PSP syndrome (FTLD-TDP-PSP).<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Methods<\/jats:title>\n            <jats:p>We reviewed clinical information of 270 patients with FTLD-TDP from the Mayo Clinic brain bank and identified 5 patients with FTLD-TDP-PSP. As a control group, we selected ten consecutive patients of pathologically confirmed PSP with clinical presentations of PSP syndrome (PSP-RS). We compared the clinical and pathological features of FTLD-TDP-PSP and PSP-RS.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Results<\/jats:title>\n            <jats:p>The most common clinical symptoms in FTLD-TDP-PSP were memory loss (100%) followed by parkinsonism (80%), early falls (60%), and behavioral variant FTD (60%). All patients with PSP-RS met the Movement Disorder Society\u2019s criteria for probable PSP, while only one FTLD-TDP-PSP met the probable PSP. Two of the five patients with FTLD-TDP-PSP had moderate or severe neuronal loss in the substantia nigra and one had moderate or severe neuronal loss in the putamen and globus pallidus.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusion<\/jats:title>\n            <jats:p>A small subset of patients with FTLD-TDP can, in rare instances, present with symptoms associated with PSP. Therefore, FTLD-TDP may be considered in differential diagnosis, especially in patients who do not meet the diagnostic criteria. Our findings emphasize the need for further clinical and biomarker studies of FTLD-TDP.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1186\/s40478-025-02058-0","type":"journal-article","created":{"date-parts":[[2025,7,10]],"date-time":"2025-07-10T08:42:47Z","timestamp":1752136967000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Frontotemporal Lobar degeneration with TDP-43 presenting as progressive supranuclear palsy syndrome"],"prefix":"10.1186","volume":"13","author":[{"given":"Aya","family":"Murakami","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shunsuke","family":"Koga","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroaki","family":"Sekiya","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masataka","family":"Nakamura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yusuke","family":"Yakushiji","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dennis W.","family":"Dickson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,7,9]]},"reference":[{"key":"2058_CR1","doi-asserted-by":"publisher","first-page":"691","DOI":"10.1007\/s00401-011-0911-2","volume":"122","author":"S Al-Sarraj","year":"2011","unstructured":"Al-Sarraj S, King A, Troakes C, Smith B, Maekawa S, Bodi I, Rogelj B, Al-Chalabi A, Hortob\u00e1gyi T, Shaw CE (2011) p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND\/ALS. 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The Mayo Clinic brain bank operates under protocols approved by the Mayo Clinic Institutional Review Board.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}},{"value":"AM received speaker fees (royalties) from Chugai Pharmaceutical Co., Ltd. And supported by a JSPS KAKENHI Grant-in-Aid for Early-Career Scientists #23K14764. YY received speaker fees (royalties) from the following companies: Daiichi Sankyo Company, Limited; Otsuka Pharmaceutical Co., Ltd.; Eisai Co., Ltd.; Chugai Pharmaceutical Co., Ltd.; Takeda Pharmaceutical Co., Ltd.; Kyowa Kirin Co., Ltd.; Alexion Pharmaceuticals, Inc.; EA Pharma Co., Ltd.; Ono Pharmaceutical Co., Ltd.; Kowa Co., Ltd.; Novartis Pharma K.K.; UCB Japan Co., Ltd.; and Eli Lilly Japan K.K. and supported by a JSPS KAKENHI Grant-in-Aid for Scientific Research (C) #24K13494. SK, HS, and DWD have no financial disclosures to declare.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Financial disclosures of all authors for the preceding 12 months"}}],"article-number":"151"}}