{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T21:34:26Z","timestamp":1792272866133,"version":"4.4.0"},"reference-count":53,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2023,11,3]],"date-time":"2023-11-03T00:00:00Z","timestamp":1698969600000},"content-version":"vor","delay-in-days":306,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare, Japan","doi-asserted-by":"publisher","award":["20FC1049"],"award-info":[{"award-number":["20FC1049"]}],"id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP19dm0107105"],"award-info":[{"award-number":["JP19dm0107105"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP20dm0107105"],"award-info":[{"award-number":["JP20dm0107105"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP22wm0425019"],"award-info":[{"award-number":["JP22wm0425019"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP22ek0109545"],"award-info":[{"award-number":["JP22ek0109545"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,11,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>The clinical presentation of corticobasal degeneration is diverse, while the background pathology of corticobasal syndrome is also heterogeneous. Therefore, predicting the pathological background of corticobasal syndrome is extremely difficult. Herein, we investigated the clinical findings and course in patients with pathologically, genetically and biochemically verified corticobasal degeneration and corticobasal syndrome with background pathology to determine findings suggestive of background disorder. Thirty-two patients were identified as having corticobasal degeneration. The median intervals from the initial symptoms to the onset of key milestones were as follows: gait disturbance, 0.0 year; behavioural changes, 1.0 year; falls, 2.0 years; cognitive impairment, 2.0 years; speech impairment, 2.5 years; supranuclear gaze palsy, 3.0 years; urinary incontinence, 3.0 years; and dysphagia, 5.0 years. The median survival time was 7.0 years; 50% of corticobasal degeneration was diagnosed as corticobasal degeneration\/corticobasal syndrome at the final presentation. Background pathologies of corticobasal syndrome (n = 48) included corticobasal degeneration (33.3%), progressive supranuclear palsy (29.2%) and Alzheimer\u2019s disease (12.5%). The common course of corticobasal syndrome was initial gait disturbance and early fall. In addition, corticobasal degeneration\u2013corticobasal syndrome manifested behavioural change (2.5 years) and cognitive impairment (3.0 years), as the patient with progressive supranuclear palsy\u2013corticobasal syndrome developed speech impairment (1.0 years) and supranuclear gaze palsy (6.0 years). The Alzheimer\u2019s disease\u2013corticobasal syndrome patients showed cognitive impairment (1.0 years). The frequency of frozen gait at onset was higher in the corticobasal degeneration\u2013corticobasal syndrome group than in the progressive supranuclear palsy\u2013corticobasal syndrome group [P = 0.005, odds ratio (95% confidence interval): 31.67 (1.46\u2013685.34)]. Dysarthria at presentation was higher in progressive supranuclear palsy\u2013corticobasal syndrome than in corticobasal degeneration\u2013corticobasal syndrome [P = 0.047, 6.75 (1.16\u201339.20)]. Pyramidal sign at presentation and personality change during the entire course were higher in Alzheimer\u2019s disease\u2013corticobasal syndrome than in progressive supranuclear palsy\u2013corticobasal syndrome [P = 0.011, 27.44 (1.25\u2013601.61), and P = 0.013, 40.00 (1.98\u2013807.14), respectively]. In corticobasal syndrome, decision tree analysis revealed that \u2018freezing at onset\u2019 or \u2018no dysarthria at presentation and age at onset under 66 years in the case without freezing at onset\u2019 predicted corticobasal degeneration pathology with a sensitivity of 81.3% and specificity of 84.4%. \u2018Dysarthria at presentation and age at onset over 61 years\u2019 suggested progressive supranuclear palsy pathology, and \u2018pyramidal sign at presentation and personality change during the entire course\u2019 implied Alzheimer\u2019s disease pathology. In conclusion, frozen gait at onset, dysarthria, personality change and pyramidal signs may be useful clinical signs for predicting background pathologies in corticobasal syndrome.<\/jats:p>","DOI":"10.1093\/braincomms\/fcad296","type":"journal-article","created":{"date-parts":[[2023,11,7]],"date-time":"2023-11-07T23:22:55Z","timestamp":1699399375000},"source":"Crossref","is-referenced-by-count":38,"title":["Clinical course of pathologically confirmed corticobasal degeneration and corticobasal syndrome"],"prefix":"10.1093","volume":"5","author":[{"given":"Ikuko","family":"Aiba","sequence":"first","affiliation":[{"name":"Department of Neurology, NHO Higashinagoya National Hospital , Nagoya, Aichi 465-8620 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuichi","family":"Hayashi","sequence":"additional","affiliation":[{"name":"Department of Neurology, Gifu University Graduate School of Medicine , Gifu 501-1194 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takayoshi","family":"Shimohata","sequence":"additional","affiliation":[{"name":"Department of Neurology, Gifu University Graduate School of Medicine , Gifu 501-1194 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mari","family":"Yoshida","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University , Nagakute, Aichi 480-1195 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuko","family":"Saito","sequence":"additional","affiliation":[{"name":"Department of Neuropathology (the Brain Bank for Aging Research), Tokyo Metropolitan Institute for Geriatrics and Gerontology , Itabashi, Tokyo 173-0015 , Japan"},{"name":"Department of Pathology and Laboratory Medicine, National Center Hospital, National Center of Neurology and Psychiatry , Kodaira, Tokyo 187-8551 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koichi","family":"Wakabayashi","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Hirosaki University Graduate School of Medicine , Hirosaki, Aomori 036-8562 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takashi","family":"Komori","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology (Neuropathology), Tokyo Metropolitan Neurological Hospital , Fuchu, Tokyo 183-0042 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7415-8159","authenticated-orcid":false,"given":"Masato","family":"Hasegawa","sequence":"additional","affiliation":[{"name":"Department of Brain & Neurosciences, Tokyo Metropolitan Institute of Medical Science , Setagaya, Tokyo 156-8506 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8828-8085","authenticated-orcid":false,"given":"Takeshi","family":"Ikeuchi","sequence":"additional","affiliation":[{"name":"Department of Molecular Genetics, Brain Research Institute, Niigata University , Chuo, Niigata 951-8585 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aya M","family":"Tokumaru","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Radiology, Tokyo Metropolitan Institute for Geriatrics and Gerontology , Itabashi, Tokyo 173-0015 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keita","family":"Sakurai","sequence":"additional","affiliation":[{"name":"Department of Radiology, National Center for Geriatrics and Gerontology , Obu, Aichi 474-8511 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shigeo","family":"Murayama","sequence":"additional","affiliation":[{"name":"Brain 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Kodaira, Tokyo 187-8551 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaki","family":"Takao","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, National Center Hospital, National Center of Neurology and Psychiatry , Kodaira, Tokyo 187-8551 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Motoko","family":"Sakai","sequence":"additional","affiliation":[{"name":"Department of Neurology, NHO Suzuka National Hospital , Suzuka, Mie 513-8501 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harutoshi","family":"Fujimura","sequence":"additional","affiliation":[{"name":"Department of Neurology, NHO Osaka Toneyama Medical Center , Toyonaka, Osaka 560-8552 , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroshi","family":"Takigawa","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Brain and Neurosciences, Faculty of Medicine, 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