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論文
- タイトル
- タイトル（英）
- Clinical course of pathologically confirmed corticobasal degeneration and corticobasal syndrome
- 参照URL
- https://researchmap.jp/masatohasegawa/published_papers/44253614
- 著者
- 著者（英）
- Ikuko Aiba,Yuichi Hayashi,Takayoshi Shimohata,Mari Yoshida,Yuko Saito,Koichi Wakabayashi,Takashi Komori,Masato Hasegawa,Takeshi Ikeuchi,Aya M Tokumaru,Keita Sakurai,Shigeo Murayama,Kazuko Hasegawa,Toshiki Uchihara,Yasuko Toyoshima,Yufuko Saito,Ichiro Yabe,Satoshi Tanikawa,Keizo Sugaya,Kentaro Hayashi,Terunori Sano,Masaki Takao,Motoko Sakai,Harutoshi Fujimura,Hiroshi Takigawa,Tadashi Adachi,Ritsuko Hanajima,Osamu Yokota,Tomoko Miki,Yasushi Iwasaki,Michio Kobayashi,Nobutaka Arai,Takuya Ohkubo,Takanori Yokota,Keiko Mori,Masumi Ito,Chiho Ishida,Masaharu Tanaka,Jiro Idezuka,Masato Kanazawa,Kenju Aoki,Masashi Aoki,Takafumi Hasegawa,Hirohisa Watanabe,Atsushi Hashizume,Hisayoshi Niwa,Keizo Yasui,Keita Ito,Yukihiko Washimi,Eiichiro Mukai,Akatsuki Kubota,Tatsushi Toda,Kenji Nakashima,Yuichi Hayashi,Takayoshi Shimohata,Mari Yoshida,Yuko Saito,Koichi Wakabayashi,Takashi Komori,Masato Hasegawa,Takeshi Ikeuchi,Aya M Tokumaru,Keita Sakurai,Shigeo Murayama,Kazuko Hasegawa,Toshiki Uchihara,Yasuko Toyoshima,Yufuko Saito,Ichiro Yabe,Satoshi Tanikawa,Keizo Sugaya,Kentaro Hayashi,Terunori Sano,Masaki Takao,Motoko Sakai,Harutoshi Fujimura,Hiroshi Takigawa,Tadashi Adachi,Ritsuko Hanajima,Osamu Yokota,Tomoko Miki,Yasushi Iwasaki,Michio Kobayashi,Nobutaka Arai,Takuya Ohkubo,Takanori Yokota,Keiko Mori,Masumi Ito,Chiho Ishida,Masaharu Tanaka,Jiro Idezuka,Masato Kanazawa,Kenju Aoki,Masashi Aoki,Takafumi Hasegawa,Hirohisa Watanabe,Atsushi Hashizume,Hisayoshi Niwa,Keizo Yasui,Keita Ito,Yukihiko Washimi,Eiichiro Mukai,Akatsuki Kubota,Tatsushi Toda,Kenji Nakashima,Shinya Tanaka,Kinya Ishikawa,Renpei Sengoku,Yasuhiro Sakashita,Tomoyasu Matubara,Kimiko Inoue,Chiaki Mori,Tomoko Saito,Takahiko Tokuda,Hisanori Kowa,Seishi Terada,Hanae Nakashima-Yasuda,Yuko Kato-Motozaki,Kiyonobu Komai,Osamu Onodera,Akiyoshi Kakita,Hiroshi Shimizu,Mari Tada,Arifumi Matsumoto,Akio Kikuchi,Mutsufusa Watanabe,Masahisa Katsuno,Tosiaki Ieda,Meiko Hashimoto Maeda,Ikuko Aiba
- 担当区分
- 概要
- 概要（英）
- Abstract 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’s disease (12.5%). The common course of corticobasal syndrome was initial gait disturbance and early fall. In addition, corticobasal degeneration–corticobasal syndrome manifested behavioural change (2.5 years) and cognitive impairment (3.0 years), as the patient with progressive supranuclear palsy–corticobasal syndrome developed speech impairment (1.0 years) and supranuclear gaze palsy (6.0 years). The Alzheimer’s disease–corticobasal syndrome patients showed cognitive impairment (1.0 years). The frequency of frozen gait at onset was higher in the corticobasal degeneration–corticobasal syndrome group than in the progressive supranuclear palsy–corticobasal syndrome group [P = 0.005, odds ratio (95% confidence interval): 31.67 (1.46–685.34)]. Dysarthria at presentation was higher in progressive supranuclear palsy–corticobasal syndrome than in corticobasal degeneration–corticobasal syndrome [P = 0.047, 6.75 (1.16–39.20)]. Pyramidal sign at presentation and personality change during the entire course were higher in Alzheimer’s disease–corticobasal syndrome than in progressive supranuclear palsy–corticobasal syndrome [P = 0.011, 27.44 (1.25–601.61), and P = 0.013, 40.00 (1.98–807.14), respectively]. In corticobasal syndrome, decision tree analysis revealed that ‘freezing at onset’ or ‘no dysarthria at presentation and age at onset under 66 years in the case without freezing at onset’ predicted corticobasal degeneration pathology with a sensitivity of 81.3% and specificity of 84.4%. ‘Dysarthria at presentation and age at onset over 61 years’ suggested progressive supranuclear palsy pathology, and ‘pyramidal sign at presentation and personality change during the entire course’ implied Alzheimer’s 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.
- 出版者・発行元
- 出版者・発行元（英）
- Oxford University Press (OUP)
- 誌名
- 誌名（英）
- Brain Communications
- 巻
- 5
- 号
- 6
- 開始ページ
- 終了ページ
- 出版年月
- 2023年11月1日
- 査読の有無
- 招待の有無
- 掲載種別
- 研究論文（学術雑誌）
- ISSN
- DOI URL
- https://doi.org/10.1093/braincomms/fcad296
- 共同研究・競争的資金等の研究課題
研究者
Hasegawa Masato
( )