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論文
- タイトル
- タイトル（英）
- Atypical TDP-43 proteinopathy clinically presenting with progressive nonfluent aphasia: A case report.
- 参照URL
- https://researchmap.jp/masatohasegawa/published_papers/43506050
- 著者
- 著者（英）
- Yuki Suzuki,Tadashi Adachi,Kentaro Yoshida,Kenta Taneda,Mayuko Sakuwa,Masato Hasegawa,Ritsuko Hanajima
- 担当区分
- 概要
- 概要（英）
- Progressive nonfluent aphasia (PNFA) is a form of frontotemporal lobar degeneration (FTLD) caused by tau and transactive response DNA-binding protein of 43 kDa (TDP-43) accumulation. Here we report the autopsy findings of a 64-year-old right-handed man with an atypical TDP-43 proteinopathy who presented with difficulties with speech, verbal paraphasia, and dysphagia that progressed over the 36 months 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-phosphorylated TDP-43 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-insoluble fractions showed hyperphosphorylated TDP-43 bands at 45 kDa and phosphorylated C-terminal fragments at approximately 25 kDa. The pathological distribution and immunoblot band pattern differ from the major TDP-43 subtype and therefore may represent a new FTLD-TDP phenotype.
- 出版者・発行元
- 出版者・発行元（英）
- 誌名
- 誌名（英）
- Neuropathology : official journal of the Japanese Society of Neuropathology
- 巻
- 号
- 開始ページ
- 終了ページ
- 出版年月
- 2023年9月17日
- 査読の有無
- 招待の有無
- 掲載種別
- ISSN
- DOI URL
- https://doi.org/10.1111/neup.12942
- 共同研究・競争的資金等の研究課題
研究者
Hasegawa Masato
( )