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特集 Corticobasal Syndrome
文献概要
はじめに
大脳皮質基底核変性症（corticobasal degeneration：CBD）は1968年にRebeizら1)によって最初に報告された。彼らは，運動拙劣，歩行障害，種々の不随意運動，眼球運動障害など多彩な症候を呈した3例を既知の疾患単位には当てはまらないとして，剖検所見をもとに“corticodentatonigral degeneration with neuronal achromasia”の名で報告した。その後，Gibbら2)は，Rebeizらの報告例を再検討するとともに自らも3剖検例の報告を行いCBDと呼ぶことを提唱した。現在では進行性核上性麻痺（progressive supranuclear palsy：PSP）や嗜銀顆粒性認知症（argyrophilic grain disease：AGD）とともに4リピートタウオパチーに分類される神経変性疾患である。
1985年以降，CBDの臨床所見，病理所見，タウ蛋白の生化学的所見が報告され，CBDは一疾患単位として確立した。しかし，CBDの臨床像は多彩であり，CBD以外のさまざまな疾患がCBDの臨床像を呈することもわかってきた。そのため現在では，CBDは病理診断名として用い，臨床診断名としてはcorticobasal syndrome（CBS）を使うようになっている3)。本稿でも，CBSは臨床診断名，CBDは病理診断名として用い，CBSを呈する疾患の病理学的背景やCBDの鑑別診断について概説する。
大脳皮質基底核変性症（corticobasal degeneration：CBD）は1968年にRebeizら1)によって最初に報告された。彼らは，運動拙劣，歩行障害，種々の不随意運動，眼球運動障害など多彩な症候を呈した3例を既知の疾患単位には当てはまらないとして，剖検所見をもとに“corticodentatonigral degeneration with neuronal achromasia”の名で報告した。その後，Gibbら2)は，Rebeizらの報告例を再検討するとともに自らも3剖検例の報告を行いCBDと呼ぶことを提唱した。現在では進行性核上性麻痺（progressive supranuclear palsy：PSP）や嗜銀顆粒性認知症（argyrophilic grain disease：AGD）とともに4リピートタウオパチーに分類される神経変性疾患である。
1985年以降，CBDの臨床所見，病理所見，タウ蛋白の生化学的所見が報告され，CBDは一疾患単位として確立した。しかし，CBDの臨床像は多彩であり，CBD以外のさまざまな疾患がCBDの臨床像を呈することもわかってきた。そのため現在では，CBDは病理診断名として用い，臨床診断名としてはcorticobasal syndrome（CBS）を使うようになっている3)。本稿でも，CBSは臨床診断名，CBDは病理診断名として用い，CBSを呈する疾患の病理学的背景やCBDの鑑別診断について概説する。
参考文献
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