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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）と進行性核上性麻痺（progressive supranuclear palsy：PSP）の臨床診断は近年，難しいものになっている。その理由は，原著以来定着した疾患概念1,2)が，病理学的に診断が確定した症例の臨床像の見直しにより，再考を要する状況となったためである。実際，CBD，PSPと臨床診断した症例の背景病理が予測されたものと異なったり，CBD，PSPと病理診断した症例の臨床像が多様であったり，CBDとPSPの両者の臨床像を呈する症例，いわゆるハイブリッド3)が存在することなどが明らかにされている。
このような混乱を避けるため，CBDやPSPという名称は病理診断名として使用し，代わって“corticobasal syndrome”（CBS)4)や“PSP-like syndrome”（PSPS)5)という名称を臨床診断名として使用するようになってきている。現在，CBDを漏れなく正確に臨床診断するには，①臨床的にはCBSを呈しながら，病理学的にはCBDでない症例を除外し，かつ②病理学的にはCBDでありながら，臨床的にはCBSを呈していない症例を見落とさず拾い上げることが必要となる（Fig.1）。
本稿では，CBDの疾患概念の成立と，臨床像の多様性について概説した後，CBSの診断基準を提示し，最後にCBSの中から背景病理をどのように鑑別すべきか議論したい。
大脳皮質基底核変性症（corticobasal degeneration：CBD）と進行性核上性麻痺（progressive supranuclear palsy：PSP）の臨床診断は近年，難しいものになっている。その理由は，原著以来定着した疾患概念1,2)が，病理学的に診断が確定した症例の臨床像の見直しにより，再考を要する状況となったためである。実際，CBD，PSPと臨床診断した症例の背景病理が予測されたものと異なったり，CBD，PSPと病理診断した症例の臨床像が多様であったり，CBDとPSPの両者の臨床像を呈する症例，いわゆるハイブリッド3)が存在することなどが明らかにされている。
このような混乱を避けるため，CBDやPSPという名称は病理診断名として使用し，代わって“corticobasal syndrome”（CBS)4)や“PSP-like syndrome”（PSPS)5)という名称を臨床診断名として使用するようになってきている。現在，CBDを漏れなく正確に臨床診断するには，①臨床的にはCBSを呈しながら，病理学的にはCBDでない症例を除外し，かつ②病理学的にはCBDでありながら，臨床的にはCBSを呈していない症例を見落とさず拾い上げることが必要となる（Fig.1）。
本稿では，CBDの疾患概念の成立と，臨床像の多様性について概説した後，CBSの診断基準を提示し，最後にCBSの中から背景病理をどのように鑑別すべきか議論したい。
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