BRAIN and NERVE－神経研究の進歩64巻4号

2012年04月発行

総説

Corticobasal syndrome―最近の進歩と今後の課題

著者： 饗場郁子¹

所属機関： ¹国立病院機構東名古屋病院神経内科

ページ範囲：P.462 - P.473

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Reveizら¹⁾により臨床病理学的に独立した疾患として報告された。その後，剖検例の蓄積によりCBDの臨床像は極めて多彩であることが明らかとなり（Fig.1)^2-10)，一方CBDの典型的臨床像（＝corticobasal syndrome：CBS³⁾）を呈する背景疾患もさまざまである（Fig.2）ことがわかってきた^2,3,6,11-20)。そのため臨床診断名と病理診断名を区別し，CBSは臨床診断名，CBDは病理診断名として使われるようになっている³⁾。本総説はCBSについて，現在までにわかっていることと今後の課題を概説したい。

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