フォア・シャヴァニ・マリー症候群—その今日的意義

Foix-Chavany-Marie Syndrome: A Clinical Overview

フォア・シャヴァニ・マリー症候群は19世紀から報告されてきた「皮質型」偽性球麻痺である。脳卒中以外にも多彩な疾患が原因となり得る。近年白質損傷も責任病巣となり得るとされ，特にfrontal aslant tractの損傷が注目されている。「古い」症候群のベッドサイドでの詳細な観察と，「新しい」画像検査や解析の組合せにより，背景にある神経システムをよりよく理解することが可能となると期待される。

Abstract

Foix-Chavany-Marie syndrome, which has been reported since the 19th century, is a cortical type of pseudobulbar palsy. Although previously most cases were due to acute recurrent stroke, recent reports have indicated that there are various causes, including infectious, developmental, epileptic and degenerative processes and various clinical courses, which may be transient or slowly progressive. Moreover, modern imaging techniques have revealed the significance of the injury of the white matter tract, such as the frontal aslant tract, rather than classical lesions of bilateral motor cortices. Considering both the findings of the bedside examination of patients with this "old" syndrome and of "new" imaging studies performed at appropriate times is expected to contribute to our understanding of the neural underpinnings of this syndrome.