Kearns-Sayre症候群の1例

A case of Kearns-Sayre syndrome in a japanese woman

緒　言

　Kearns-Sayre症候群は，その三徴候を外眼筋麻痺，網膜色素変性症，心ブロックと，定義されている^1）。我が国での本症例の報告は，少なく^2），その不全型，移行型と思われる症例もあるものと思われる。先に佐橋ら^3）がパーキンソニズムを伴つた神経筋疾患の一例として，カテコールアミン代謝異常を示唆した報告を行なつたが，著者らは，この症例の眼科的臨床所見について詳述し，若干の文献的考察を加えてみた。

Progressive external ophthalmoplegia may occur in association with other systemic abnormalities. One of them is called as Kearns-Sayre syndrome. The various combinations of clinical signs and laboratory findings are apt to be called as a new syndrome.

We have seen a 49-year-old Japanese woman with external opthalmoplegia, ptosis and many other myopathic involvements. Also neurologic abnor-malities such as bundle-branch block, deafness and pigmentary retinopathy have been observed.