卵黄様黄斑変性の一家系について

Hereditary Vilelline Macular Dystrophy in a Family

緒言

　わが国の卵黄様黄斑変性については湯口^36），塚原ら^32）33），松井ら^21）および山田^35）の報告があるが，いずれも一家系に単独に発生したもので，同一家系に2人以上みられた例はまだない。著者らは最近同胞2人の両眼に卵黄様黄斑変性を認め，母親の両眼に黄斑部変性があり，経過観察中に同胞の1人の"卵黄"が変化していく過程を観察したので報告したい。

1) An autosomal dominant family pedigree with the vitelline dystrophy of the macula was presented.

2) The vitellinc macular dystrophy was pre-sent in each eye of both of the two sibsips (8 and 10 years old, respectively). Their mother show-ed the macular degeneration on both eyes, which was presumed as the end-stage of the vitelline dystrophy.

3) The chromosome study of the peripheral white blood cells failed to reveal any anomaly in all of the three tested.

4) The electro-oculogram was pathologic, show-ing no light rise, while the erectro-retinogram was normal in all the three.