周辺部に骨小体様白斑を伴つた黄斑変性症

Macular Degeneration with Irregular White Patches in the Peripheral Fundus

I．緒言

　黄斑部の変性性疾患に関しては，古来幾多の報告があるが，Best （1905），Stargardt （1909），Oatmann （1911），Behr （1920）らが，遺伝性，家族性黄斑変性症を多数，詳細に報告し，本疾患を体系づけている。しかし，なお症例によつては，鑑別診断に困難を感ずることも少なくない。以下に報告するものは，家族性，遺伝性に発生したと思われ，網膜周辺部に骨小体様，さんご状の小白斑を伴つた黄斑変性で，その鑑別診断に困難を感じた症例である。

A report is presented of a male of 23 years of age in whom gradual visual deterioration set in since the age of 7 years. Corrected vision was 0.2 each. Relative scotoma and metamorphopsia was present in the central visual field, while the peripheral visual field was intact except for a slight nasal defect.

The macular area showed irregularly brazen appearance indicative of degenerative processes (shown as Fig.2 in the text). The peripheral fundus was diffusely atrophic and was strewn with irregular white patches assuming the shape of bone corpuscles. Dark adaptation was only moderately impaired.