網膜疾患の電気生理学的分析—Stargardt病と伴性遺伝性若年網膜分離症

New approach to clectrodiagnosis in diseases of the retina. (3) Analysis of Stargardt's disease and X-linked juvenile retinoschisis

（1） Stargardt病の症例では高浸透圧応答がう異常であり網膜色素上皮の障害が示唆された。

（2）伴性遺伝性若年網膜分離症の症例では網膜色素上皮および視細胞には異常は検出されず，主な異常は網膜内層にある。

（3）上記の両疾患は電気生理学的に明らかに主病変層を異にしており，電気生理学的検索は両者の鑑別に有用である。

A case of Slargarclt's disease and a case of X-linked juvenile retionoschisis without peripheral involvement were electrophysiologically studied. The examined ERG responses were the oscillatory potentials (OP), the photopic b-wave (b_D), the scotopic b-wave (b_s) and the rapid off-response. The light peak/dark trough ratio (L/D), the hyperosmolarity response and the Diamox response were tested by use of the EOG.

The case of Stargardt's disease was a 36-year-old female. Both fundi showed "beaten bronze" mac-ular atrophic lesions with perifoveal yellowish flecks in a garland fashion.