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高度の視力障害,羞明,先天性眼球振盪を伴った強度の色覚異常を有する兄妹について検索した.
両症例の視力は両眼ともに0.1(0.1)であったが,眼底には異常がみられなかった.各種色覚検査表で強度異常が示され,色相配列検査では赤緑異常様のパターンを呈した.
兄の4°部位における暗順応下スペクトル感度からはCIE暗所視比視感度類似の反応が得られた.また,中心部位における白色順応下スペクトル感度測定の結果,錐体系の反応が得られたが.正常者と比較し長波長側で感度の低下がみられた.しかしながら,第1色弱例のものよりは長波長側で感度は良好であった.Rayleigh均等において赤-緑の混色目盛りは赤側に移行がみられたが,単色目盛りは第1色弱と比較し大きな値を呈した.
以上の結果から,本症例は杆体系および錐体系の反応を有するものの,特に赤および緑錐体の視色素のoptical densityの減少によるincomplete achromatopsiaの一種と推察した.
We observed strong dyschromatopsia in a 9-year-old boy and his siter aged 5 years. Both manifested reduced visual acuity at 0.1 in either eye. No fundus abnormalities were present. They could not read any of Ishihara's plates except Nr. 1. They made over 400 errors on the Farnsworth-Munsell 100-hue test with a red-green axis. Spectral sensitivities of the brother at 4° temporally at scotopic levels were similar to the CIE scotopic curve. His spectral sen-sitivity at the fovea at photopic levels showed a reduced cone response in the long wavelength re-gion. It was higher than in protanomalies. The red-green settings of color matchings shifted towards red. Yellow settings showed a higher number than in protanomalous settings.
The findings indicate that the cases manifest a kind of incomplete achromatopsia with reduced ef-fective opitcal density of red and green cones.
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