Yagasaki K, Miyake Y, Litao R E, Ichikawa K
Doc Ophthalmol. 1986 Jun 16;63(1):73-82. doi: 10.1007/BF00153014.
An unusual form of retinal degeneration is reported in 15-year-old girl and 11-year-old girl with different pedigrees, which resembles the cases reported by Gouras and associates (1983). The subjective symptoms in these patients included decreased visual acuity, photophobia, anomalous color vision and night blindness. Electroretinograms (ERGs) in these two patients were identical in substance and revealed drastic alterations in both photopic and scotopic functions. The stimulus versus intensity response curve in a single-flash ERG showed an unusual form. This peculiar supernormal response was elicited by bright stimuli although the stimulus threshold was extremely elevated.
据报道，两名来自不同家系的15岁女孩和11岁女孩患有一种罕见的视网膜变性，其症状与Gouras及其同事（1983年）报道的病例相似。这些患者的主观症状包括视力下降、畏光、色觉异常和夜盲。这两名患者的视网膜电图（ERG）本质上相同，显示明视觉和暗视觉功能均有显著改变。单次闪光ERG中的刺激与强度反应曲线呈现出异常形态。尽管刺激阈值极高，但明亮刺激仍能引发这种特殊的超常反应。
