本病比较少见,为家族遗传性眼病,曾见到两例并做了家系调查报导于下。例1 史××女48岁。已婚安徽籍主诉自幼暗视力差。近10年来症状加重,视野逐渐缩小,夜间难以行走。眼部检查:视力右眼0.02,左眼0.1,不能矫正。外眼无特殊发现。眼底检查:整个眼底遍布结晶样闪辉亮点,大小一致,互不连接,越向黄斑中心越密集,周边部较少,偶见褐黑色不规则色素块,黄斑部未被波及。裂隙灯加接触镜检查:白闪光亮点多位于视网膜血管后面不隆起,视乳头苍白边介清楚,动脉狭窄,静 The disease is relatively rare, for family hereditary eye disease, had seen two cases and made a pedigree survey reported in the next. Example 1 history × × female 48 years old. Married Anhui nationality complained of poor visual acuity since childhood. In the past 10 years, the symptoms have worsened, the visual field gradually narrowed, and it was difficult to walk at night. Eye examination: right eye 0.02, left eye 0.1, can not be corrected. No special eye outside the discovery. Fundus examination: the entire fundus throughout the crystal-like shimmering bright spots, the same size, do not connect each other, the more dense the more the center of the macular, fewer peripheral parts, and occasionally irregular black brown pigment block, the macula has not been affected. Slit lamp plus contact lens examination: white flash highlights are located in the back of the retinal blood vessels do not bulge, clear edge of the optic nerve, narrow artery, stenosis