Stargardt氏網膜黃斑部變性症－家族病例報告

Stargardt's Disease-Two Cases in Sublings

10.30048/ACTASOS.199112.0159

曾國亮(Gow-Lieng Tseng)；劉秀雯(Shiow-Wen Liou)

Stargardt's disease ； fundus flavimaculatus

台灣眼科醫學會會刊

30卷4期（1991 / 12 / 04）

191 - 196

繁體中文

本篇報告兩位罹患Stargardt氏網膜黃斑部變性症的兄妹，患者的視力從小不良，且有逐漸惡化的現象，眼底呈現卵圓形或牛眼樣黃斑部萎縮性退化，黃斑部週圍偶有色素斑分散，但沒有黃色斑點，眼底螢光攝影呈現特殊的黃斑部早期螢光增強，而脈絡膜螢光減少的現象；視野檢查有中心暗點，辨色力檢查有紅色弱，眼電氣生理檢查(EOG、ERG)則為正常。

Two cases of Stargardt's disease in sublings were studied. Both of them complained of markedly decreased vision since childhood and central scotoma of both visual fields. The fundus appearance showed characteristic atrophic macular degeneration without flecks. Fluorescence angiography showed macular hyperfluorescence on a dark background of minimal choroidal fluorescence. According to the family history, recessive type of inheritance was considered.