【摘要】 目的　探讨 Stargardt病的临床表现及眼底荧光血管造影和视觉电生理检查的改变 ,为临床诊断提供依据。方法　对 34例 (6 8只眼 ) Stargardt病患者进行眼底荧光血管造影 (FFA)和视觉电生理检查 (EOG、FERG、PVEP)。结果　 (1) Stargardt病患者中视力低于 0 .1者 5 4只眼 ;0 .2～ 0 .4者 10只眼 ;0 .5～ 0 .9者 4只眼。(2 ) 6 4只眼眼底黄斑区均有 1.5～ 3.2 PD大小的横椭圆形萎缩性病灶 ,呈金属样或银箔样反光 ;5 6只眼眼底有黄色斑点。(3) FFA检查 :6 4只眼黄斑部病变为强透见荧光 ,典型者呈“牛眼”状 ;合并黄色斑点病变者多数为透见荧光 ,少数为遮蔽荧光 ;6 0只眼呈现脉络膜湮没征。所有病例均未见荧光素渗漏。 (4 ) EOG:5 8只眼表现为光峰电位降低或Arden比降低 ;FERG:大部分患眼有不同程度的 ERG波形异常 ,表现为 ERGa、b波幅轻度、中度降低 ;PVEP:46只眼 P1 0 0 波幅降低和峰时延迟 ,18只眼为熄灭型。结论　 (1)眼底荧光血管造影检查可对 Stargardt病的诊断提供充分依据 ,尤其对此病的早期诊断优于其他方法 ;(2 )视觉电生理检查对 Stargardt病的诊断、病程演进及预后判断有重要参考价值更多还原

【Abstract】 Objective To investigate the clinical characteristics of the Stargardt’s disease and to evaluate the value of the fundus fluorescein angiography(FFA) and the visual eletrophysiological examination in Stargardt’s disease.Methods Routine eye examination,visual electrophysiological examination(EOG?FERG?PVEP)and FFA were performed in 34 patients(68 eyes) with Stargardt’s disease (20 men and 14 women,aged 5～40,on an average,of 19.38).Results (1)Visual acuity in 54 eyes were below 0.1(79%);10 eyes were 0.2～0.3;other were from 0.5 to 0.9.(2)64 eyes showed macular lesions that were horizontal ellipse in shape,1.5～3.2 PD in horizontal diameter and tinged with metal or beated copper color.Fundus flavimaculatus was found in 56 eyes.(3)On FFA,window defect hyperfluorescence was found in the macular lesion of ocular fundus in 64 eyes,with typical "bull’s eye" pattern.Transmitted fluorescence was found in majority with flarimaculatus,but in minority only showed blocked fluorescence.A "dark choroid" was revealed in 60 eyes.All cases had not been found the fluorescein leakage.(4)EOG:The photopeak electropotential or the ratio of Arden in 58 eyes were lower than normal;FERG:The a-wave and b-wave indicated mild or moderate descent in majority cases;PVEP:The peak value of P 100 declined and the interspike interval delayed in 46 eyes;18 eyes of the whole cases were extinguishment type.Conclusion (1)FFA may provide sufficient basis in the diagnosis of Stargardt’s disease,especially in the early stage.(2)Visual electrophysiological examination has important value in the diagnosis?the development and the judgment of prognosis in Stargardt’s disease更多还原