Armstrong J D, Meyer D, Xu S, Elfervig J L
Vitreoretinal Foundation, Memphis, Tennessee 38120, USA.
Ophthalmology. 1998 Mar;105(3):448-57; discussion 457-8. doi: 10.1016/S0161-6420(98)93026-3.
To understand better the shared characteristics of Stargardt's macular dystrophy (SMD) and fundus flavimaculatus (FF) by reviewing the clinical morphologic and retinal function changes in a large group of affected patients.
The study design was a retrospective case review.
Fifty-two patients with SMD and 48 patients with FF were observed from 1 to 22 years.
Visual acuity (VA), visual fields (VFs), fundus photographs (FPs), fluorescein angiography (FA), electro-oculography (EOG), and electroretinography (ERG) were studied at various intervals.
Changes of VA, VF, FP, FA, EOG ratio, and ERG amplitudes and implicit time at different periods in patients with SMD and patients with FF were measured.
Visual acuity decreased gradually in both the SMD and FF groups, and once the 20/200 level was reached, little further change occurred. The yellowish flecks associated with these entities faded with time, and areas of retinal pigment epithelial (RPE) and choriocapillary atrophy developed. In advanced longstanding disease, retinal vessel attenuation and peripheral pigmentary changes were noted. Ninety-four percent of patients with FF were noted to have macular dystrophy at their last visit. Patients who had only central lesions did not have peripheral lesions develop. Intrafamilial variation in the funduscopic pattern was shown in four families. The EOG ratio was abnormal in 2.6% of the patients with SMD and in 48.6% of the patients with FF. An abnormal scotopic ERG was noted in 21.1% of the patients with FF and in none of the patients with SMD. The photopic ERG was abnormal in 32.4% of the patients with FF and in 5.4% of the patients with SMD. In patients with FF, a statistical dependence was noted between the duration of the disease and the ERG results, but no such correlation was seen in the SMD group.
Morphologic changes and retinal function deterioration are more severe in patients with FF than in patients with SMD. The duration of the disease has a greater effect on patients with FF than on patients with SMD. These clinical morphologic and physiologic data may be used to supplement laboratory molecular biologic studies and aid in the further classification of these entities.
通过回顾一大组患病患者的临床形态学和视网膜功能变化，更好地了解斯塔加特黄斑营养不良（SMD）和眼底黄色斑点症（FF）的共同特征。
本研究设计为回顾性病例分析。
观察了52例SMD患者和48例FF患者，观察时间为1至22年。
在不同时间间隔对视力（VA）、视野（VF）、眼底照片（FP）、荧光素血管造影（FA）、眼电图（EOG）和视网膜电图（ERG）进行研究。
测量SMD患者和FF患者在不同时期的VA、VF、FP、FA、EOG比值以及ERG振幅和潜伏时间的变化。
SMD组和FF组的视力均逐渐下降，一旦降至20/200水平，视力变化就很小了。与这些疾病相关的黄色斑点随时间逐渐消退，视网膜色素上皮（RPE）和脉络膜毛细血管萎缩区域出现。在晚期长期患病的患者中，可观察到视网膜血管变细和周边色素沉着改变。94%的FF患者在最后一次就诊时被发现患有黄斑营养不良。仅有中心病变的患者未出现周边病变。在四个家族中显示出眼底镜检查模式的家族内差异。2.6%的SMD患者和48.6%的FF患者EOG比值异常。21.1%的FF患者暗视ERG异常，而SMD患者均无异常。32.4%的FF患者明视ERG异常，5.4%的SMD患者明视ERG异常。在FF患者中，观察到疾病持续时间与ERG结果之间存在统计学相关性，但在SMD组中未观察到这种相关性。
FF患者的形态学变化和视网膜功能恶化比SMD患者更严重。疾病持续时间对FF患者的影响比对SMD患者更大。这些临床形态学和生理学数据可用于补充实验室分子生物学研究，并有助于对这些疾病进行进一步分类。
