Moloney J B, Mooney D J, O'Connor M A
Am J Ophthalmol. 1983 Jul;96(1):57-65. doi: 10.1016/0002-9394(83)90455-5.
Ten patients with Stargardt's disease and 14 with fundus flavimaculatus underwent thorough ophthalmic examinations, retinal photography, and, when possible, fluorescein angiography. Retinal function was also assessed by static and kinetic perimetry, the Farnsworth-Munsell 100-hue test, electro-oculography, and electroretinography. Visual acuity and color discrimination were reduced in all patients (mean visual acuity, 20/120; mean error score, 365). On electroretinography all patients had some significant abnormality of cone function and 24 eyes had abnormal rod function (mean Vmax, 298.3). Electrooculographic findings were abnormal in 24 eyes and borderline in ten others. These abnormalities were similar in both groups but more severe in fundus flavimaculatus. Stargardt's disease and fundus flavimaculatus did not co-exist in any family studied and the mean duration of disease was similar in both, indicating that Stargardt's disease did not progress to fundus flavimaculatus. Both the age of onset and duration significantly affected the severity of fundus flavimaculatus but neither had a significant effect on Stargardt's disease.
10例患有Stargardt病的患者和14例患有黄斑部黄色斑点症的患者接受了全面的眼科检查、视网膜摄影，并在可能的情况下进行了荧光素血管造影。还通过静态和动态视野检查、法恩斯沃思-芒塞尔100色觉试验、眼电图和视网膜电图评估了视网膜功能。所有患者的视力和色觉辨别能力均下降（平均视力为20/120；平均误差评分为365）。在视网膜电图检查中，所有患者的视锥细胞功能均有一些明显异常，24只眼的视杆细胞功能异常（平均最大反应值为298.3）。24只眼的眼电图检查结果异常，另外10只眼处于临界状态。两组的这些异常情况相似，但在黄斑部黄色斑点症中更为严重。在所研究的任何家族中，Stargardt病和黄斑部黄色斑点症都不同时存在，且两者的平均病程相似，这表明Stargardt病不会发展为黄斑部黄色斑点症。发病年龄和病程均显著影响黄斑部黄色斑点症的严重程度，但对Stargardt病均无显著影响。
