Skalka H W
Am J Ophthalmol. 1979 Mar;87(3):286-91. doi: 10.1016/0002-9394(79)90064-3.
A father and son with pigmented paravenous retinochoroidal atrophy demonstrated the classical fundus appearance of bilateral sharply circumscribed patches of retinochoroidal atrophy and pigmentation along the retinal veins. Our patients, and the preponderance of males in previously reported cases of this disease, suggest the possibility of Y-chromosome mediated male-to-male transmission. Electrophysiologic findings in our patients included elevated dark-adaptation thresholds, slightly reduced electroretinographic amplitudes, slightly prolonged a- and b-wave implicit times, and (in Case 1) abnormal electro-oculographic ratios. These findings indicate a diffuse tapetoretinal degeneration, and suggest that pigmented paravenous retinochoroidal atrophy is another incomplete form of retinitis pigmentosa.
一位患有色素性静脉旁视网膜脉络膜萎缩的父子，其眼底表现为典型的双侧沿视网膜静脉分布的边界清晰的视网膜脉络膜萎缩和色素沉着斑。我们的患者以及此前报道的该疾病病例中男性占多数，提示存在Y染色体介导的男性间传播的可能性。我们患者的电生理检查结果包括暗适应阈值升高、视网膜电图振幅略有降低、a波和b波潜伏时间略有延长，以及（病例1）眼电图比率异常。这些发现表明存在弥漫性视网膜色素上皮变性，并提示色素性静脉旁视网膜脉络膜萎缩是色素性视网膜炎的另一种不完全形式。
