Szamier R B, Berson E L
Invest Ophthalmol Vis Sci. 1982 May;22(5):559-70.
An ultrastructural study was done on a postmortem donor eye from an 84-year-old male with night blindness, pericentral ring scotomas, attenuated retinal arterioles, and intraretinal bone spicule pigmentation distributed around the midperiphery. Unusual findings observed 3 years prior to death were preserved central vision and peripheral visual field and rod and cone electroretinogram amplitudes reduced only about 60% below normal. Ultrastructural examination of the retina revealed apparently normal numbers of cones in the fovea, reduced numbers of rods on the foveal slope, absence of photoreceptors in the midperiphery, and large areas of preserved rods and cones in the far periphery in all quadrants. Other conspicuous findings were the presence of autophagic vacuoles in remaining cones and nonpigmented epithelial cells around some retinal vessels in the midperiphery. Comparison of visual acuities, visual fields, and fundus appearance reported at age 54 and at age 81, combined with ultrastructural observations of the retina at age 84, suggested that this patient's condition was minimally, if at all, progressive over the last 30 years of his life. Classification of this disease as pericentral retinitis pigmentosa is considered.
对一名84岁患有夜盲症、中心周围环形暗点、视网膜小动脉变细以及视网膜中周部出现骨针状色素沉着的男性供体死后眼球进行了超微结构研究。在死亡前3年观察到的异常发现是中心视力和周边视野得以保留，视杆和视锥细胞视网膜电图振幅仅比正常水平降低约60%。视网膜的超微结构检查显示，黄斑中心凹处视锥细胞数量明显正常，黄斑斜坡处视杆细胞数量减少，中周部无感光细胞，所有象限的远周边部有大片视杆和视锥细胞得以保留。其他明显的发现是，中周部一些视网膜血管周围剩余的视锥细胞和无色素上皮细胞中存在自噬空泡。54岁和81岁时报告的视力、视野和眼底外观情况，结合84岁时视网膜的超微结构观察结果，表明该患者的病情在其生命的最后30年中即使有进展也是微乎其微的。考虑将这种疾病归类为中心性视网膜色素变性。
