成人异染性脑白质营养不良视网膜的超微结构
The ultrastructure of the retina in adult metachromatic leukodystrophy.
作者信息
Goebel H H, Shimokawa K, Argyrakis A, Pilz H
出版信息
Am J Ophthalmol. 1978 Jun;85(6):841-9. doi: 10.1016/s0002-9394(14)78115-2.
A 46-year-old woman afflicted with biochemically proven metachromatic leukodystrophy had only mild optic atrophy shortly before her death. Repeated earlier ophthalmoscopic examinations had not revealed any retinal abnormalities. Light microscopy of the retina showed strong acid phosphatase activity in both enlarged ganglionic cells and pigment epithelial cells. Demyelination of both optic nerves was not noted. Ultrastructurally, membranous lysosomal residual bodies were confined to ganglionic cells. We found lipofuscin material in pigment epithelial cells, but also within metachromatic leukodystrophy-specific residual bodies of ganglionic cells. The presence of lipofuscin represents the "wear-and-tear" phenomenon, possibly enhanced by the metachromatic leukodystrophy.
一名经生化证实患有异染性脑白质营养不良的46岁女性在死前仅出现轻度视神经萎缩。早期多次眼底检查未发现任何视网膜异常。视网膜的光镜检查显示，增大的神经节细胞和色素上皮细胞中均有强烈的酸性磷酸酶活性。未发现双侧视神经脱髓鞘。超微结构上，膜性溶酶体残余体局限于神经节细胞。我们在色素上皮细胞中发现了脂褐素物质，在神经节细胞的异染性脑白质营养不良特异性残余体内也发现了脂褐素物质。脂褐素的存在代表了“磨损”现象，可能因异染性脑白质营养不良而加剧。
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