Pathology of retinitis pigmentosa.
Pathology of retinitis pigmentosa.
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DOI：
10.1016/s0161-6420(82)34620-5
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发表时间：
1982-12
期刊：
影响因子：
13.7
通讯作者：
S. Gartner;P. Henkind
中科院分区：
文献类型：
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作者：
S. Gartner;P. Henkind
视网膜色素变性患者的眼睛是在尸检中获得的。它们在纤维素中处理，并在光镜下检查。最早的视网膜变性发生在赤道区，然后向周围和中央扩展。在最早受累的眼睛中，从受累程度较低的黄斑到赤道处退化程度较高的视网膜的过渡区可以显示出一个序列。我们的材料发生退化性变化的可能顺序如下：(1)原子核从外层核层向棒状、锥状层和外层丛状层迁移；(2)外核层光感受器及其细胞核的退化和丧失；(3)外层网状层连接纤维的损耗；(4)视网膜色素上皮（RPE）向视网膜内迁移，主要在血管周围，但也有孤立的球和斑点（这种疾病的突出特征是继发性的，仅在光感受器及其细胞核丢失之后）；(5)视网膜与视网膜色素上皮或布鲁氏膜的斑点或大面积粘连；(6)细胞核和神经节细胞层部分细胞可能发生跨神经元变性。椎间盘的胶质细胞增生是普遍的，因为椎间盘的胶质膜延伸到视网膜后，在黄斑区尤其突出。由于材料是在16至35年前获得的，我们缺乏电生理和家族性数据，并且不可能使用电子显微镜
Eyes from patients with retinitis pigmentosa were obtained at autopsy. They were processed in celloidin and examined by light microscopy. The earliest evidence of retinal degeneration occurred in the equatorial zone and then extended peripherally and centrally. In the eyes with the earliest involvement, a sequence could be demonstrated in the zone of transition from the less involved macula to the more degenerated retina at the equator. The probable order for the development of degenerative changes in our material appeared to be as follows: (1) migration of nuclei from the outer nuclear layer to the rod and cone layer and the outer plexiform layer; (2) degeneration and loss of photoreceptors and their nuclei in the outer nuclear layer; (3) loss of connecting fibers in the outer plexiform layer; (4) migration of the retinal pigment epithelium (RPE) into the retina, mainly around the blood vessels, but also as isolated balls and spots (this prominent feature, which characterizes the disease, is secondary and only follows the loss of the photoreceptors and their nuclei); (5) adhesion of the retinal to the retinal pigment epithelium or Bruch’s membrane in spots or broad areas and; (6) possible transneuronal degeneration of some cells in the inner nuclear and ganglion cell layers. Gliosis of the disc was universal as was the presence of glial membranes from the disc extending over the posterior retina, especially prominent in the macular region. As the material was obtained from 16 to 35 years ago, we lack electrophysiologic and familial data and electron microscopy was not possible