Pathologic findings in the retinal pigment epitheliopathy associated with the amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam.
Pathologic findings in the retinal pigment epitheliopathy associated with the amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam.
复制标题
与关岛肌萎缩侧索硬化症/帕金森病-痴呆症相关的视网膜色素上皮病的病理学发现。
DOI：
10.1016/s0161-6420(93)31696-9
复制
发表时间：
1993
期刊：
影响因子：
13.7
通讯作者：
Kurland,LT
中科院分区：
文献类型：
--
作者：
Campbell,RJ;Steele,JC;Cox,TA;Loerzel,AJ;Belli,M;Belli,DD;Kurland,LT
背景：一种色素上皮病，类似于眼内丝虫病发生在大约10%的大样本的查莫罗人口的关岛年龄39岁或以上，该比率约为50%，其中有肌萎缩性侧索硬化症或帕金森氏症痴呆综合征（lytico bodig）。这是第一个病理报告的视网膜色素上皮病变中看到的设置肌萎缩侧索硬化症/帕金森氏症痴呆症复杂的关岛，是基于检查13只眼睛从7 patients.Results/结论：与细胞内色素减少相关的funduscopic和肉眼外观的视网膜色素上皮细胞的衰减的局灶性区域。没有看到幼虫，也没有炎症的证据。这种色素上皮病的发病机制尚未确定。
Background:A pigment epitheliopathy that resembles ophthalmomyiasis interna occurs in approximately 10% of a large sample of the Chamorro population of Guam age 39 years or older; the rate is approximately 50% among those who have amyotrophic lateral sclerosis or Parkinsonism-dementia complex (lytico bodig).Methods:Since publication of an earlier clinical report of affected patients, several of them have died of their neurologic disease, and their eyes were obtained for pathologic study. This is the first pathologic report of the retinal pigment epitheliopathy seen in the setting of amyotrophic lateral sclerosis/Parkinsonism-dementia complex of Guam and is based on examination of 13 eyes from 7 patients.Results/Conclusion:Focal areas of attenuation of the retinal pigment epithelium in association with a reduced amount of intracellular pigment correlated with the funduscopic and gross appearance. No larvae were seen, and there was no evidence of inflammation. The pathogenesis of this pigment epitheliopathy remains undetermined.