与关岛肌萎缩侧索硬化症/帕金森病-痴呆综合征相关的视网膜色素上皮病变的病理发现。
Pathologic findings in the retinal pigment epitheliopathy associated with the amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam.
作者信息
Campbell R J, Steele J C, Cox T A, Loerzel A J, Belli M, Belli D D, Kurland L T
机构信息
Department of Ophthalmology, Mayo Clinic, Rochester, MN 55905.
出版信息
Ophthalmology. 1993 Jan;100(1):37-42. doi: 10.1016/s0161-6420(93)31696-9.
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.
背景
在关岛39岁及以上的查莫罗人群的大量样本中，约10%出现了一种类似于眼内蝇蛆病的色素上皮病变；在患有肌萎缩侧索硬化症或帕金森病痴呆综合征（lytico bodig）的人群中，这一比例约为50%。
方法
自早期关于受影响患者的临床报告发表以来，其中一些患者已死于神经疾病，他们的眼睛被用于病理研究。这是在关岛肌萎缩侧索硬化症/帕金森病痴呆综合征背景下所见视网膜色素上皮病变的首份病理报告，基于对7名患者的13只眼睛的检查。
结果/结论：视网膜色素上皮的局灶性萎缩区域与细胞内色素量减少相关，这与眼底镜检查和大体外观相符。未发现幼虫，也没有炎症迹象。这种色素上皮病变的发病机制仍未确定。
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