成人型半乳糖唾液酸贮积症：3例眼部表现

Adult-form galactosialidosis: ocular findings in three cases.

作者信息

Usui T, Takagi M, Abe H, Iwata K, Tsuji S, Miyatake T

机构信息

Department of Ophthalmology, Niigata University School of Medicine, Japan.

出版信息

Ophthalmologica. 1991;203(4):176-9. doi: 10.1159/000310248.

DOI:10.1159/000310248

PMID:1784463

Abstract

We reported ocular findings of 3 patients with adult-form galactosialidosis. Diffuse fine opacities in the deep layer of the corneal stroma, an obscure cherry-red spot of the macula and optic nerve atrophy were noted in all cases. Punctate lenticular opacities were observed in 2 cases. Slowly progressive visual disturbance was seen in all cases, and it might be due to secondary optic nerve atrophy caused by retinal ganglion cell death.

摘要

我们报告了3例成人型唾液酸贮积症患者的眼部检查结果。所有病例均发现角膜基质深层弥漫性细混浊、黄斑区樱桃红斑模糊及视神经萎缩。2例观察到晶状体点状混浊。所有病例均出现缓慢进展的视力障碍，这可能是由于视网膜神经节细胞死亡导致继发性视神经萎缩所致。