Three Cases of Stargadt's Disease.
- Author:
Dong Kwang CHOI
1
;
Hee Chul KIM
Author Information
1. Kong's Eye Clinic, Seoul, Korea.
- Publication Type:Case Report
- MeSH:
Adolescent;
Adult;
Humans;
Macular Degeneration;
Retinal Pigment Epithelium;
Retinal Vessels;
Scotoma;
Siblings;
Visual Acuity
- From:Journal of the Korean Ophthalmological Society
1978;19(3):295-301
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Stargardt's disease, first described in detail by Stargardt in 1909, is a recessively inherited macular dystrophy which evidences itself by reduced visual acuity, bilaterality, slowly progressive lesion, occurring familially, starting in youth (8 to 15 years). We have recently seen macular dystrophies occurring in siblings, three out of six. All developed this disease between 9 to 15 years and vision deteriorate progressively to the adult hood. Ophthalmoscopic examination revealed slight pale dise, narrowing of retinal vessels, definite alterations in the macular region associated with the development of red-yellow flecks in the center surrounded by yellow-white flecks in the paracentral region. Angiogram showed the evidence of marked alteration in the retinal pigment epithelium on the macula bilaterally. All cases were noted by ring shaped area of mottled hyperfluorescene but central flecks were nonfluorescent. Field examination showed the central scotoma to a small targets and ERG the subnormal in photopic and scotopic responces. The Ishihara test disclosed a mild to moderate red-green dyschromatopsia.
