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Central areolar pigment epithelial dystrophy.

V M Hermsen·G F Judisch
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Abstract

7 affected and 4 normal individuals from three consecutive generations of a family with central areolar pigment epithelial dystrophy were examined. The pedigree was indicative of an autosomal dominant disorder with variable expression. The macular changes varied from numerous small drusen-like defects to large colobomatous appearing lesions while the visual acuities ranged from 6/4 to 3/120. Some of those with excellent visual acuity had small central scotomata. Urinary metabolic screening, plasma amino acid levels and 24-hour urinary amino acid levels were normal.

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ElectrooculographyElectroretinographyFluorescein AngiographyGenes, DominantHumansMacular DegenerationPedigreePigment Epithelium of EyeJournal Article

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V M Hermsen, G F Judisch. 1984. Central areolar pigment epithelial dystrophy.. https://doi.org/10.1159/000309388

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