Reticular dystrophy of the retinal pigment epithelium. A clinical and electrophysiologic study of three generations.
Reticular dystrophy of the retinal pigment epithelium is characterized by a posterior pattern of pigment clumping like a "fishnet with knots." Four patients in three successive generations were seen with typical reticular dystrophy. A fifth patient had abnormal dark adaptation. In this family reticular dystrophy was characterized by the typical reticular pigmentary pattern, good visual acuity, normal electroretinographic findings, abnormal electro-oculographic findings, and abnormal dark adaptation. The pedigree indicates autosomal dominance as the inheritance pattern.
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D003623:Dark Adaptation; D004585:Electrooculography; D004596:Electroretinography; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D010857:Pigment Epithelium of Eye / Q000473:pathology*; D012162:Retinal Degeneration / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D014792:Visual Acuity