Stargardt's disease is another early onset macular dystrophy. However, as originally described in 1909, the majority of families demonstrate an autosomal recessive inheritance pattern. Patients affected with Stargardt's disease are characterized by atrophic macular lesions and "flecklike" deposits at the level of the retinal pigment epithelium, frequently in the midperiphery of the macula.
Retinal appearance of a patient with Stargardt-like dominant macular dystrophy. The macula appears atrophic and is surrounded by yellowish flecklike deposits.
A number of families have been described with the features of Stargardt's disease but with an autosomal dominant inheritance pattern. We used classic linkage analysis in a large family to localize the disease-causing gene to chromosome 6.
