Stargardt disease

Disease Overview

Stargardt disease is a genetic eye disorder that causes progressive vision loss. It affects the macula, an area of the retina responsible for sharp, central vision. Vision loss is due to abnormal accumulation of a fatty yellow pigment (lipofuscin) in the cells within the macula. People with Stargardt disease also have problems with night vision, and some have problems with color vision. The signs and symptoms of Stargardt disease typically appear in late childhood to early adulthood and worsen over time.[3073] It is most commonly caused by mutations in the ABCA4 gene and inherited in an autosomal recessive manner. Rarely it may be caused by mutations in other genes and inherited in an autosomal dominant manner.[3073][10847] There is currently no treatment, but various services and devices can help affected people carry out daily activities and maintain their independence.[10848]

Synonyms

• Stargardt macular dystrophy
• Juvenile onset macular degeneration