Stargardt's Disease: A Hereditary Form of Vision Loss
Medical Author: Melissa Conrad Stoppler, MD
Stargardt's disease (also known as Stargardt's macular dystrophy) is a hereditary, or inheritable, form of the eye condition known as macular degeneration. In macular degeneration, the macula (the center part of the retina of the eye where vision is sharpest) deteriorates, leading to a loss in central vision that interferes with reading, driving, and other daily activities. In Stargardt's disease, the symptoms of vision loss often begin as early as age 6 or 7. Since the macula is also responsible for our sharpest color vision, those with Stargardt's disease also have a decrease in color vision.
The disease is named for a German ophthalmologist, Karl Stargardt, who described the condition in the early 20th century. About 25,000 people in the U.S. have Stargardt's disease, which usually follows a rapidly progressive course that can lead to legal blindness, although peripheral vision generally remains intact and the overall degree of vision loss varies among those affected. The retinal damage often also leads to an increase in sensitivity to light (photophobia).