Stargardt disease

An hereditary eye disease that is one of the most frequent causes of macular degeneration in childhood. (The macula is the part of the retina that is responsible for central vision.) The disease has its onset between the ages of 7 and 12 years, follows a rapidly progressive course, and arrives at a poor final visual outcome. Although the visual acuity is severely reduced, peripheral vision remains normal throughout life. People with Stargardt disease have a hole in the center of their vision. One, a runner named Marla Runyan, cannot see the tape at the finish line. She can see the track beneath her feet with peripheral vision, but her competitors are visible to her only as "shards of color and smudged faces and gauzy hairstyles." There are several genetic forms of Stargardt disease. One is caused by mutation (change) in a gene called the ABCR gene on chromosome 1. The disease is named for a German ophthalmologist, Karl Stargardt, who described and illustrated it in 1909.

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an autosomal recessive type of macular degeneration, usually occurring between the ages of six and twenty, marked by abnormal pigmentation and other changes in the macular area with rapid loss of visual acuity. Called also Stargardt macular degeneration.

Medical dictionary. 2011.

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