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Juvenile Macular Degeneration: Stargardt's and Best Disease

Juvenile macular degeneration is the term for several inherited eye diseases -- including Stargardt's disease, Best disease, and juvenile retinoschisis -- that affect children and young adults. These rare diseases cause central vision loss that may begin in childhood or young adulthood.

Unfortunately, there is no treatment available for these diseases, which are caused by gene mutations passed down in families. Visual aids, adaptive training, and other types of assistance can help young people with vision loss remain active. Researchers continue to look for ways to prevent and treat juvenile macular degeneration (JMD).

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Genetic counseling can help families understand these eye disorders and sort out the risks of passing them on to their children. Counseling also helps families understand how their loved one's vision is affected.

These diseases damage the macula, which is the tissue in the center of the retina at the back of the eye. The macula provides our sharp, central vision so we can do things like read and drive. It also allows us to see color and helps us recognize faces. (Age-related macular degeneration is a leading cause of vision loss in older adults.)

Below is an overview of some of these hereditary eye diseases that lead to juvenile macular degeneration.

Stargardt Disease

Stargardt disease is the most common form of juvenile macular degeneration. It's named after German ophthalmologist Karl Stargardt, who discovered it in 1901. Stargardt disease affects about one in 10,000 children in the U.S. Although the disease starts before age 20, a person may not notice vision loss until age 30 to 40.

Signs of Stargardt disease. The condition can be diagnosed by yellow-white spots that appear in and around the macula. If the spots appear throughout the back of the eye, then it is called fundus flavimaulatus. These deposits are an abnormal buildup of a fatty substance produced during normal cell activity.

Stargardt disease symptoms. Symptoms include difficulty reading and gray or black spots in the central vision. Loss of vision occurs gradually at first and affects both eyes. Once vision reaches 20/40, the disease progresses more rapidly, eventually reaching 20/200, which is legal blindness. Some people lose vision to 10/200 very quickly over a few months. Most people will have vision loss ranging from 20/100 to 20/400 by age 30 to 40.

Stargardt disease does not cause a loss of peripheral, or side, vision. Most people don't lose night vision, but may have trouble adjusting between dark and light environments. Color vision is also affected in later stages of the disease.

Genetics ofStargardt disease. Researchers have identified a specific gene that they believe causes Stargardt disease. If both parents carry one mutated gene and one normal gene, their children have a 25% chance of developing the disease. People who inherit only one mutated gene don't develop the disease. However, they can pass on the disease without knowing it.

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