Best disease is an inherited condition that affects the macula of your eyes. You may lose central vision but keep side vision. Researchers are working on treatments, including gene therapy.
Best disease is an inherited disease that affects the retinas of your eyes. It causes the macula, which is the central part of the retina, to degrade. This means that you can have problems with your central vision, or seeing things that are right in front of you. Best disease may not affect peripheral (side) vision.
Other names for Best disease include vitelliform macular dystrophy or vitelliform dystrophy. Dystrophy is the medical name for the degeneration of an organ.
There’s a form of vitelliform macular dystrophy that doesn’t start when you’re young but instead happens when you get older, usually from 40 to 60 years of age. This is the adult-onset type of the disease.
It’s not clear how many people actually inherit Best disease. One study indicates that Best disease happens in an estimated 1 in 16,500 to 1 in 21,000 people. Another estimate puts the figure at about 1 in 15,000 people and yet another puts the figure at 1 in 10,000 people.
Best disease can go through six stages. They are:
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You’ll probably find out about Best disease after an eye exam. Sometimes there are no signs and symptoms. When there are, signs and symptoms may include:
A genetic condition causes Best disease.
Best disease is an autosomal dominant condition. This means that only one parent needs to have an altered gene to pass it on. It also means that half of the children of a parent with an autosomal trait will get that particular trait. In this case, the trait is Best disease.
The gene that’s affected in Best disease is the gene that can be affected in some types of retinitis pigmentosa, a group of inherited eye diseases.
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In many cases, a healthcare provider will find Best disease when you’re between five and 10 years old or by the time you’re 20 years old.
An eye care specialist will first take a medical history and do an eye exam. They may also ask for imaging tests and measure how thick your choroid is. Tests may include:
The eye care provider may also want to speak to family members or examine their eyes.
There’s no cure for Best disease at this time.
Managing Best disease depends on the symptoms and stage of the condition. Once it’s diagnosed, you’ll need to keep a regular schedule of eye appointments so any changes can be found early.
In early stages, you might not need any treatment. You may need treatment for other conditions, such as corrective lenses for refractive errors. People with Best disease often have farsightedness. If you have cataracts, you may need cataract surgery.
If you have choroid neovascularization and you’ve developed a choroid neovascularization membrane, the provider may suggest the following to stop the growth of new blood vessels:
Your provider may also suggest you use low vision aids like magnifying devices and other helpful tools.
Researchers are working on treatments or preventatives that use genetic material. Gene therapy is experimental.
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You can’t stop yourself from developing Best disease. If you have the genetic background to pass on Best disease, you may be interested in genetic counseling.
Best disease isn’t fatal, but it’s not curable, either. You won’t go blind from Best disease, but you may have low vision.
Certain types of foods may help with eye health. Consider eating fish a few times per week and consuming leafy green vegetables along with other types of fruits and vegetables and nuts.
If you have Best disease, you should have regular healthcare appointments, including regular eye care appointments. Let the care provider know about any changes in your vision or your health. You may want to ask questions like:
Both Stargardt disease and Best disease are genetic disorders that affect the macula. But they’re caused by changes in different genes.
Stargardt disease may lead to yellow or white flecks in your retina. Best disease may cause a large yellow oval to appear because a cyst forms under your macula.
A note from Cleveland Clinic
Living with a condition that causes low vision brings its own challenges. However, there are many resources that can make things easier if you have Best disease. Ask a healthcare provider for recommendations for services.
Last reviewed on 09/07/2022.
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Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy