What is best disease?

Best disease is also known as early-onset vitelliform macular dystrophy. It usually appears in childhood, but the onset of symptoms and the severity of vision loss vary widely. The adult-onset form of Best disease begins later, usually in mid-adulthood, and tends to cause vision loss that worsens slowly over time. The two forms of Best disease each have characteristic changes in the macula that can be detected during an eye examination. Over time, the abnormal accumulation of lipofuscin can damage cells that are critical for clear central vision. As a result, people with this disorder often lose their central vision, and their eyesight may become blurry or distorted. There is no current treatment for Best disease although research is on-going in the area of gene therapy which may lead to a treatment in the future.


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