Best's Disease

Best’s disease is a disease categorized as a hereditary chorioretinal dystrophy. It is a rare, genetic disease that is characterized by a large, yellow protein that forms under the retinal pigment epithelium (RPE) in the area of the macula.
Inherited eye conditions usually present themselves early in life. Best’s disease is usually diagnosed during childhood, but the onset and severity of symptoms varies. Often, the large, fluid-filled drusen (resembling an egg yolk) beneath the RPE does not affect vision. Eventually, though, this drusen will rupture, and fluid and protein molecules will spread beneath the retina. Central vision will become worse over time, but not to the point of blindness. Best’s disease cannot be cured, but it is possible to adapt quite well to limited vision. Peripheral vision will not be affected, and one eye will often retain good central vision.
Only one copy of the gene responsible for Best’s disease is needed to cause its expression. Therefore, if one parent has the condition, there is a 50/50 chance that a child will inherit the disease. A similar form of Best’s disease that occurs later in life, and whose pattern of inheritance is unknown, is Adult Vitelliform Macular Degeneration.

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