Definition of Best's Disease

Best's disease is an inherited (genetic) disease that can cause progressive vision loss. Best's disease is the early-onset form of a condition known as vitelliform macular dystrophy, in which the cells of the macula, an area near the center of the retina, are damaged. Symptoms include loss of central vision and blurry or distorted vision. The condition typically does not affect side (peripheral) vision or the ability to see at night. Best's disease is caused by a mutation in a gene known as BEST1 and is inherited in an autosomal dominant manner, meaning only one copy of the defective gene (from either parent) is needed to cause the condition.