Vitelliform Macular Dystrophy

In this genetic eye disease, a fatty yellow pigment (lipofuscin) builds up in the cells under the macula. Gradually these deposits accumulate and damage the photoreceptor cells that are critical for sharp, straight ahead vision.

Best Disease

How fast the retinal disease progresses is also unpredictable since it is different for everyone who has it. One thing, however that those with this form of macular dystrophy have in common, is that normally both eyes are affected.

1) loss of central vision, and

2) blurred vision

3) distorted vision - straight lines look wavy or wiggly.

This accumulation of lipofuscin can be detected during an eye exam.

There are two forms:

1) The childhood, early-onset form known as Best Disease.

2) The adult-onset form begins later, usually in mid-adulthood, and tends to cause vision loss that worsens slowly over time.

The two genes, BEST1 and PRPH2, develop mutations which then cause this disease to develop. Obviously, BEST1 mutations are responsible for Best disease. Only in some cases does it cause the adult-onset form. Changes in the PRPH2 gene can also cause the adult-onset form.

Surprisingly, in less than a quarter of all people with this type of macular dystrophy, have these mutated genes been found. In most cases, the cause of the adult-onset form is unknown.

Genetic testing can be done to identify if someone carries the genetic mutation for this retinal condition.

Go from Vitelliform Macular Dystrophy to Juvenile Macular Degeneration

Go from Vitelliform Macular Dystrophy to Macular Degeneration

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