This is a rare inherited form of retinal degeneration in which the retina (light sensing tissue lining the inside of the eye) degenerates.
Vision is normal until about age 50 years when it progressively worsens. The retina has an unusual glistening appearance with small cysts present. The major changes occur in the macula which is a specialized are of the retina containing the greatest concentration of light sensing cells called the rods and cones. Since this is the area that we use for our most detailed seeing tasks, our vision is most affected when it is damaged. The remainder of the eye is relatively normal.
Individuals with this condition are otherwise healthy.
Only a few families have been reported and the pattern of inheritance is characteristic of autosomal dominant inheritance. A parent with this condition can expect that on average half of his or her children will inherit the same condition. No responsible gene change has been identified.
The diagnosis is made by an ophthalmologist. No effective treatment is known but low vision aids could be helpful.