The chances of such parents producing a child with the condition are 1 in 4. However, the chances of an affected parent having an affected child are very small, unless the other partner also carries the recessive gene for the condition. Stargardt s Disease is the most common form of Inherited Juvenile Macular Degeneration. Stargardt s disease is almost always inherited as an autosomal recessive disorder. It is inherited when both parents carry one gene for the disease paired with one normal gene.
Carriers are unaffected because they have only one copy of the disease gene.
In the past few years, the ABCR gene has been shown to be the cause of Stargardt s in a large number of people. It is possible in families where Stargardt s disease has occurred to screen individuals to establish whether or not they are carriers of the ABCR gene. However, it is thought that there are other genes involved in causing this condition and these tests are still only available in a research environment. You should suspect Stargardt s Disease in every young person where there is a history of progressive visual acuity loss. Electroretinography the tiny electrical currents generated by the millions of light sensitive cells in the retina in response to standardised flashes of light are measured.