It is characterized by degeneration of the retina and of the choroid. It has an X-linked inheritance pattern and affects males. Female carriers may experience mild symptoms of the disease. This retinal degenerative disease is associated with a deficiency in the enzyme ornithine aminotransferase. Myopia, night blindness, reduction in peripheral vision and cataracts are characteristic of this syndrome.
Gyrate atrophy is an autosomal recessive disease.
Leber Congenital Amaurosis Leber congenital amaurosis is characterized by severe visual impairment from birth or very early childhood. It is an autosomal recessive disease. Leber congenital amaurosis causes visual problems that are different from those of Leber optic neuropathy, a condition that is not caused by primary degeneration of the retina. Early onset forms are inherited macular degenerations that include Best disease, Stargardt disease, fundus flavimaculatus and other rare macular dystrophies like Sorsby's macular dystrophy and North Carolina macular dystrophy. Age-related macular degeneration is the leading cause of central vision loss in people over the age of 60.