Leber’s congenital amaurosis (LCA) is a rare, inherited retinal degenerative disorder that causes infants to be born with severely impaired vision. Of all the retinal degenerative diseases, LCA has the earliest age of onset and can be the most severe. LCA is part of a group of diseases called retinitis pigmentosa (RP).
RP affects the retina’s ability to respond to light. Cones and rods, the photoreceptor cells of the retina, capture and process light. When these cells break down and die, it results in progressive vision loss. With most forms of RP, the rods, which are mainly in the outer regions of the retina, degenerate first, leading to loss of peripheral and night vision. As the disease progresses, the more centrally located cones are similarly affected resulting in loss of color perception and central (reading) vision. RP is typically diagnosed in adolescents and young adults. The rate of progression and degree of visual loss varies from person to person. Most people with RP are legally blind by age 40. Currently, there is no cure for the RP group of diseases, but research in RP and Leber’s congenital amaurosis is ongoing as understanding of genetic diseases is rapidly growing.

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