Inherited Retinal Degenerations

In this issue of IrJO Movassat et al1 (P: 33-38) present a case series of leber s congenital amaurosis (LCA). LCA is the most severe form of inherited retinal dystrophy responsible for congenital blindness with an early age of onset and accounts for at least 5% of all inherited retinal diseases which affect about one in 3500 individuals in the world. Usually degenerative anomalies of the retina initially affect the rods which cause night-blindness and regression of the visual fields, but progressively the cones, retinal vessels and other cells get involved in this degenerative process. In the past decade many efforts have been focused on the genetics and pathogenesis of these blinding diseases. Although, more than 100 genes have been discovered for these degenerative diseases, we are at the beginning of a long road