Inherited macular dystrophies

The term of macular dystrophies is usually applied to a group of inherited macular affections, primarily originating G-om a diffuse abnormality of the pigment epithelium and causing visible changes of the macular area and of the posterior pole of the retina. These diseases are always progressive and usually lead to decreased visual acuity more or less late ill life. All th ese conditions are transmitted as autosomal dominant or recessive traits. T remendous progress has occurred during the last decade in the identificatio n of the causal disease genes. This has promoted better comprehension of th e physiopathogeny of these diseases, may facilitate earlier diagnosis and m ay permit presymptomatic screening of high-risk individuals especially in t he age-related macular degenerations which represent the most common cause of visual impairment in the elderly.