Classification of the diffuse chorioretinal heredodystrophies or peripheral tapetoretinal dystrophies.

Abstract

The best classification of the diffuse chorioretinal heredodystrophies (predominantly peripheral tapetoretinal dystrophies) is that which is based on the mode of heredity. Thus, it is possible to distinguish the autosomal recessive forms (Leber's disease, pigmentary retinopathy, Bietti's crystalline fundus dystrophy, albipunctate retinopathy, choroidal atrophy and gyrate atrophy of the chorioid and retina), the autosomal dominant forms (certain forms of pigmentary retinopathy, choroidal atrophy, progressive cone dystrophy and Sorsby's pseudo-inflammatory dystrophy), and the sex-lined forms, whether recessive (pigmentary retinopathy), dominant (pigmentary retinopathy) or intermediate (pigmentary retinopathy and choroideraemia).