Hereditary macular degeneration is characterized by bilateral degenerative changes in the macular area without a simultaneous degeneration in the central nervous system.
This hereditary- macular degeneration was first described by Rayner Battern in 1897. Since then, not only this degeneration but also many other types of hereditary macular degeneration have been described.
In 1940, Behr classified macular degeneration into six types: Infantile. juvenile, Adolescent, Adult, Presenile, Senile types. In 1973, Hughes classified this degeneration, by electro andpsychophysiologic evidence, as: 1) Progressive cone degeneration mainly affecting the photoreceptors; 2) Stargardt´s disease or fundus flavimacultus type II; 3) Best´s disease or vitelliform degeneration probably affecting primarily the basal portion of the pigment epithelium cells. 4) Doyne´s or hereditary drusen affecting Bruch´s membrane. and 5) Central choroid sclerosis affecting the choriocapillaries.
Upon reviewing the literatures relating to this disease, two case reports have been included here of hereditary macular degeneration without apparent cause which involved a brother and sister of one family.
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