The Dystrophies of the Macula

With a genetic ntote by H. GRUNEBERG (London) FAMILIAL occurrence of lesions in the fundus situated mainly or exclusively in the central area has been recorded independently by a number of early observers: Hutchinson in 1875, Lang in 1885, Liebrecht in 1895, Batten in 1897, Haas in 1898, Doyne in 1899, Dujardin in 1904, Best in 1905, and Stargardt in 1909. As long as they constituted isolated observations the intricate problem of the unity of the different appearances shown by the families recorded and the no less complicated problem of the relationship of these cases to those showing familial macular lesions with psychic or neurological disturbances (Tay-Sachs disease and cerebro-macular degeneration) did not arise. Inevitably, inci- dental observations tended to assume an exaggerated significance in. early reports. Thus in Doyne's cases the " honey-comb appearance "-by no means a characteristic feature in some of Doyne's later cases-has dominated the discussions on this affection. Best's belief that in his cases the lesion was congenital and stationary has likewise tended to emphasize features which are probably not valid, but led Stargardt to differentiate his own cases as developing in childhood and showing a progressive course. These difficulties have become less real when subsequent copyright.