Since 1897, when R. D. Batten1 reported two cases in brothers, each of whom showed symmetrical affections in the two eyes consisting of dark spots in the macula and pallor of the optic nerve heads, a small number of cases in families have been reported.
These cases belong in the classification of familial degenerative diseases, among which those affecting the eye are the amaurotic family idiocy of Tay and Sachs; the juvenile type of amaurotic family idiocy of Spielmeyer,2 Vogt, F. E. Batten3 and others, and familial macular degeneration with and without dementia.
Between Tay-Sachs' disease on the one hand and macular degeneration without dementia on the other there are, of course, great differences; but analysis of many cases of familial disease seems to show a gradual transition from one to the other. In fact, when one attempts to put together syndromes and to give them definite
CLARK HS. FAMILIAL MACULAR DEGENERATION WITH AND WITHOUT DEMENTIA: WITH A REPORT OF TWO NEW CASES OF THE TYPE WITH DEMENTIA. JAMA. 1918;71(22):1799–1804. doi:https://doi.org/10.1001/jama.1918.02600480015005
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