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November 1, 1919


JAMA. 1919;73(18):1328-1331. doi:10.1001/jama.1919.02610440008002

In the American literature there has recently appeared the report of several cases of macular degeneration occurring in two or more members of the same childship, of a type originally described by R. D. Batten and subsequently by Stargardt, Jennings, Lutz, Darier and Pusey.

In all, nine families unquestionably suffering from this familial affection have been reported, the entire number of individual cases in these families totaling twenty-six.

The most striking feature of this disease is that it is familial; conformity to Bolling's law of heredity has been complete in all reported cases. The lesion is macular in the beginning and is rather closely confined throughout the course of the disease to this area and its immediate vicinity. Subjectively, this is evidenced by a central scotoma with no restriction of the peripheral field. The disease in all reported cases to date begins in early childhood; the fall in central vision

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