HEREDOMACULAR degeneration is classified1 as one of the subtypes of primary retinal degeneration because it is said to involve the neuroepithelium (rod-cone layer), known as the primary, or first, neuron layer of the retina. The disease is characterized by involvement of the macula, in a bilateral fashion, having hereditary character but lacking any central nervous system change as part of the ocular disease.
Jonathan Hutchinson made the first contribution to this group in 1875. In 1920, when Behr2 recognized the familial tendency and indicated the probability that the change in the lesion was due to aging and stress, he subdivided the group into the infantile, juvenile, presenile, and senile types. Since then, many cases have been described, and from these it is concluded that for clinical purposes3 three rather definite forms exist and the macula assumes a different pattern in each of these types.
Type I. This
McFARLAND CB. HEREDODEGENERATION OF THE MACULA LUTEA: A Study of the Clinical and Pathologic Aspects. AMA Arch Ophthalmol. 1955;53(2):224–228. doi:10.1001/archopht.1955.00930010226009
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