FAMILIAL CEREBRO‐MACULAR DEGENERATION (THE STENGEL‐BATTEN‐MAYOU‐SPIELMEYER‐VOGT‐STOCK DISEASE): EVALUATION OF THE PHOTORECEPTORS

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Abstract
In material from 7 patients with familial cerebro-macular degeneration, a grave visual disability developed within a few yr after disease onset at 3 1/2-7 yr of age. Night vision was only moderately reduced. A severe red-green color vision defect was demonstrated in 3 patients. This is in accord with the loss of red and green cone responses found in 1 patient by chromatic adaptation studies. A remarkably good response of the blue cone system was registered. Normal pulse amplitudes were found by dynamic tonometry in 4 patients indicating good choroidal circulation. Degeneration of the neuroepithelium apparently is of primary type. This selectivity of response function loss might also be related to parallel degenerations taking place in ganglion cells.

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