The enhanced S cone syndrome: an analysis of receptoral and post-receptoral changes

The purpose of the study was to test the hypothesis that the retinae of patients with enhanced S cone syndrome (ESCS) have more S cones than the normal retina and these cones have replaced some of the L and M cones. Standard and spectral full-field electroretinograms, measurements of L, M, and S cone system sensitivities and S cone acuity were obtained from three patients with ESCS. The results were qualitatively consistent with the presence of more S cones and more S cone ganglion cells. To test this hypothesis further, a model of the receptoral and post-receptoral components of the S cone system was used in conjunction with psychophysical measurements of S cone system sensitivity under flashed and steady-state adaptation conditions. Within the context of the model, the data were consistent with an increase in the number of S cones and S - (L + M) ganglion cells and with a decrease in the total L + M cone input to each S - (L + M) ganglion cell.