Enhanced S cone syndrome: evidence for an abnormally large number of S cones.
The cellular basis of the hypersensitivity of the S (blue) cone system in patients with enhanced S cone syndrome was examined by analyzing ERGs from three patients. The patients had large alpha-waves in response to the blue and white flashes. These alpha-waves were shown to be driven nearly entirely by the S cones. Although these S cone alpha-waves were 4-6 times the size of the normal L/M cone alpha-wave, they are of the same form, and could be quantitatively described with the same model previously shown to fit cone alpha-waves. We propose that the retina of these patients has many more S cones than the normal retina and that these cones replace some of the normal L/M cones and many of the rods.
Hood
D C
DC
Department of Psychology, Columbia University, New York, NY 10027, USA.
Cideciyan
A V
AV
Roman
A J
AJ
Jacobson
S G
SG
D000293:Adolescent; D002648:Child; D003117:Color Vision Defects / Q000503:physiopathology; D004596:Electroretinography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010775:Photic Stimulation; D017949:Retinal Cone Photoreceptor Cells / Q000503:physiopathology*; D012162:Retinal Degeneration / Q000503:physiopathology*; D013577:Syndrome