Color perception profiles in central achromatopsia.

Central achromatopsia is an impairment of color perception caused by damage to the visual association cortex. Its psychophysical underpinnings remain poorly defined. We report our attempt to characterize the defect along critical dimensions of color space, taking advantage of the same standardized tasks that allow detailed profiles in patients with retinal cone defects. We studied two patients. The results in patient 1 showed that perceptual color space was collapsed along the red-green (R-G) and short-wavelength-sensitive cone (S-cone) dimensions but that discriminations along achromatic dimensions were relatively preserved. Additional observations showed that the defect was dependent on target size, and that processing of surface and light source effects that differ from color (eg, transparency) was intact. Patient 2 showed a less severe color processing defect involving signals arising from the S-cones of the retina, although an R-G defect was also present. The profiles in these two patients demonstrate that central achromatopsia encompasses a range of color processing impairments with varied psychophysical characteristics.