Cerebral achromatopsia

Abstract

AbstractChromatic sensitivity was assessed in patients with pregeniculate and postgeniculate lesions. We examine changes in central vision along at foveal fixation and in each of the four paracentral quadrants of the visual field using the Colour Assessment and Diagnosis (CAD) test, a dynamic luminance contrast masking technique that isolates the use of Red / Green (RG) and Yellow / Blue (YB) colour signals. Because the extraction of colour signals in early visual processing involves opponent mechanisms, subjects with congenital RG loss of sensitivity exhibit symmetric increase in thresholds towards the long wavelength (“red”) and middle wavelength (“green”) regions of the spectrum locus. This is also frequently the case with acquired loss of chromatic sensitivity as a result of retinal or optic nerve disease. The findings from this study represent an exception to this rule. The patients investigated showed a marked asymmetry in colour thresholds. The study reveals how discrete, localized damage to visual pathways can cause selective loss of visual function and how the latter varies with retinal topography.The 36 pregeniculate patients investigated exhibited impairment in both RG and YB. Loss of colour vision was symmetrical for both RG and YB discrimination. Some pregeniculate patients exhibited substantial loss of colour vision in areas where perimetric loss was largely absent. All quadrants revealed similar symmetric loss of colour sensitivity.In 23 patients with postgeniculate lesions, patients with pre‐striate damage exhibited symmetric loss of chromatic sensitivity which affected either one or both chromatic mechanisms. Striate or extra‐striate lesions tended to exhibit general loss of colour vision within the area identified by visual field testing. More specific chromatic loss was associated with less impaired areas that were often normal on perimetric testing. Some patients with striate or extra‐striate lesions presented with asymmetric chromatic loss for one or more colour categories. When striate or extra‐striate lesions were also accompanied by underlying pre‐striate damage, chromatic sensitivity loss was always symmetric.The findings from this study show that the loss of chromatic sensitivity in cerebral achromatopsia depends strongly on the exact location and extent of the lesion with significant variation over the visual field. The same subject can exhibit loss of chromatic sensitivity that is either RG or YB specific, but also, spatially localized, non‐opponent loss of sensitivity for primary colours.