Cerebral achromatopsia

Clinic

• Cerebral achromatopsia is a type of color-blindness caused by damage to the cerebral cortex of the brain, rather than abnormalities in the cells of the eye's retina.

• It is often confused with congenital achromatopsia but underlying physiological deficits of the disorders are completely distinct.
• A similar, but distinct, deficit called color agnosia exists in which a person has intact color perception (as measured by a matching task) but has deficits in color recognition, such as knowing which color they are looking at.

• Pathophysiology: Cortical damage esp ventral occipitotemporal cortex

Co-occurrence with other deficits

• Prosopagnosia 72%.
• Poor spatial acuity

Research

• Probably it is related to CJD
• Approximately 10–18% of patients with sCJD report visual symptoms include blurred vision, nystagmus, diplopia, metamorphopsias, dyschromatopsia, supranuclear palsies, visuospatial disturbances, saccades, visual hallucinations, and anopsia (Cooper et al., 2005; Kropp et al., 1999; Lenk et al., 2018; Lueck et al., 2000; Ntantos et al., 2018; Proulx et al., 2008; Rabinovici et al., 2006; Will and Matthews, 1984; Wong et al., 2015). In the Heidenhain variant of sCJD, the visual symptoms are thought to arise from neurodegeneration of the visual occipital cortex (Baiardi et al., 2016). In other forms of sCJD, the neuroanatomic defects that underlie visual symptoms are less clear.