occipital cortical dysgenesis

A Rare Visuospatial Disorder

Cases with visuospatial abnormalities provide opportunities for understanding the underlying cognitive mechanisms. Three cases of visual mirror-reversal have been reported: AH (McCloskey, 2009), TM (McCloskey, Valtonen, & Sherman, 2006) and PR (Pflugshaupt et al., 2007). This research reports a fourth case, BS -- with focal occipital cortical dysgenesis -- who displays highly unusual visuospatial abnormalities. They initially produced mirror reversal errors similar to those of AH, who -- like the patient in question -- showed a selective developmental deficit. Extensive examination of BS revealed phenomena such as: mirror reversal errors (sometimes affecting only parts of the visual fields) in both horizontal and vertical planes; subjective representation of visual objects and words in distinct left and right visual fields; subjective duplication of objects of visual attention (not due to diplopia); uncertainty regarding the canonical upright orientation of everyday objects; mirror reversals during saccadic eye movements on oculomotor tasks; and failure to integrate visual with other sensory inputs (e.g., they feel themself moving backwards when visual information shows they are moving forward). Fewer errors are produced under conditions of certain visual variables. These and other findings have led the researchers to conclude that BS draws upon a subjective representation of visual space that is structured phenomenally much as it is anatomically in early visual cortex (i.e., rotated through 180 degrees, split into left and right fields, etc.). Despite this, BS functions remarkably well in their everyday life, apparently due to extensive compensatory mechanisms deployed at higher (executive) processing levels beyond the visual modality.