Progressive cortical visual failure associated with occipital calcification and coeliac disease with relative preservation of the dorsal 'action' pathway

We describe the first reported case of a patient with coeliac disease and cerebral occipital calcification who shows a progressive and seemingly selective failure to recognise visual stimuli. This decline was tracked over a study period of 22 years and occurred in the absence of primary sensory or widespread intellectual impairment. Subsequent tests revealed that although the patient was unable to use shape and contour information to visually identify objects, she was nevertheless able to use this information to reach, grasp and manipulate objects under central, immediate vision. This preservation of visuo-motor control was echoed in her day-to-day ability to navigate and live at home independently. We conclude that occipital calcification following coeliac disease can lead to prominent higher visual failure that, under prescribed viewing conditions, is consistent with separable mechanisms for visual perception and action control.