Severe, persistent visual impairment associated with occipital calcification and coeliac disease

While coeliac disease is primarily a disease of the digestive system, there have been several reports of neurological effects, both motor and cognitive. Here, we present the case of a woman with coeliac disease, under dietary control, in whom there is profound long-standing visual disturbance including reduction of visual fields, loss of rapid flicker and colour sensitivity and severe deficits in acuity. Structural magnetic resonance imaging (MRI) indicates large regions of calcification and abnormal tissue that is restricted to the occipital cortex, particularly the posterior region. Functional MRI indicates an absence of normal visual activation in the primary visual cortex, but at least in one hemisphere, there is neural activity to moving stimuli in visual motion area hMT+. White matter microstructure in the pathway between the lateral geniculate nucleus and hMT+ is normal compared to healthy control subjects, but is severely abnormal between the lateral geniculate nucleus and primary visual cortex. This case study illustrates the very specific nature of cortical deficit that can arise in association with coeliac disease, and highlights the importance of early dietary control for the disease.