Best Post of September 2018: A Case of Posterior Cortical Atrophy

The next in our "Best of the Month" series is from September 7, 2018:

Posterior cortical atrophy (PCA) is a neurodegenerative syndrome that is characterised by progressive decline in visuospatial, visuoperceptual, literacy, and praxic skills. The progressive neurodegeneration affecting parietal, occipital, and occipitotemporal cortices that underlies PCA is attributable to Alzheimer's disease in most patients. However, alternative underlying causes, including dementia with Lewy bodies, corticobasal degeneration, and prion disease, have also been identified, and not all patients with PCA have atrophy on clinical imaging. (Crutch, S. J., Lehmann, M., Schott, J. M., Rabinovici, G. D., Rossor, M. N., & Fox, N. C. (2012). Posterior cortical atrophy. Lancet Neurology, 11(2), 170-178)

This is a case of a 60-year-old female with PCA:

Although there is diffuse neocortical atrophy, note the prominent occipital lobe atrophy

Tau stain of temporal lobe section highlights Alzheimer pathology

Pallor of the substantia nigra was noted, and Lewy bodies noted microscopically (see inset)

Alpha-synuclein staining showed neocortical Lewy bodies (here in the frontal lobe)

The final diagnosis was Alzheimer disease and Diffuse Neocortical Lewy Body Disease in the setting of Posterior Cortical Atrophy.