• Sebastian J Crutch, Manja Lehmann, Jonathan M Schott, Gil D Rabinovici, Martin N Rossor, and Nick C Fox.
• Dementia Research Centre, Institute of Neurology, University College London, London, UK. s.crutch@drc.ion.ucl.ac.uk
• Lancet Neurol. 2012 Feb 1; 11 (2): 170178170-8.

AbstractPosterior 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. This heterogeneity has led to inconsistencies in diagnosis and terminology and difficulties in comparing studies from different centres, and has restricted the generalisability of findings from clinical trials and investigations of factors that drive phenotypic variability. Important challenges remain, including the identification of factors associated not only with the selective vulnerability of posterior cortical regions but also with the young age of onset of PCA. Greater awareness of the syndrome and agreement over the correspondence between syndrome-level and disease-level classifications are needed to improve diagnostic accuracy, clinical management, and the design of research studies.Copyright © 2012 Elsevier Ltd. All rights reserved.

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