Journal
LANCET NEUROLOGY
Volume 11, Issue 2, Pages 170-178Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/S1474-4422(11)70289-7
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Funding
- AVID Radiopharmaceutical
- Department of Health's National Institute for Health Research (NIHR)
- Alzheimer's Research UK
- Alzheimer's Society
- US National Institutes of Health [NIA K23-AG031861 (PI)]
- Alzheimer's Association
- John Douglas French Alzheimer's Foundation
- Medical Research Council (UK)
- Medical Research Council [G0601846] Funding Source: researchfish
- National Institute for Health Research [NF-SI-0508-10123] Funding Source: researchfish
- MRC [G0601846] Funding Source: UKRI
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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. 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.
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