Posterior Cortical Atrophy

Jonathan M. Schott, BSc, MD, FRCP, FEAN, SFHEA; Sebastian J. Crutch, PhD, CPsych p. 52-75 February 2019, Vol.25, No.1 doi: 10.1212/CON.0000000000000696
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PURPOSE OF REVIEW: This article presents an overview of the clinical syndrome of posterior cortical atrophy (PCA), including its pathologic underpinnings, clinical presentation, investigation findings, diagnostic criteria, and management.

RECENT FINDINGS: PCA is usually an atypical form of Alzheimer disease with relatively young age at onset. New diagnostic criteria allow patients to be diagnosed on a syndromic basis as having a primary visual (pure) form or more complex (plus) form of PCA and, when possible, on a disease-specific basis using biomarkers or underlying pathology. Imaging techniques have demonstrated that some pathologic processes are concordant (atrophy, hypometabolism, tau deposition) with clinical symptoms and some are discordant (widespread amyloid deposition). International efforts are under way to establish the genetic underpinnings of this typically sporadic form of Alzheimer disease. In the absence of specific disease-modifying therapies, a number of practical suggestions can be offered to patients and their families to facilitate reading and activities of daily living, promote independence, and improve quality of life

SUMMARY: While rare, PCA is an important diagnostic entity for neurologists, ophthalmologists, and optometrists to recognize to allow for early accurate diagnosis and appropriate patient management. PCA provides an important opportunity to investigate the causes of selective vulnerability in Alzheimer disease.

Address correspondence to Dr Jonathan M. Schott, Dementia Research Centre, UCL Queen Square Institute of Neurology, Box 16, Queen Square, London WC1N 3BG, UK, j.schott@ucl.ac.uk.

RELATIONSHIP DISCLOSURE: Dr Schott serves on advisory boards for Biogen; Eli Lilly and Company; F Hoffman-La Roche Ltd; and Merck & Co, Inc, and on the drug safety monitoring board for AXON Neuroscience SE. Dr Schott has received personal compensation for speaking engagements for Biogen, Eli Lilly and Company, and GE Healthcare Worldwide and receives research/grant support from Alzheimer’s Research UK, Avid Radiopharmaceuticals, Brain Research UK, British Heart Foundation, Engineering and Physical Sciences Research Council (EP/J020990/1), European Commission Horizon 2020, Medical Research Council Dementias Platform UK (MR/L023784/1), Weston Brain Institute, and Wolfson Foundation. Dr Schott receives royalties from Henry Stewart Talks and Oxford University Press. Dr Crutch receives research/grant support from the Continued on page 75Alzheimer’s Society, (AS-PG-14-022), The Dunhill Medical Trust (R337/0214), the Economic and Social Research Council-National Institute for Health Research (ES/L001810/1), and the Engineering and Physical Sciences Research Council (EP/M006093/1).

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Drs Schott and Crutch report no disclosures.

© 2019 American Academy of Neurology.