Abstract
Posterior cortical atrophy (PCA), also known as Benson’s syndrome, is a neurodegenerative syndrome characterized by progressive visuospatial and visuoperceptual dysfunction. PCA most commonly occurs due to Alzheimer’s disease (AD) pathology; however, it is associated with other underlying pathologies, including dementia with Lewy bodies (DLB), corticobasal degeneration (CBD), and prion disease. Patients often exhibit symptoms at a younger age than typical amnestic AD, often in their 50s–60s, with nonspecific visual complaints that may be misinterpreted as ophthalmologic in etiology. Imaging can support the diagnosis when regional atrophy or decreased cortical FDG avidity is present, specifically in the occipitoparietal and occipitotemporal regions, noting absence of these imaging findings does not exclude the diagnosis. This chapter reviews the unique entity of PCA and its currently recognized diagnostic criteria, clinical presentation, multimodal imaging features, histopathology, and treatment options.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Similar content being viewed by others
References
Crutch SJ, Lehmann M, Schott JM, Rabinovici GD, Rossor MN, Fox NC. Posterior cortical atrophy. Lancet Neurol [Internet]. 2012;11(2):170–8. https://doi.org/10.1016/S1474-4422(11)70289-7.
Holden SK, Bettcher BM, Pelak VS. Update on posterior cortical atrophy. Curr Opin Neurol. 2020;33(1):68–73.
Crutch SJ, Schott JM, Rabinovici GD, Murray M, Snowden JS, van der Flier WM, et al. Consensus classification of posterior cortical atrophy on behalf of the Alzheimer’s association ISTAARTAtypical Alzheimer’s disease and associated syndromes professional interest area HHS public access. Alzheimers Dement [Internet]. 2017;13(8):870–84. Available from: http://creativecommons.org/licenses/by/4.0/.
Suárez-González A, Henley SM, Walton J, Crutch SJ. Posterior cortical atrophy: an atypical variant of Alzheimer disease. Psychiatr Clin North Am [Internet]. 2015;38(2):211–20. https://doi.org/10.1016/j.psc.2015.01.009.
Lehmann M, Crutch SJ, Ridgway GR, Ridha BH, Barnes J, Warrington EK, et al. Cortical thickness and voxel-based morphometry in posterior cortical atrophy and typical Alzheimer’s disease. Neurobiol Aging [Internet]. 2011;32(8):1466–76. https://doi.org/10.1016/j.neurobiolaging.2009.08.017.
Whitwell JL, Graff-Radford J, Singh TD, Drubach DA, Senjem ML, Spychalla AJ, et al. 18F-FDG PET in posterior cortical atrophy and dementia with lewy bodies. J Nucl Med. 2017;58(4):632–8.
Lundeen TF, Seibyl JP, Covington MF, Eshghi N, Kuo PH. Signs and artifacts in amyloid PET. Radiographics. 2018;38(7):2123–33.
Hof PR, Vogt BA, Bouras C, Morrison JH. Atypical form of Alzheimer’s disease with prominent posterior cortical atrophy: a review of lesion distribution and circuit disconnect in cortical visual pathways. Vis Res. 1997;37(24):3609–25.
Tang-Wai DF, Graff-Radford NR, Boeve BF, Dickson DW, Parisi JE, Crook R, Caselli RJ, Knopman DS, Peterson RC. Clincial, genetic and neuropathologic characteristics of posterior cortical atrophy. Neurology. 2004;63(7):1168–74.
Xia C, Makaretz SJ, Caso C, McGinnis S, Gomperts SN, Sepulcre J, et al. Association of in vivo [18F]AV-1451 tau PET imaging results with cortical atrophy and symptoms in typical and atypical Alzheimer disease. JAMA Neurol. 2017;74(4):427–36.
Videaud H, Torny F, Cartz-Piver L, Deschamps-Vergara N, Couratier P. Prise en charge non médicamenteuse de l’atrophie corticale postérieure : Expérience préliminaire d’un programme psychoéducatif. Rev Neurol (Paris). 2012;168(11):861–7.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2022 The Author(s), under exclusive license to Springer Nature Switzerland AG
About this chapter
Cite this chapter
Roytman, M., Ivanidze, J. (2022). Posterior Cortical Atrophy. In: Franceschi, A.M., Franceschi, D. (eds) Hybrid PET/MR Neuroimaging. Springer, Cham. https://doi.org/10.1007/978-3-030-82367-2_24
Download citation
DOI: https://doi.org/10.1007/978-3-030-82367-2_24
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-82366-5
Online ISBN: 978-3-030-82367-2
eBook Packages: MedicineMedicine (R0)