Consensus classification of posterior cortical atrophy

Jonathan M. Schott, Manja Lehmann, Silvia Primativo, Martin N. Rossor, Natalie S. Ryan, Timothy J. Shakespeare, Aida Suárez González, Keir X.X. Yong, Nick C. Fox, Sebastian J. Crutch*, Melissa Murray, Julie S. Snowden, Wiesje M. van der Flier, Yolande Pijnenburg, Philip Scheltens, Bradford C. Dickerson, Rik Vandenberghe, Samrah Ahmed, Christopher Butler, Thomas H. BakBradley F. Boeve, Jonathan Graff-Radford, Stefano F. Cappa, Mathieu Ceccaldi, Leonardo Cruz de Souza, Bruno Dubois, Olivier Felician, Douglas Galasko, Neill R. Graff-Radford, Patrick R. Hof, Pierre Krolak-Salmon, Eloi Magnin, Mario F. Mendez, Peter J. Nestor, Chiadi U. Onyike, Victoria S. Pelak, David F. Tang-Wai, Maria Carrillo, Alzheimer’s Association ISTAART Atypical Alzheimer’s Disease and Associated Syndromes Professional Interest Area

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


Introduction A classification framework for posterior cortical atrophy (PCA) is proposed to improve the uniformity of definition of the syndrome in a variety of research settings. Methods Consensus statements about PCA were developed through a detailed literature review, the formation of an international multidisciplinary working party which convened on four occasions, and a Web-based quantitative survey regarding symptom frequency and the conceptualization of PCA. Results A three-level classification framework for PCA is described comprising both syndrome- and disease-level descriptions. Classification level 1 (PCA) defines the core clinical, cognitive, and neuroimaging features and exclusion criteria of the clinico-radiological syndrome. Classification level 2 (PCA-pure, PCA-plus) establishes whether, in addition to the core PCA syndrome, the core features of any other neurodegenerative syndromes are present. Classification level 3 (PCA attributable to AD [PCA-AD], Lewy body disease [PCA-LBD], corticobasal degeneration [PCA-CBD], prion disease [PCA-prion]) provides a more formal determination of the underlying cause of the PCA syndrome, based on available pathophysiological biomarker evidence. The issue of additional syndrome-level descriptors is discussed in relation to the challenges of defining stages of syndrome severity and characterizing phenotypic heterogeneity within the PCA spectrum. Discussion There was strong agreement regarding the definition of the core clinico-radiological syndrome, meaning that the current consensus statement should be regarded as a refinement, development, and extension of previous single-center PCA criteria rather than any wholesale alteration or redescription of the syndrome. The framework and terminology may facilitate the interpretation of research data across studies, be applicable across a broad range of research scenarios (e.g., behavioral interventions, pharmacological trials), and provide a foundation for future collaborative work.

Original languageEnglish
Pages (from-to)870-884
Number of pages15
JournalAlzheimer's and Dementia
Issue number8
Publication statusPublished - 1 Aug 2017

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