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author Crutch, Sebastian J.
Schott, Jonathan M.
Rabinovici, Gil D.
Murray, Melissa
Snowden, Julie S.
van der Flier, Wiesje M.
Dickerson, Bradford C.
Vandenberghe, Rik
Ahmed, Samrah
Bak, Thomas H.
Boeve, Bradley F.
Butler, Christopher
Cappa, Stefano F.
Ceccaldi, Mathieu
de Souza, Leonardo Cruz
Dubois, Bruno
Felician, Olivier
Galasko, Douglas
Graff-Radford, Jonathan
Graff-Radford, Neill R.
Hof, Patrick R.
Krolak-Salmon, Pierre
Lehmann, Manja
Magnin, Eloi
Mendez, Mario F.
Nestor, Peter J.
Onyike, Chiadi U.
Pelak, Victoria S.
Pijnenburg, Yolande
Primativo, Silvia
Rossor, Martin N.
Ryan, Natalie S.
Scheltens, Philip
Shakespeare, Timothy J.
González, Aida Suárez
Tang-Wai, David F.
Yong, Keir X. X.
Carrillo, Maria
Fox, Nick C.
author_facet Crutch, Sebastian J.
Schott, Jonathan M.
Rabinovici, Gil D.
Murray, Melissa
Snowden, Julie S.
van der Flier, Wiesje M.
Dickerson, Bradford C.
Vandenberghe, Rik
Ahmed, Samrah
Bak, Thomas H.
Boeve, Bradley F.
Butler, Christopher
Cappa, Stefano F.
Ceccaldi, Mathieu
de Souza, Leonardo Cruz
Dubois, Bruno
Felician, Olivier
Galasko, Douglas
Graff-Radford, Jonathan
Graff-Radford, Neill R.
Hof, Patrick R.
Krolak-Salmon, Pierre
Lehmann, Manja
Magnin, Eloi
Mendez, Mario F.
Nestor, Peter J.
Onyike, Chiadi U.
Pelak, Victoria S.
Pijnenburg, Yolande
Primativo, Silvia
Rossor, Martin N.
Ryan, Natalie S.
Scheltens, Philip
Shakespeare, Timothy J.
González, Aida Suárez
Tang-Wai, David F.
Yong, Keir X. X.
Carrillo, Maria
Fox, Nick C.
author_sort Crutch, Sebastian J.
collection PubMed
description 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.
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spelling pubmed-57884552018-01-29 Consensus classification of posterior cortical atrophy Crutch, Sebastian J. Schott, Jonathan M. Rabinovici, Gil D. Murray, Melissa Snowden, Julie S. van der Flier, Wiesje M. Dickerson, Bradford C. Vandenberghe, Rik Ahmed, Samrah Bak, Thomas H. Boeve, Bradley F. Butler, Christopher Cappa, Stefano F. Ceccaldi, Mathieu de Souza, Leonardo Cruz Dubois, Bruno Felician, Olivier Galasko, Douglas Graff-Radford, Jonathan Graff-Radford, Neill R. Hof, Patrick R. Krolak-Salmon, Pierre Lehmann, Manja Magnin, Eloi Mendez, Mario F. Nestor, Peter J. Onyike, Chiadi U. Pelak, Victoria S. Pijnenburg, Yolande Primativo, Silvia Rossor, Martin N. Ryan, Natalie S. Scheltens, Philip Shakespeare, Timothy J. González, Aida Suárez Tang-Wai, David F. Yong, Keir X. X. Carrillo, Maria Fox, Nick C. Alzheimers Dement Article 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. 2017-03-02 2017-08 /pmc/articles/PMC5788455/ /pubmed/28259709 http://dx.doi.org/10.1016/j.jalz.2017.01.014 Text en This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Crutch, Sebastian J.
Schott, Jonathan M.
Rabinovici, Gil D.
Murray, Melissa
Snowden, Julie S.
van der Flier, Wiesje M.
Dickerson, Bradford C.
Vandenberghe, Rik
Ahmed, Samrah
Bak, Thomas H.
Boeve, Bradley F.
Butler, Christopher
Cappa, Stefano F.
Ceccaldi, Mathieu
de Souza, Leonardo Cruz
Dubois, Bruno
Felician, Olivier
Galasko, Douglas
Graff-Radford, Jonathan
Graff-Radford, Neill R.
Hof, Patrick R.
Krolak-Salmon, Pierre
Lehmann, Manja
Magnin, Eloi
Mendez, Mario F.
Nestor, Peter J.
Onyike, Chiadi U.
Pelak, Victoria S.
Pijnenburg, Yolande
Primativo, Silvia
Rossor, Martin N.
Ryan, Natalie S.
Scheltens, Philip
Shakespeare, Timothy J.
González, Aida Suárez
Tang-Wai, David F.
Yong, Keir X. X.
Carrillo, Maria
Fox, Nick C.
Consensus classification of posterior cortical atrophy
title Consensus classification of posterior cortical atrophy
title_full Consensus classification of posterior cortical atrophy
title_fullStr Consensus classification of posterior cortical atrophy
title_full_unstemmed Consensus classification of posterior cortical atrophy
title_short Consensus classification of posterior cortical atrophy
title_sort consensus classification of posterior cortical atrophy
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5788455/
https://www.ncbi.nlm.nih.gov/pubmed/28259709
http://dx.doi.org/10.1016/j.jalz.2017.01.014
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