Consensus classification of posterior cortical atrophy

Sebastian J. Crutch, Jonathan M. Schott, Gil D. Rabinovici, Melissa Murray, Julie S. Snowden, Wiesje M. van der Flier, Bradford C. Dickerson, Rik Vandenberghe, Samrah Ahmed, Thomas H. Bak, Bradley F. Boeve, Christopher Butler, Melissa E Murray, Mathieu Ceccaldi, Leonardo Cruz de Souza, Bruno Dubois, Olivier Felician, Douglas Galasko, Jonathan Graff-Radford, Neill R. Graff-RadfordPatrick R. Hof, Pierre Krolak-Salmon, Manja Lehmann, Eloi Magnin, Mario F. Mendez, Peter J. Nestor, Bradley F Boeve, Jonathan Graff-Radford, Yolande Pijnenburg, Silvia Primativo, Martin N. Rossor, Natalie S. Ryan, Philip Scheltens, Timothy J. Shakespeare, Aida Suárez González, Neill R Graff Radford, Keir X X Yong, Maria Carrillo, Nick C. Fox

Research output: Contribution to journalArticle

96 Citations (Scopus)

Abstract

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 (US)
JournalAlzheimer's and Dementia
DOIs
StateAccepted/In press - 2017

Fingerprint

Atrophy
Consensus
Research
Lewy Body Disease
Prion Diseases
Terminology
Neuroimaging
Biomarkers
Pharmacology

Keywords

  • Alzheimer's disease
  • Biomarker
  • Clinico-radiological syndrome
  • Pathophysiology
  • Posterior cortical atrophy

ASJC Scopus subject areas

  • Epidemiology
  • Health Policy
  • Developmental Neuroscience
  • Geriatrics and Gerontology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Psychiatry and Mental health

Cite this

Crutch, S. J., Schott, J. M., Rabinovici, G. D., Murray, M., Snowden, J. S., van der Flier, W. M., ... Fox, N. C. (Accepted/In press). Consensus classification of posterior cortical atrophy. Alzheimer's and Dementia. https://doi.org/10.1016/j.jalz.2017.01.014

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; Murray, Melissa E; 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.; Boeve, Bradley F; Graff-Radford, Jonathan; Pijnenburg, Yolande; Primativo, Silvia; Rossor, Martin N.; Ryan, Natalie S.; Scheltens, Philip; Shakespeare, Timothy J.; Suárez González, Aida; Graff Radford, Neill R; Yong, Keir X X; Carrillo, Maria; Fox, Nick C.

In: Alzheimer's and Dementia, 2017.

Research output: Contribution to journalArticle

Crutch, SJ, Schott, JM, Rabinovici, GD, Murray, M, Snowden, JS, van der Flier, WM, Dickerson, BC, Vandenberghe, R, Ahmed, S, Bak, TH, Boeve, BF, Butler, C, Murray, ME, Ceccaldi, M, de Souza, LC, Dubois, B, Felician, O, Galasko, D, Graff-Radford, J, Graff-Radford, NR, Hof, PR, Krolak-Salmon, P, Lehmann, M, Magnin, E, Mendez, MF, Nestor, PJ, Boeve, BF, Graff-Radford, J, Pijnenburg, Y, Primativo, S, Rossor, MN, Ryan, NS, Scheltens, P, Shakespeare, TJ, Suárez González, A, Graff Radford, NR, Yong, KXX, Carrillo, M & Fox, NC 2017, 'Consensus classification of posterior cortical atrophy', Alzheimer's and Dementia. https://doi.org/10.1016/j.jalz.2017.01.014
Crutch SJ, Schott JM, Rabinovici GD, Murray M, Snowden JS, van der Flier WM et al. Consensus classification of posterior cortical atrophy. Alzheimer's and Dementia. 2017. https://doi.org/10.1016/j.jalz.2017.01.014
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 ; Murray, Melissa E ; 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. ; Boeve, Bradley F ; Graff-Radford, Jonathan ; Pijnenburg, Yolande ; Primativo, Silvia ; Rossor, Martin N. ; Ryan, Natalie S. ; Scheltens, Philip ; Shakespeare, Timothy J. ; Suárez González, Aida ; Graff Radford, Neill R ; Yong, Keir X X ; Carrillo, Maria ; Fox, Nick C. / Consensus classification of posterior cortical atrophy. In: Alzheimer's and Dementia. 2017.
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title = "Consensus classification of posterior cortical atrophy",
abstract = "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.",
keywords = "Alzheimer's disease, Biomarker, Clinico-radiological syndrome, Pathophysiology, Posterior cortical atrophy",
author = "Crutch, {Sebastian J.} and Schott, {Jonathan M.} and Rabinovici, {Gil D.} and Melissa Murray and Snowden, {Julie S.} and {van der Flier}, {Wiesje M.} and Dickerson, {Bradford C.} and Rik Vandenberghe and Samrah Ahmed and Bak, {Thomas H.} and Boeve, {Bradley F.} and Christopher Butler and Murray, {Melissa E} and Mathieu Ceccaldi and {de Souza}, {Leonardo Cruz} and Bruno Dubois and Olivier Felician and Douglas Galasko and Jonathan Graff-Radford and Graff-Radford, {Neill R.} and Hof, {Patrick R.} and Pierre Krolak-Salmon and Manja Lehmann and Eloi Magnin and Mendez, {Mario F.} and Nestor, {Peter J.} and Boeve, {Bradley F} and Jonathan Graff-Radford and Yolande Pijnenburg and Silvia Primativo and Rossor, {Martin N.} and Ryan, {Natalie S.} and Philip Scheltens and Shakespeare, {Timothy J.} and {Su{\'a}rez Gonz{\'a}lez}, Aida and {Graff Radford}, {Neill R} and Yong, {Keir X X} and Maria Carrillo and Fox, {Nick C.}",
year = "2017",
doi = "10.1016/j.jalz.2017.01.014",
language = "English (US)",
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T1 - Consensus classification of posterior cortical atrophy

AU - Crutch, Sebastian J.

AU - Schott, Jonathan M.

AU - Rabinovici, Gil D.

AU - Murray, Melissa

AU - Snowden, Julie S.

AU - van der Flier, Wiesje M.

AU - Dickerson, Bradford C.

AU - Vandenberghe, Rik

AU - Ahmed, Samrah

AU - Bak, Thomas H.

AU - Boeve, Bradley F.

AU - Butler, Christopher

AU - Murray, Melissa E

AU - Ceccaldi, Mathieu

AU - de Souza, Leonardo Cruz

AU - Dubois, Bruno

AU - Felician, Olivier

AU - Galasko, Douglas

AU - Graff-Radford, Jonathan

AU - Graff-Radford, Neill R.

AU - Hof, Patrick R.

AU - Krolak-Salmon, Pierre

AU - Lehmann, Manja

AU - Magnin, Eloi

AU - Mendez, Mario F.

AU - Nestor, Peter J.

AU - Boeve, Bradley F

AU - Graff-Radford, Jonathan

AU - Pijnenburg, Yolande

AU - Primativo, Silvia

AU - Rossor, Martin N.

AU - Ryan, Natalie S.

AU - Scheltens, Philip

AU - Shakespeare, Timothy J.

AU - Suárez González, Aida

AU - Graff Radford, Neill R

AU - Yong, Keir X X

AU - Carrillo, Maria

AU - Fox, Nick C.

PY - 2017

Y1 - 2017

N2 - 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.

AB - 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.

KW - Alzheimer's disease

KW - Biomarker

KW - Clinico-radiological syndrome

KW - Pathophysiology

KW - Posterior cortical atrophy

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