期刊
ALZHEIMERS & DEMENTIA
卷 13, 期 8, 页码 870-884出版社
ELSEVIER SCIENCE INC
DOI: 10.1016/j.jalz.2017.01.014
关键词
Posterior cortical atrophy; Alzheimer's disease; Clinico-radiological syndrome; Pathophysiology; Biomarker
资金
- Alzheimer's Association
- International Society
- ESRC/NIHR [ES/L001810/1]
- EPSRC [EP/M006093/1, EP/J020990/1]
- Alzheimer's Research UK Senior Research Fellowship
- MRC [MR/K010395/1, CSUB19166]
- NIHR Oxford Biomedical Research Centre
- NIH [AG005131, P50AG05146, P50 AG005138, R01-AG045611, P50-AG023501, U54-NS092089, R01-AG038791, R01-AG048234]
- Jane Tanger Black Scholarship for Young-Onset Dementias
- Nancy H. Hall Memorial Fund for Geriatric Psychiatry
- NIHR Queen Square Dementia BRU
- NIHR UCL/H Biomedical Research Centre
- Wolfson Foundation
- ARUK [ARUK-Network 2012-6-ICE, ARUK-PG2014-1946]
- Brain Research Trust [UCC14191]
- European Union's Horizon 2020 research and innovation program [666992]
- Brain Exit Fellowship
- American College of Radiology
- Tau Consortium
- Association for Frontotemporal Degeneration
- Michael J. Fox Foundation
- Alzheimer's Research UK Fellowship [ARUK-RF2013-5]
- Stichting Alzheimer Nederland
- Stichting VUmc fonds
- Alzheimers Research UK [ARUK-NSG2017-4, ARUK-RF2013-5, ARUK-SRF2013-8] Funding Source: researchfish
- Economic and Social Research Council [ES/L001810/1] Funding Source: researchfish
- Engineering and Physical Sciences Research Council [EP/J020990/1, EP/M006093/1] Funding Source: researchfish
- Medical Research Council [MR/K010395/1] Funding Source: researchfish
- National Institute for Health Research [CL-2009-13-004, NF-SI-0513-10134, NF-SI-0512-10033] Funding Source: researchfish
- EPSRC [EP/J020990/1, EP/M006093/1] Funding Source: UKRI
- ESRC [ES/L001810/1] Funding Source: UKRI
- MRC [MR/K010395/1] Funding Source: UKRI
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 PCAwere 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 syndromeand 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. (C) 2017 The Authors. Published by Elsevier Inc. on behalf of the Alzheimer's Association.
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