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March 15, 2011; 76 (11) Views and Reviews

Classification of primary progressive aphasia and its variants

M.L. Gorno-Tempini, A.E. Hillis, S. Weintraub, A. Kertesz, M. Mendez, S.F. Cappa, J.M. Ogar, J.D. Rohrer, S. Black, B.F. Boeve, F. Manes, N.F. Dronkers, R. Vandenberghe, K. Rascovsky, K. Patterson, B.L. Miller, D.S. Knopman, J.R. Hodges, M.M. Mesulam, M. Grossman
First published February 16, 2011, DOI: https://doi.org/10.1212/WNL.0b013e31821103e6
M.L. Gorno-Tempini
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Classification of primary progressive aphasia and its variants
M.L. Gorno-Tempini, A.E. Hillis, S. Weintraub, A. Kertesz, M. Mendez, S.F. Cappa, J.M. Ogar, J.D. Rohrer, S. Black, B.F. Boeve, F. Manes, N.F. Dronkers, R. Vandenberghe, K. Rascovsky, K. Patterson, B.L. Miller, D.S. Knopman, J.R. Hodges, M.M. Mesulam, M. Grossman
Neurology Mar 2011, 76 (11) 1006-1014; DOI: 10.1212/WNL.0b013e31821103e6

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Abstract

This article provides a classification of primary progressive aphasia (PPA) and its 3 main variants to improve the uniformity of case reporting and the reliability of research results. Criteria for the 3 variants of PPA—nonfluent/agrammatic, semantic, and logopenic—were developed by an international group of PPA investigators who convened on 3 occasions to operationalize earlier published clinical descriptions for PPA subtypes. Patients are first diagnosed with PPA and are then divided into clinical variants based on specific speech and language features characteristic of each subtype. Classification can then be further specified as “imaging-supported” if the expected pattern of atrophy is found and “with definite pathology” if pathologic or genetic data are available. The working recommendations are presented in lists of features, and suggested assessment tasks are also provided. These recommendations have been widely agreed upon by a large group of experts and should be used to ensure consistency of PPA classification in future studies. Future collaborations will collect prospective data to identify relationships between each of these syndromes and specific biomarkers for a more detailed understanding of clinicopathologic correlations.

Footnotes

  • Study funding: National Institute of Neurological Disorders and Stroke R01NS050915, National Institute on Aging P50AG023501 and P01 AG019724 (M.G.-T.); National Institute on Deafness and Communication Disorders DC008552, National Institute on Aging (Alzheimer Disease Center) AG13854, NIDCD DC008552 (M.M.M., S.W.); AG17586, AG15116, NS44266, NS53488 (M.G.); The Wellcome Trust and a Brain Exit Scholarship (J.R.); R01AG034499-02 (M.M.); NIH ROI NS047691 and RO1DC05375 (A.H.); National Institute on Aging, P50 AG16574, U01 AG06786, RO1 AG15866, and U24 AG26395, and the Alzheimer's Association (IIRG-05-14560) (B.B.); and research from the Australian Research Council Federation (J.H.).

  • AD
    Alzheimer disease
    FTLD
    frontotemporal lobar degeneration
    PPA
    primary progressive aphasia

  • Editorial, page 942

  • Received December 10, 2009.
  • Accepted November 9, 2010.
  • Copyright © 2011 by AAN Enterprises, Inc.
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  • Article
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    • DEVELOPMENT OF THE PROPOSED CLASSIFICATION OF PPA AND ITS VARIANTS
    • WORKING RESEARCH CRITERIA
    • DISCUSSION
    • DISCLOSURE
    • Footnotes
    • REFERENCES
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