Cited by 5,141Dementia and Cognitive Impairment ResearchNeurobiology of Language and BilingualismAmyotrophic Lateral Sclerosis Research

The DOI matches its Crossref record; 51 of the 51 references it cites were found in a registry or an index.

Classification of primary progressive aphasia and its variants

Open access

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.

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Crossref · 10.1212/wnl.0b013e31821103e6

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  1. Serieux P. Sur un cas de surdite verbale pure. Rev Med 1893;13:733–750.

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Metadata

Authors
Gorno-Tempini, M. L., Hillis, A. E., Weintraub, S., Kertesz, A., Mendez, M., Cappa, S. F., Ogar, J. M., Rohrer, J. D., Black, S., Boeve, B. F., Manes, F., Dronkers, N. F., Vandenberghe, R., Rascovsky, K., Patterson, K., Miller, B. L., Knopman, D. S., Hodges, J. R., Mesulam, M. M., & Grossman, M.
Affiliations
  • From the Memory and Aging Centre (M.L.G.-T., J.M.O., K.R., B.L.M.), Department of Neurology, University of California at San Francisco, San Francisco; Center for Mind/Brain Sciences (M.L.G.-T.), University of Trento, Trento, Italy; Department of Neurology (A.E.H.), Johns Hopkins University, Baltimore, MD; Department of Neurology (S.W., M.M.M.), Northwestern University, Chicago, IL; Cognitive Neurology (A.K.), University of Western Ontario, London, Canada; Department of Neurology (M.M.), University...
  • Northwestern University (US)
Venue
Neurology
Published
2011-03-15
Volume
76
Issue
11
Pages
1006-1014
ISSN
  • 0028-3878 (ISSN-L)
  • 1526-632X
OpenAlex
W2138190873
Cited by
5,141
References
51
Citations by year
2026: 340 · 2025: 424 · 2024: 439 · 2023: 395 · 2022: 449 · 2021: 455 · 2020: 431 · 2019: 391 · 2018: 331 · 2017: 293 · 2016: 263 · 2015: 249 · 2014: 212 · 2013: 222 · 2012: 160
FWCI
143.53
Type
journal article
Language
en
Open access
Locations
  • Neurology (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
  • Institutional Research Information System (Università degli Studi di Trento) (submittedVersion, other-oa)
  • UCL Discovery (University College London) (submittedVersion)
Funders
  • U.S. Department of Veterans Affairs
  • Alzheimer's Association
  • Bristol-Myers Squibb (AG17586)
  • Eli Lilly and Company (AG17586)
  • Pfizer (AG17586)
  • GlaxoSmithKline
  • Sanofi
  • Heart and Stroke Foundation of Canada
  • Baxter International
  • Wellcome Trust
  • Canadian Stroke Network
  • Myriad Genetics
  • Alzheimer Society
  • University of Cambridge
  • University of Oxford
  • Elan
  • Fonds Wetenschappelijk Onderzoek (P6/29)
  • Eisai
  • KU Leuven
  • Vlaamse regering
  • H. Lundbeck A/S
  • National Institutes of Health (AG019724, NS53488, AG 17586, AG13854, AG16574, AG17586, AG15116, NS44266, NS53488)
  • Forest Laboratories
  • Canadian Institutes of Health Research
  • Natural Sciences and Engineering Research Council of Canada
  • Australian Research Council
  • National Institute on Aging (AG15116, R01AG015866, AG13854, RO1 AG15866, AG 16574, U01AG006786, R01AG015116, P01AG017586, AG17586, R01AG034499, AG019724, U24AG026395, P30AG013854, U19AG010483, P01AG019724, P50AG023501, P50AG016574)
  • National Institute on Deafness and Other Communication Disorders (AG13854, R01DC008552, DC008552, R01DC005375)
  • National Institute of Neurological Disorders and Stroke (NS53488, NS-44266, P50NS053488, R0I NS047691, R01NS047691, R01NS044266, R01NS050915)
  • National Center for Research Resources (UL1RR025741)
Topics
  • Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
  • Life Sciences › Neuroscience › Cognitive Neuroscience › Neurobiology of Language and Bilingualism
  • Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
Keywords
primary progressive aphasia, nonfluent/agrammatic variant, semantic variant, logopenic variant, clinical criteria, brain atrophy patterns, clinicopathological correlation, biomarkers
MeSH terms
Atrophy, Brain, Dementia, Humans, Neuropsychological Tests, Aphasia, Primary Progressive

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