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Classification of primary progressive aphasia and its variants

2011· article· en· 5,112 citations· W2138190873 on OpenAlex· 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.

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The record

Venue
Neurology
Topic
Genomics and Rare Diseases
Field
Biochemistry, Genetics and Molecular Biology
Canadian institutions
Funders
National Center for Research ResourcesNational Institute on Deafness and Other Communication DisordersNational Institute on AgingAustralian Research CouncilNational Institutes of HealthUniversity of OxfordVlaamse regeringKU LeuvenBristol-Myers SquibbForest LaboratoriesCanadian Institutes of Health ResearchFonds Wetenschappelijk OnderzoekElanEisaiNational Institute of Neurological Disorders and StrokeUniversity of CambridgeNatural Sciences and Engineering Research Council of CanadaBaxter InternationalPfizerHeart and Stroke Foundation of CanadaCanadian Stroke NetworkWellcome TrustGlaxoSmithKlineAlzheimer SocietyH. Lundbeck A/SMyriad GeneticsEli Lilly and CompanySanofiAlzheimer's AssociationU.S. Department of Veterans Affairs
Keywords
Primary progressive aphasiaConsistency (knowledge bases)OperationalizationNatural language processingComputer scienceReliability (semiconductor)AphasiaMedicinePsychologyArtificial intelligencePathologyFrontotemporal dementiaCognitive psychologyDiseaseDementia
Has abstract in OpenAlex
yes