Journal
NEUROLOGY
Volume 76, Issue 11, Pages 1006-1014Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1212/WNL.0b013e31821103e6
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Funding
- National Institute of Neurological Disorders and Stroke [R01NS050915]
- National Institute on Aging [P50AG023501, P01 AG019724, RO1 AG15866, U24 AG26395]
- National Institute on Deafness and Communication Disorders [DC008552]
- National Institute on Aging (Alzheimer Disease Center) [AG13854]
- NIDCD [DC008552, AG17586, AG15116, NS44266, NS53488]
- Wellcome Trust
- Brain Exit Scholarship [R01AG034499-02]
- NIH [ROI NS047691, RO1DC05375]
- Alzheimer's Association [IIRG-05-14560]
- Australian Research Council Federation
- NIH (NINDS, NIA)
- John Douglas French Alzheimer's Foundation
- Alzheimer's Association
- Larry L. Hillblom Foundation
- Koret Family Foundation
- McBean Family Foundation
- NIH (NINDS, NIDCD)
- NIH (NIA, NIDCD)
- Elan Corporation, Pfizer Inc.
- Lundbeck Inc.
- Lawson Research Institute
- American Neurological Society
- Whitaker professorship
- Human Brain Mapping and on the Advisory Boards of the Cure Alzheimer Fund
- Association for Frontotemporal Dementia
- NIH
- Brain Exit Scholarship
- Pfizer Inc.
- Eisai Inc.
- Myriad Genetics, Inc.
- Cephalon, Inc.
- US Department of Veterans Affairs
- GE Healthcare
- Eli Lilly and Company
- Medivation, Inc.
- Novartis
- Research Foundation Flanders
- Interuniversity Attraction Pole, KU Leuven [P6/29]
- Stichting Alzheimer Onderzoek
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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. Neurology (R) 2011;76:1006-1014
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