Classification of primary progressive aphasia and its variants
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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Copyright © 2011 by AAN Enterprises, Inc.
Publication History
Received: December 10, 2009
Accepted: November 9, 2010
Published online: February 16, 2011
Published in print: March 15, 2011
Disclosure
Dr. Gorno-Tempini receives research support from the NIH (NINDS, NIA), the John Douglas French Alzheimer's Foundation, the Alzheimer's Association, the Larry L. Hillblom Foundation, the Koret Family Foundation, and the McBean Family Foundation. Dr. Hillis serves as an Associate Editor for Brain and Aphasiology, Co-Editor-in-Chief for Behavioural Neurology, a Section Editor for Nature Reviews Neurology, and on the editorial boards of Brain and Language and Cognitive Neuropsychology; serves as a consultant for the Charles Dana Foundation; and receives research support from the NIH (NINDS, NIDCD). Dr. Weintraub serves on the editorial boards of Alzheimer's and Dementia, the Turkish Journal of Neurology, Dementia & Neuropsychologia, and the Journal of the Brazilian Academy of Neurology; and receives research support from the NIH (NIA, NIDCD). Dr. Kertesz serves on a scientific advisory board for Pfizer Inc.; serves on the editorial boards of Cognitive and Behavioral Neurology and Aphasiology; receives royalties from the publication of The Western Aphasia Battery (Grune and Stratton, 1982); and has received research support from Elan Corporation, Pfizer Inc., Lundbeck Inc., the Lawson Research Institute, the American Neurological Society, and the Whitaker professorship. Dr. Mendez serves on the editorial boards of Brain, Annals of Neurology, and Human Brain Mapping and on the Advisory Boards of the Cure Alzheimer Fund and the Association for Frontotemporal Dementia. Dr. Cappa has received speaker honoraria from Novartis; serves as Co-Editor-in-Chief for Behavioural Neurology, an Associate Editor for Neurological Sciences, Action Editor for Cortex, and on the editorial boards of Aphasiology, Neuropsychological Rehabilitation, and Future Neurology; receives publishing royalties for Cognitive Neurology: A Textbook (Oxford University Press, 2009) and Cognitive Neurology (Imperial College Press, 2009); and has received speaker honoraria from Janssen and Novartis. J.M. Ogar receives research support from the NIH. Dr. Rohrer has received research support from the Wellcome Trust and from a Brain Exit Scholarship. Dr. Black serves on a scientific advisory board for Pfizer Inc.; has received funding for travel or speaker honoraria from Pfizer Inc., Eisai Inc., and Myriad Genetics, Inc.; serves on the editorial board of Alzheimer's Research & Therapy; is an inventor on a patent re: INCAS (Integrated NeuroCognitive Assessment System): Cognitive Assessment Tool and Method; has served as a consultant for Pfizer Inc., Janssen, Novartis, Lundbeck Inc., Myriad Genetics, Inc., GlaxoSmithKline, Schering-Plough Corp., Elan Corporation, Wyeth, and Bristol-Myers Squibb; serves on speakers' bureaus for Janssen, Novartis, Lundbeck Inc., Pfizer Inc., Eisai Inc., and Myriad Genetics, Inc.; and receives research support from Roche, GlaxoSmithKline, Pfizer Inc., Novartis, Lundbeck Inc., Myriad Genetics, Inc., Boehringer Ingelheim, Sanofi-Aventis, Eisai Inc., the CIHR, the NIH, the Canada Foundation for Innovation, the Heart and Stroke Foundation of Ontario, Canadian Stroke Network, Sunnybrook Health Sciences Centre, NSERC, the Alzheimer Society of Canada, and the Alzheimer's Association US. Dr. Boeve receives royalties from the publication of Behavioral Neurology of Dementia (Cambridge University Press, 2009); has served as a consultant for GE Healthcare; and receives research support from Myriad Genetics, Inc., Cephalon, Inc., the NIH, and the Alzheimer's Association. Dr. Manes reports no disclosures. Dr. Dronkers receives research support from the NIH and the US Department of Veterans Affairs. Dr. Vandenberghe serves on a scientific advisory board for AC Immune SA; serves on the editorial board of Frontiers in Neuroscience and Translational Neuroscience; receives research support from GE Healthcare, Pfizer Inc., Eli Lilly and Company, Medivation, Inc., Novartis, Research Foundation Flanders, Interuniversity Attraction Pole P6/29, KU Leuven, and Stichting Alzheimer Onderzoek. Dr. Rascovsky reports no disclosures. Dr. Patterson serves as Consulting Editor for Cortex and receives publishing royalties for Pyramids and Palmtrees Test (Thames Valley Publishing Company, 1992–present). Dr. Miller serves on a scientific advisory board for the Alzheimer's Disease Clinical Study, serves as an Editor for Neurocase, and as an Associate Editor of ADAD; receives royalties from the publication of Behavioral Neurology of Dementia (Cambridge, 2009), Handbook of Neurology (Elsevier, 2009), and The Human Frontal Lobes (Guilford, 2008); serves as a consultant for Lundbeck Inc., Allon Therapeutics, Inc., and Novartis; has served on speakers' bureaus for Novartis and Pfizer Inc.; and receives research support from Novartis, the NIH, and the State of California Alzheimer's Center. Dr. Knopman serves as Deputy Editor of Neurology®; has served on data safety monitoring boards for Sanofi-Aventis, GlaxoSmithKline, and Eli Lilly and Company; is an investigator in clinical trials sponsored by Elan Corporation, Baxter International Inc., and Forest Laboratories, Inc.; and receives research support from the NIH. Dr. Hodges serves on editorial boards of Aphasiology, Cognitive Neuropsychiatry, and Cognitive Neuropsychology; receives royalties from publication of Cognitive Assessment for Clinicians (Oxford University Press, 2007) and Frontotemporal Dementia Syndromes (Cambridge University Press, 2007); and receives fellowship support from the Australian Research Council Federation. Dr. Mesulam serves on the scientific advisory boards for the Cure Alzheimer Fund and the Association on Frontotemporal Dementia; serves on the editorial boards of Brain, Annals of Neurology, Human Brain Mapping, and Journal of Cognitive Neuroscience; receives royalties from the publication of Principles of Behavioral and Cognitive Neurology (Oxford University Press, 2000); and receives research support from the NIH (NIDCD, NIA). Dr. Grossman serves on a scientific advisory board for Allon Therapeutics, Inc.; serves as Editor for Cognitive and Behavioral Neurology; has served as a consultant for Pfizer Inc. and Forest Laboratories, Inc.; and receives research support from the NIH.
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