Classification of primary progressive aphasia and its variants

• Published in: Neurology Vol. 76; no. 11; pp. 1006 - 1014
• Main Authors: GORNO-TEMPINI, M. L, HILLIS, A. E, 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, WEINTRAUB, S, KERTESZ, A, MENDEZ, M, CAPPA, S. F, OGAR, J. M, ROHRER, J. D, BLACK, S, BOEVE, B. F
• Format: Journal Article Conference Proceeding
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins 15-03-2011
• Subjects: Aphasia, Primary Progressive - classification; Aphasia, Primary Progressive - pathology; Atrophy - pathology; Biological and medical sciences; Brain - pathology; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Dementia - pathology; Humans; Medical sciences; Neurology; Neuropsychological Tests; Views and Reviews; Communication disorder; Nervous system diseases; Central nervous system disease; Classification; Aphasia; Neurological disorder; Language disorder; Cerebral disorder
• ISSN: 0028-3878; 1526-632X
• DOI: 10.1212/wnl.0b013e31821103e6

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.