title: 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.
authors.affiliation: Memory and Aging Center, Department of Neurology, UCSF, 350 Parnassus Avenue, Suite 905, San Francisco, CA 94143-1207, USA. marilu@memory.ucsf.edu
authors.forename: M L
authors.initials: ML
authors.lastname: Gorno-Tempini
authors.forename: A E
authors.initials: AE
authors.lastname: Hillis
authors.forename: S
authors.initials: S
authors.lastname: Weintraub
authors.forename: A
authors.initials: A
authors.lastname: Kertesz
authors.forename: M
authors.initials: M
authors.lastname: Mendez
authors.forename: S F
authors.initials: SF
authors.lastname: Cappa
authors.forename: J M
authors.initials: JM
authors.lastname: Ogar
authors.forename: J D
authors.initials: JD
authors.lastname: Rohrer
authors.forename: S
authors.initials: S
authors.lastname: Black
authors.forename: B F
authors.initials: BF
authors.lastname: Boeve
authors.forename: F
authors.initials: F
authors.lastname: Manes
authors.forename: N F
authors.initials: NF
authors.lastname: Dronkers
authors.forename: R
authors.initials: R
authors.lastname: Vandenberghe
authors.forename: K
authors.initials: K
authors.lastname: Rascovsky
authors.forename: K
authors.initials: K
authors.lastname: Patterson
authors.forename: B L
authors.initials: BL
authors.lastname: Miller
authors.forename: D S
authors.initials: DS
authors.lastname: Knopman
authors.forename: J R
authors.initials: JR
authors.lastname: Hodges
authors.forename: M M
authors.initials: MM
authors.lastname: Mesulam
authors.forename: M
authors.initials: M
authors.lastname: Grossman
mesh_terms: D018888:Aphasia, Primary Progressive / Q000145:classification* / Q000473:pathology; D001284:Atrophy / Q000473:pathology; D001921:Brain / Q000473:pathology; D003704:Dementia / Q000473:pathology; D006801:Humans; D009483:Neuropsychological Tests