Abstract
Primary progressive aphasia (PPA) is classified into three variants, logopenic variant PPA (lvPPA), nonfluent agrammatic PPA (nfaPPA), and semantic variant PPA (svPPA), based on clinical (syndromic) characteristics with support from neuroimaging and/or underlying neuropathology. Classification of PPA variants provides information valuable to disease management. International consensus criteria are widely employed to identify PPA subtypes; however, classification is complex, and some individuals do not fit neatly into the subtyping scheme. In this review, diagnostic challenges and their implications are discussed, possible explanations for these challenges are explored, and approaches to address PPA classification are considered.
Highlights
Primary progressive aphasia (PPA) is a neurodegenerative clinical syndrome characterized by the predominance and insidious onset of language impairments, and gradual deterioration of these abilities over time, associated with atrophy of the language network of the brain, including frontal, temporal, and parietal regions of the left hemisphere[1,2,3]
PPA is classified as a rare disease by the Genetic and Rare Diseases Information Center of the National Center for Advancing Translational Sciences (NCATS)[4]
Three different PPA variants are specified by international consensus criteria based on clinical presentation with support from patterns of atrophy and/or underlying neuropathology: logopenic variant PPA, nonfluent agrammatic PPA, and semantic variant PPA3
Summary
F1000 Faculty Reviews are written by members of the prestigious F1000 Faculty. They are commissioned and are peer reviewed before publication to ensure that the final, published version is comprehensive and accessible. The reviewers who approved the final version are listed with their names and affiliations. Any comments on the article can be found at the end of the article
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