Linguistic Aspects of Primary Progressive Aphasia

Primary progressive aphasia (PPA) refers to a disorder of declining language associated with neurodegenerative diseases such as frontotemporal degeneration and Alzheimer disease. Variants of PPA are important to recognize from a medical perspective because these syndromes are clinical markers suggesting specific underlying pathology. In this review, I discuss linguistic aspects of PPA syndromes that may prove informative for parsing our language mechanism and identifying the neural representation of fundamental elements of language. I focus on the representation of word meaning in a discussion of semantic variant PPA, grammatical comprehension and expression in a discussion of nonfluent/agrammatic variant PPA, the supporting role of short-term memory in a discussion of logopenic variant PPA, and components of language associated with discourse in a discussion of behavioral variant frontotemporal dementia. PPA provides a novel perspective that uniquely addresses facets of language and its disorders while complementing traditional aphasia syndromes that follow stroke.