Language impairment in Primary Progressive Aphasia

Primary progressive aphasia (PPA) is a neurodegenerative syndrome associated with atrophy of the frontal and temporal regions of the left hemisphere, typically leading to language impairment. PPA is a heterogeneous condition, in which the most prominent clinical feature is a difficulty with language (deficit of language production, object naming, syntax, or word comprehension), while other cognitive domains are not affected at the onset and in the early stages of the disease. The recommendations for the diagnosis of PPA proposed by an international group of experts include a classification of PPA and its three main variants, namely, the non-fluent/agrammatic variant, the semantic variant, and the logopenic variant. This chapter describes the clinical features, neuroanatomical correlates, demographic features, and course of each of these variants, followed by a description of key issues related to the assessment and behavioral treatment of PPA.