Neuropsychological Assessment of Primary Progressive Aphasia (PPA)

: http://si p://pubs.as The goal of this article is to outline the utility of both language and non-language testing in making a diagnosis of logopenic, nonfluent/agrammatic, and semantic variant primary progressive aphasias PPA as well as delineate important behavioral and speech features that can be detected via clinical observation. We review speech/language presentations, non-language cognitive domains, and behavioral manifestations associated with each disorder. Patients with logopenic variant PPA evidence non-language cognitive impairments that include acalculia, phonological working memory deficits, and mild/variable difficulties with memory and visuospatial functions. In contrast, patients with nonfluent/agrammatic variant PPA display non-language impairments in executive functions, and show relative preservation of memory and visuospatial functions. Finally, semantic variant patients display behavioral changes in social comportment as well as non-language difficulties with category fluency and arithmetic facts; they display relative preservation, if not enhancement, of visuospatial functions. In summary, broad neural networks that support both language and non-language functions are affected in PPA syndromes, thus a comprehensive assessment of additional neuropsychological domains may aid in solidifying and subtyping PPA diagnoses. Primary progressive aphasias (PPA) are a group of neurodegenerative clinical syndromes, characterized by progressive, early deficits in language and/or speech functioning. Three syndromes are included in this umbrella category and include the logopenic, nonfluent/agrammatic, and semantic variants. Each syndrome is characterized by a prototypical phenotype that is driven by disruption in specific neural networks. As such, the presentation of each disorder includes an array of language, speech, cognitive, and behavioral disturbances that collectively contribute to and ultimately define the clinical picture seen in an evaluation. Whereas language deficits are the cardinal features of PPA syndromes, impairment in other cognitive and psychosocial domains is frequently evident, particularly with disease progression, rendering it important to conduct a full neuropsychological evaluation of patients whose symptoms suggest this diagnosis. The goal of this article is to provide an overarching view of PPA clinical syndromes using the lens of neuropsychological assessment. Our perspective is based on recent research in the field and also emphasizes the neuropsychological assessment practices employed by our institution at the University of California, San Francisco Memory and Aging Center (UCSF MAC). We hope to convey the importance of (a) conducting comprehensive cognitive evaluations that assess multiple domains, both in terms of language and non-language measures; (b) characterizing relative strengths and weaknesses, through examination of performance; and (c) appreciating the neuroanatomy that