Dysexecutive Symptoms in Primary Progressive Aphasia: Beyond Diagnostic Criteria

Abstract

Primary progressive aphasia (PPA) is a heterogeneous neurodegenerative condition in which the most prominent clinical feature is language difficulties. Other cognitive domains have been described to remain unaffected at the early stages of the disease and, therefore, excluded from diagnostic criteria. However, we show in this article that executive function (EF) disorders may be present in the 3 variants (nonfluent/agrammatic, logopenic, and semantic) of PPA. We also illustrate changes in language and EF by means of a 3-year behavioral and neuroimaging longitudinal study of a patient suffering from the semantic variant of PPA. This review provides an update on current knowledge of PPA, suggesting that dysexecutive symptoms may be encountered in the 3 PPA variants, in their early phases and/or in more advanced stages, when atrophy extends to adjacent brain areas.