Behavior Symptoms in Primary Progressive Aphasia Variants

Abstract

Primary progressive aphasia (PPA) is a clinical syndrome characterized by an isolated and progressive deterioration of language abilities, in the context of otherwise preserved cognition and activities of daily living. Prominent behavior changes at symptom onset represent exclusionary criteria for PPA. However, in the last years, it has been acknowledged that early, mild behavior changes may accompany or precede language deficits in this condition. The frequency of these neuropsychiatric symptoms across the three variants of PPA is an area still being explored. Here we present a revision on this topic and additional research results conducted in our PPA clinical series. Overall, studies indicate a high frequency of behavior changes in PPA (above 80%), with depression, apathy, anxiety, agitation, irritability, as well as abnormal appetite, lack of insight, and disinhibition being frequently endorsed by these patients. The semantic variant is characterized by decreased empathy, disinhibition-like behaviors, changes in appetite/food preferences, and obsessive-compulsive behaviors. Negative behaviors, such as apathy, are more commonly associated with the nonfluent and logopenic variants, being significantly more frequent in the former. Delusions and hallucinations have also been reported in association with logopenic variant. These differences are likely to reflect distinct underlying neurodegenerative diseases, with implications for disease management. (PsycINFO Database Record (c) 2017 APA, all rights reserved)