Few studies have reported neuropsychiatric symptoms (NPS) in primary progressive aphasia (PPA), a neurodegenerative disorder that primarily affects the left hemisphere. Depression is associated with left-sided stroke, but it remains unclear whether depression and other NPS are also associated with PPA. The authors compared the frequency of neuropsychiatric symptoms in 55 cases of PPA with 110 cognitively normal persons matched for age, sex, and education. Depression, apathy, agitation, anxiety, appetite change, and irritability are associated with PPA. Hallucinations, delusions, and night-time behavior were not associated with PPA.