Primary progressive aphasia: a case report on diagnostic issues

Abstract

Primary progressive aphasia (PPA), a neurodegenerative condition of insidious onset which has language impairment as the most salient and significant initial feature may provide diagnostic challenges due to obstacles in confirming normal functioning in other cognitive domains. PPA is listed by the office of rare diseases (National Institute of Health) as a rare condition affecting less than 200,000 people in the entire United States population. The close similarities with the clinical signs of Alzheimer’s disease and the behavioural variant of frontotemporal dementia in the later years, may also allow for a missed or false diagnosis if not recognised early enough. This case report describes a 63-year-old Caucasian female diagnosed with PPA and elaborates on the clinical presentation, the role of neuropsychology in arriving at a potential diagnosis and highlights a management approach.