An Atypical Case Of Primary Progressive Aphasia: Implications For Differentiating The Semantic Variant

Abstract Objective Primary progressive aphasia is a neurodegenerative condition consisting of three primary variants. The semantic variant (svPPA) is typically associated with anomia and word-finding issues paired with fluent speech, although other symptoms can arise over time including non-verbal deficits and behavioral changes. A complex case of svPPA with evidence of robust impairment across multiple domains in the presence of notable psychosocial and medical factors is presented. Method Sixty-year-old woman with complaints of worsening memory and language changes in the context of marked behavioral disinhibition, deterioration of daily functioning, and indication of delusions was seen for outpatient neuropsychological evaluation. Medical history includes severe depression, anxiety, hypertension, and rheumatic heart disease. CT scan was significant for frontal and temporal atrophy and chronic right cerebellar infarct. Results Neuropsychological evaluation revealed uniformly extremely low scores. Presentation was notable for inappropriate behaviors, and expressive and receptive language difficulties. Significant confusion and functional impairment were evident. Her time awareness varied and she at some moments spoke about events from 20-30 years ago as if they were recent. Global language impairment was evident on formal testing and clearly moderated all other performances, including tasks of memory which were impaired. Notable weakness in visual-spatial processing and executive function were also present. Conclusions Complex medical and psychosocial history, and atypical decline patterns complicated the diagnosis of a patient with profound progressive expressive language impairment. SvPPA is offered as the diagnosis.