Assessment of disease impact through health-related quality of life measurement in primary progressive aphasia

INTRODUCTION: Measurements of health-related quality of life (HRQoL) are important for capturing disease impact beyond physical health and relative to other diseases but have rarely been assessed in primary progressive aphasia (PPA). METHODS: HRQoL was characterized overall, by sex and subtype in PPA (n = 118) using the Health Utilities Index-2/3 (HUI2/3). Multiple linear regression assessed associations between HRQoL and language severity. RESULTS: Multi-attribute HUI2/3 summary scores indicated moderate to severe impairment. Scores did not differ by sex and were more severe for semantic than non-semantic PPA. Language severity scores showed significant associations with HUI multi-attribute scores and select single-attribute measures (hearing, sensation, cognition, and speech) with less language impairment associated with better functional capacity related to HRQoL. DISCUSSION: This study identified poor HRQoL in a relatively large PPA cohort. HRQoL measures aid in determining patient perspective, policy decision making, and resource allocation. Results may be used to advocate for PPA support. Highlights: Primary progressive aphasia (PPA) negatively impacts health-related quality of life. Health utilities index scores are associated with Western Aphasia Battery performance in PPA. Severity of language impairment in PPA is associated with poorer quality of life.