Quality of life and caregiver burden in familial frontotemporal lobar degeneration: Analyses of symptomatic and asymptomatic individuals within the LEFFTDS cohort

Objective: The Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects evaluates familial frontotemporal lobar degeneration (FTLD) kindreds with MAPT, GRN, or C9orf72 mutations. Objectives were to examine whether health-related quality of life (HRQoL) correlates with clinical symptoms and caregiver burden, and whether self-rated and informant-rated HRQoL would correlate with each other. Methods: Individuals were classified using the Clinical Dementia Rating (CDR^®) Scale plus National Alzheimer's Coordinating Center (NACC) FTLD. HRQoL was measured with DEMQOL and DEMQOL-proxy; caregiver burden with the Zarit Burden Interview (ZBI). For analysis, Pearson correlations and weighted kappa statistics were calculated. Results: The cohort of 312 individuals included symptomatic and asymptomatic individuals. CDR^® plus NACC FTLD was negatively correlated with DEMQOL (r = −0.20, P =.001), as were ZBI and DEMQOL (r = −0.22, P =.0009). There was fair agreement between subject and informant DEMQOL (κ = 0.36, P <.0001). Conclusion: Lower HRQoL was associated with higher cognitive/behavior impairment and higher caregiver burden. These findings demonstrate the negative impact of FTLD on individuals and caregivers.