QUALITY OF LIFE AMONG SUBJECTS AND INFORMANTS IN FAMILIAL FRONTOTEMPORAL DEMENTIA: PRELIMINARY DATA IN THE LEFFTDS COHORT

The Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects (LEFFTDS) Consortium involves 8 North American centers with the purpose of evaluating subjects with the 3 most common genetic mutations in familial FTD. We hypothesized that 1) quality of life (QOL) measures for subjects and informants would correlate with clinical status, and 2) QOL ratings for subjects and their informants would be correlated with each other. QOL was assessed using the DEMQOL (self-report) and DEMQOL-proxy (informant reporting on the subject) measures, in which the overall QOL ratings were very good, good, fair and poor. Subjects were assessed by a clinician using a modified version of the Clinical Dementia Rating (CDR), with a score of 0 reflecting normal neurologic functioning, 0.5 reflecting minimally symptomatic FTD, and ≥1 reflecting overtly symptomatic FTD. The frequencies of the combined very good/good ratings from the subjects were: CDR=0, 94.8%; 0.5, 75%; and ≥1, 77.1%, whereas the combined very good/good ratings from the informants were: CDR=0, 98.3%; 0.5, 75%; and ≥1, 61.1%. Utilizing a Spearman correlation for the very good, good, and fair/poor (poor was combined with fair due to only 7 ratings being viewed as poor) groups, the subject (n=179) and informant (n=191) QOL scores were found to have a statistically significant, moderate negative correlation to CDR scores (r=-0.4139 and r=-0.4011, respectively). A weighted Kappa analysis comparing the subject and informant QOL reports showed a fair agreement (κ=0.3570). These findings indicate that poorer QOL is associated with poorer clinical status, suggesting that even mild FTD features impact QOL. The fair agreement between subjects and informants on these QOL measures could reflect different degrees of insight on FTD features and the resulting impact on QOL. Additional work on QOL in this cohort will provide more insights on how FTD impacts QOL in subjects and informants, particularly in the phenoconversion from asymptomatic to overtly symptomatic FTD.