- Gentry, Melanie T;
- Lapid, Maria I;
- Syrjanen, Jeremy;
- Calvert, Kendrick;
- Hughes, Samantha;
- Brushaber, Danielle;
- Kremers, Walter;
- Bove, Jessica;
- Brannelly, Patrick;
- Coppola, Giovanni;
- Dheel, Christina;
- Dickerson, Bradley;
- Dickinson, Susan;
- Faber, Kelley;
- Fields, Julie;
- Fong, Jamie;
- Foroud, Tatiana;
- Forsberg, Leah;
- Gavrilova, Ralitza;
- Gearhart, Deb;
- Ghoshal, Nupur;
- Goldman, Jill;
- Graff‐Radford, Jonathan;
- Graff‐Radford, Neill;
- Grossman, Murray;
- Haley, Dana;
- Heuer, Hilary;
- Hsiung, Ging‐Yuek;
- Huey, Edward;
- Irwin, David;
- Jones, David;
- Jones, Lynne;
- Kantarci, Kejal;
- Karydas, Anna;
- Knopman, David;
- Kornak, John;
- Kramer, Joel;
- Kukull, Walter;
- Lucente, Diane;
- Lungu, Codrin;
- Mackenzie, Ian;
- Manoochehri, Masood;
- McGinnis, Scott;
- Miller, Bruce;
- Pearlman, Rodney;
- Petrucelli, Len;
- Potter, Madeline;
- Rademakers, Rosa;
- Ramos, Eliana Marisa;
- Rankin, Katherine;
- Rascovsky, Katya;
- Sengdy, Pheth;
- Shaw, Leslie;
- Tatton, Nadine;
- Taylor, Joanne;
- Toga, Arthur;
- Trojanowski, John;
- Weintraub, Sandra;
- Wong, Bonnie;
- Wszolek, Zbigniew;
- Boeve, Bradley F;
- Boxer, Adam;
- Rosen, Howard;
- Consortium, the LEFFTDS
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.