@article{2769628ebbd846328e8e42bbad4b0913,
title = "Quality of life and caregiver burden in familial frontotemporal lobar degeneration: Analyses of symptomatic and asymptomatic individuals within the LEFFTDS cohort",
abstract = "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{\textregistered}) 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{\textregistered} 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.",
keywords = "C9orf72, GRN, MAPT, TDP-43, frontotemporal dementia, quality of life, tau",
author = "{the LEFFTDS Consortium} and Gentry, {Melanie T.} and Lapid, {Maria I.} and Jeremy Syrjanen and Kendrick Calvert and Samantha Hughes and Danielle Brushaber and Walter Kremers and Jessica Bove and Patrick Brannelly and Giovanni Coppola and Christina Dheel and Bradley Dickerson and Susan Dickinson and Kelley Faber and Julie Fields and Jamie Fong and Tatiana Foroud and Leah Forsberg and Ralitza Gavrilova and Deb Gearhart and Nupur Ghoshal and Jill Goldman and Jonathan Graff-Radford and Neill Graff-Radford and Murray Grossman and Dana Haley and Hilary Heuer and Hsiung, {Ging Yuek} and Edward Huey and David Irwin and David Jones and Lynne Jones and Kejal Kantarci and Anna Karydas and David Knopman and John Kornak and Joel Kramer and Walter Kukull and Diane Lucente and Codrin Lungu and Ian Mackenzie and Masood Manoochehri and Scott McGinnis and Bruce Miller and Rodney Pearlman and Len Petrucelli and Madeline Potter and Rosa Rademakers and Zbigniew Wszolek and Boeve, {Bradley F.}",
note = "Funding Information: This work is supported by the National Institutes of Health (grants U01 AG045390, PI: Dr. Bradley F Boeve; U54 NS092089, PI: Dr. Adam L Boxer; U24 AG021886, PI: Dr. Tatiana M Foroud; and U01 AG016976, PI: Dr. Walter Anthony Kukull). Funding Information: informationThis work is supported by the National Institutes of Health (grants U01 AG045390, PI:?Dr.?Bradley F Boeve; U54 NS092089, PI: Dr. Adam L Boxer; U24 AG021886, PI: Dr. Tatiana M Foroud; and U01 AG016976, PI: Dr. Walter Anthony Kukull).We extend our appreciation to Drs. John Hsiao and Dallas Anderson from the National Institute on Aging, Drs. Marg Sutherland and Codrin Lungu from the National Institute of Neurological Disorders and Stroke, the staff of all centers, and particularly to our individuals and their families for their participation in this protocol. Publisher Copyright: {\textcopyright} 2020 the Alzheimer's Association",
year = "2020",
month = aug,
day = "1",
doi = "10.1002/alz.12095",
language = "English (US)",
volume = "16",
pages = "1115--1124",
journal = "Alzheimer's and Dementia",
issn = "1552-5260",
publisher = "Elsevier Inc.",
number = "8",
}