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®) 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.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 1115-1124 |
| Number of pages | 10 |
| Journal | Alzheimer's and Dementia |
| Volume | 16 |
| Issue number | 8 |
| DOIs | |
| State | Published - Aug 1 2020 |
Keywords
- C9orf72
- GRN
- MAPT
- TDP-43
- frontotemporal dementia
- quality of life
- tau
ASJC Scopus subject areas
- Epidemiology
- Health Policy
- Developmental Neuroscience
- Clinical Neurology
- Geriatrics and Gerontology
- Psychiatry and Mental health
- Cellular and Molecular Neuroscience
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Quality of life and caregiver burden in familial frontotemporal lobar degeneration : Analyses of symptomatic and asymptomatic individuals within the LEFFTDS cohort. / the LEFFTDS Consortium.
In: Alzheimer's and Dementia, Vol. 16, No. 8, 01.08.2020, p. 1115-1124.Research output: Contribution to journal › Article › peer-review
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TY - JOUR
T1 - Quality of life and caregiver burden in familial frontotemporal lobar degeneration
T2 - Analyses of symptomatic and asymptomatic individuals within the LEFFTDS cohort
AU - the LEFFTDS Consortium
AU - Gentry, Melanie T.
AU - Lapid, Maria I.
AU - Syrjanen, Jeremy
AU - Calvert, Kendrick
AU - Hughes, Samantha
AU - Brushaber, Danielle
AU - Kremers, Walter
AU - Bove, Jessica
AU - Brannelly, Patrick
AU - Coppola, Giovanni
AU - Dheel, Christina
AU - Dickerson, Bradley
AU - Dickinson, Susan
AU - Faber, Kelley
AU - Fields, Julie
AU - Fong, Jamie
AU - Foroud, Tatiana
AU - Forsberg, Leah
AU - Gavrilova, Ralitza
AU - Gearhart, Deb
AU - Ghoshal, Nupur
AU - Goldman, Jill
AU - Graff-Radford, Jonathan
AU - Graff-Radford, Neill
AU - Grossman, Murray
AU - Haley, Dana
AU - Heuer, Hilary
AU - Hsiung, Ging Yuek
AU - Huey, Edward
AU - Irwin, David
AU - Jones, David
AU - Jones, Lynne
AU - Kantarci, Kejal
AU - Karydas, Anna
AU - Knopman, David
AU - Kornak, John
AU - Kramer, Joel
AU - Kukull, Walter
AU - Lucente, Diane
AU - Lungu, Codrin
AU - Mackenzie, Ian
AU - Manoochehri, Masood
AU - McGinnis, Scott
AU - Miller, Bruce
AU - Pearlman, Rodney
AU - Petrucelli, Len
AU - Potter, Madeline
AU - Rademakers, Rosa
AU - Wszolek, Zbigniew
AU - Boeve, Bradley F.
N1 - Funding Information: M. Gentry: nothing to disclose; M. Lapid: receives research support from NIH; J. Syrjanen: nothing to disclose; K. Calvert: nothing to disclose; S. Hughes: nothing to disclose; D. Brushaber: nothing to disclose; W. Kremers: receives research funding from AstraZeneca, Biogen, Roche, DOD, and NIH; J. Bove: nothing to disclose; P. Brannelly: employed by the Rainwater Charitable Foundation; G. Coppola: receives research support from NIH; C. Dheel: nothing to disclose; B. Dickerson: receives research support from NIH; S. Dickinson: on staff at the Association for Frontotemporal Degeneration and a member of the National Institute for Neurological Disorders and Stroke Advisory Council; K. Faber: receives research support from NIH; J. Fields: receives research support from NIH; J. Fong: nothing to disclose; T. Foroud: receives research support from NIH; L. Forsberg: receives research support from NIH; R. Gavrilova: receives research support from NIH; D. Gearhart: nothing to disclose; N. Ghoshal: has participated or is currently participating in clinical trials of anti‐dementia drugs sponsored by the following companies: Bristol Myers Squibb, Eli Lilly/Avid Radiopharmaceuticals, Janssen Immunotherapy, Novartis, Pfizer, Wyeth, SNIFF (The Study of Nasal Insulin to Fight Forgetfulness) study, and A4 (The Anti‐Amyloid Treatment in Asymptomatic Alzheimer's Disease) trial; she receives research support from Tau Consortium and Association for Frontotemporal Dementia and is funded by the NIH; J. Goldman: is serving as a consultant to the Novartis Alzheimer's Prevention Advisory Board; she receives research support from NIH, HDSA, New York State Department of Health (RFA # 1510130358); J. Graff‐Radford: receives research support from the NIH; J. Graff‐Radford: receives research support from the NIH; N. Graff‐Radford: receives royalties from UpToDate, has participated in multicenter therapy studies by sponsored by Biogen, TauRx, AbbVie, Novartis, and Lilly; he receives research support from NIH; M. Grossman: receives grant support from NIH, Avid, and Piramal; participates in clinical trials sponsored by Biogen, TauRx, and Alector; serves as a consultant to Bracco and UCB; and serves on the Editorial Board of Neurolog; D. Haley: nothing to disclose; H. Heuer: receives research support from NIH; G.‐Y. Hsiung: has served as an investigator for clinical trials sponsored by AstraZeneca, Eli Lilly, and Roche / Genentech; he receives research support from Canadian Institutes of Health Research and the Alzheimer Society of British Columbia; E. Huey: receives research support from NIH; D. Irwin: receives support from NIH, Brightfocus Foundation, and Penn Institute on Aging; D. Jones: receives research support from NIH and the Minnesota Partnership for Biotechnology and Medical Genomics; L. Jones: nothing to disclose; K. Kantarci: served on the Data Safety Monitoring Board for Takeda Global Research & Development Center, Inc.; data monitoring boards of Pfizer and Janssen Alzheimer Immunotherapy; research support from the Avid Radiopharmaceuticals, Eli Lilly, the Alzheimer's Drug Discovery Foundation, and NIH; A. Karydas: nothing to disclose; D. Knopman: serves on the DSMB of the DIAN‐TU study, is a site PI for clinical trials sponsored by Biogen, Lilly and the University of Southern California, and is funded by NIH; J. Kornak: has provided expert witness testimony for Teva Pharmaceuticals in Forest Laboratories Inc. et al. v. Teva Pharmaceuticals USA, Inc., Case Nos. 1:14‐cv‐00121 and 1:14‐cv‐00686 (D. Del. filed Jan. 31, 2014 and May 30, 2014) regarding the drug Memantine; for Apotex/HEC/Ezra in Novartis AG et al. v. Apotex Inc., No. 1:15‐cv‐975 (D. Del. filed Oct. 26, 2015, regarding the drug Fingolimod; he has also given testimony on behalf of Puma Biotechnology in Hsingching Hsu et al, versus Puma Biotechnology, INC., et al. 2018 regarding the drug Neratinib and he receives research support from the NIH; J. Kramer: receives research support from NIH; W. Kukull: receives research support from NIH; D. Lucente: receives research support from NIH; C. Lungu: honoraria for editorial work from Elsevier, Inc.; I. Mackenzie: receives research funding from Canadian Institutes of Health Research; M. Manoochehri: nothing to disclose; S. McGinnis: has served as an investigator for clinical trials sponsored by AbbVie, Allon Therapeutics, Biogen, Bristol‐Myers Squibb, C2N Diagnostics, Eisai Inc., Eli Lilly and Co., Genentech, Janssen Pharmaceuticals, Medivation, Merck, Navidea Biopharmaceuticals, Novartis, Pfizer, and TauRx Therapeutics and receives research support from NIH; B. Miller: receives research support from NIH; R. Pearlman: employed by The Bluefield Project; L. Petrucelli: receives research support from NIH; M. Potter: receives research support from NIH; R. Rademakers: receives research funding from NIH and the Bluefield Project to Cure Frontotemporal Dementia; E. Ramos: nothing to disclose; K. Rankin: receives research support from NIH; K. Rascovsky: receives research support from NIH; P. Sengdy: nothing to disclose; L. Shaw: receives research support from NIH; N. Tatton: employed by the Association for Frontotemporal Degeneration; J. Taylor: nothing to disclose; A. Toga: receives research support from NIH and the Alzheimer's Association; J. Trojanowski: may accrue revenue in the future on patents submitted by the University of Pennsylvania wherein he is coinventor and he received revenue from the sale of Avid to Eli Lily as coinventor on Aβ amyloid imaging–related patents submitted by the University of Pennsylvania; he receives research support from the NIH and several nonprofits; S. Weintraub: receives research support from NIH; B. Wong: receives research support from NIH; Z. Wszolek: supported by the NIH, Mayo Clinic Center for Regenerative Medicine, the gift from Carl Edward Bolch, Jr., and Susan Bass Bolch, The Sol Goldman Charitable Trust, and Donald G. and Jodi P. Heeringa; he has also received grant funding support from Allergan, Inc. (educational grant), and Abbvie (medication trials); B. Boeve: has served as an investigator for clinical trials sponsored by GE Healthcare and Axovant,e receives royalties from the publication of a book entitled (Cambridge Medicine, 2009, 2017), serves on the Scientific Advisory Board of the Tau Consortium, receives research support from NIH, the Mayo Clinic Dorothy and Harry T. Mangurian Jr. Lewy Body Dementia Program and the Little Family Foundation; A. Boxer: receives research support from NIH, the Tau Research Consortium, the Association for Frontotemporal Degeneration, Bluefield Project to Cure Frontotemporal Dementia, Corticobasal Degeneration Solutions, the Alzheimer's Drug Discovery Foundation, and the Alzheimer's Association; he has served as a consultant for Aeton, Abbvie, Alector, Amgen, Arkuda, Ionis, Iperian, Janssen, Merck, Novartis, Samumed, Toyama, and UCB, and received research support from Avid, Biogen, BMS, C2N, Cortice, Eli Lilly, Forum, Genentech, Janssen, Novartis, Pfizer, Roche, and TauRx; H. Rosen: has received research support from Biogen Pharmaceuticals, has consulting agreements with Wave Neuroscience and Ionis Pharmaceuticals, and receives research support from NIH. Behavioral Neurology of Dementia 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: © 2020 the Alzheimer's Association Copyright: Copyright 2020 Elsevier B.V., All rights reserved.
PY - 2020/8/1
Y1 - 2020/8/1
N2 - 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.
AB - 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.
KW - C9orf72
KW - GRN
KW - MAPT
KW - TDP-43
KW - frontotemporal dementia
KW - quality of life
KW - tau
UR - http://www.scopus.com/inward/record.url?scp=85087837259&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85087837259&partnerID=8YFLogxK
U2 - 10.1002/alz.12095
DO - 10.1002/alz.12095
M3 - Article
AN - SCOPUS:85087837259
VL - 16
SP - 1115
EP - 1124
JO - Alzheimer's and Dementia
JF - Alzheimer's and Dementia
SN - 1552-5260
IS - 8
ER -