@article{e13ed56408f941f4b37e0ccebed953bb,
title = "Comparison of sporadic and familial behavioral variant frontotemporal dementia (FTD) in a North American cohort",
abstract = "Introduction: Behavioral variant frontotemporal dementia (bvFTD) may present sporadically or due to an autosomal dominant mutation. Characterization of both forms will improve understanding of the generalizability of assessments and treatments. Methods: A total of 135 sporadic (s-bvFTD; mean age 63.3 years; 34% female) and 99 familial (f-bvFTD; mean age 59.9; 48% female) bvFTD participants were identified. f-bvFTD cases included 43 with known or presumed chromosome 9 open reading frame 72 (C9orf72) gene expansions, 28 with known or presumed microtubule-associated protein tau (MAPT) mutations, 14 with known progranulin (GRN) mutations, and 14 with a strong family history of FTD but no identified mutation. Results: Participants with f-bvFTD were younger and had earlier age at onset. s-bvFTD had higher total Neuropsychiatric Inventory Questionnaire (NPI-Q) scores due to more frequent endorsement of depression and irritability. Discussion: f-bvFTD and s-bvFTD cases are clinically similar, suggesting the generalizability of novel biomarkers, therapies, and clinical tools developed in either form to the other.",
keywords = "C9orf72, GRN, MAPT, bvFTD, clinical trials, frontotemporal dementia, genetics",
author = "{on behalf of the ARTFL and LEFFTDS consortia} and Heuer, {Hilary W.} and P. Wang and K. Rascovsky and A. Wolf and B. Appleby and J. Bove and Y. Bordelon and P. Brannelly and Brushaber, {D. E.} and C. Caso and G. Coppola and B. Dickerson and S. Dickinson and K. Domoto-Reilly and K. Faber and J. Ferrall and J. Fields and A. Fishman and J. Fong and T. Foroud and Forsberg, {L. K.} and D. Gearhart and B. Ghazanfari and N. Ghoshal and J. Goldman and J. Graff-Radford and N. Graff-Radford and I. Grant and M. Grossman and D. Haley and Hsiung, {G. Y.} and E. Huey and D. Irwin and D. Jones and K. Kantarci and A. Karydas and D. Kaufer and D. Kerwin and D. Knopman and J. Kornak and Kramer, {J. H.} and R. Kraft and Kremers, {W. K.} and W. Kukull and I. Litvan and P. Ljubenkov and Mackenzie, {I. R.} and M. Maldonado and M. Manoochehri and S. McGinnis",
note = "Funding Information: informationThis work is supported by the National Institutes of Health [grants U01 AG045390, U54 NS092089, U24 AG021886, and U01 AG016976].We extend our appreciation to Drs. John Hsiao and Dallas Anderson from the National Institute on Aging; Drs. Marg Sutherland, Codrin Lungu, and Roderick Corriveau from the National Institute of Neurological Disorders and Stroke; the clinicians and coordinators at all ARTFL/LEFFTDS centers, and particularly to our patients and their families for their participation in this research. Publisher Copyright: {\textcopyright} 2020 the Alzheimer's Association",
year = "2020",
month = jan,
day = "1",
doi = "10.1002/alz.12046",
language = "English",
volume = "16",
pages = "60--70",
journal = "Alzheimer's and Dementia",
issn = "1552-5260",
number = "1",
}