Comparison of sporadic and familial behavioral variant frontotemporal dementia (FTD) in a North American cohort. Issue 1 (8th January 2020)

• Record Type: Journal Article
• Authors: Heuer, Hilary W.; Wang, P.; Rascovsky, K.; Wolf, A.; Appleby, B.; Bove, J.; Bordelon, Y.; Brannelly, P.; Brushaber, D.E.; Caso, C.; Coppola, G.; Dickerson, B.; Dickinson, S.; Domoto‐Reilly, K.; Faber, K.; Ferrall, J.; Fields, J.; Fishman, A.; Fong, J.; Foroud, T.; Forsberg, L.K.; Gearhart, D.; Ghazanfari, B.; Ghoshal, N.; Goldman, J.; Graff‐Radford, J.; Graff‐Radford, N.; Grant, I.; Grossman, M.; Haley, D.; Hsiung, G.‐Y.; Huey, E.; Irwin, D.; Jones, D.; Kantarci, K.; Karydas, A.; Kaufer, D.; Kerwin, D.; Knopman, D.; Kornak, J.; Kramer, J.H.; Kraft, R.; Kremers, W.K.; Kukull, W.; Litvan, I.; Ljubenkov, P.; Mackenzie, I.R.; Maldonado, M.; Manoochehri, M.; McGinnis, S.; McKinley, E.; Mendez, M.F.; Miller, B.L.; Onyike, C.; Pantelyat, A.; Pearlman, R.; Petrucelli, L.; Potter, M.; Rademakers, R.; Ramos, E.M.; Rankin, K.P.; Roberson, E.D.; Rogalski, E.; Sengdy, P.; Shaw, L.; Syrjanen, J.; Tartaglia, M.C.; Tatton, N.; Taylor, J.; Toga, A.; Trojanowski, J.; Weintraub, S.; Wong, B.; Wszolek, Z.; Boeve, B.F.; Rosen, H.J.; Boxer, A.L.;
• Is Part Of: Alzheimer's & dementia. Volume 16:Issue 1(2020)
• Page Start: 60
• Page End: 70
• Publication Date: 2020-01-08
• Subjects: bvFTD -- C9orf72 -- clinical trials -- frontotemporal dementia -- genetics -- GRN -- MAPT; Alzheimer's disease -- Periodicals; Alzheimer Disease -- Periodicals; Dementia -- Periodicals; Démence; Maladie d'Alzheimer; Périodique électronique (Descripteur de forme); Ressource Internet (Descripteur de forme); 616.83
• DOI: 10.1002/alz.12046
• Languages: English
• ISSNs: 1552-5260

Abstract

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.