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Comparison of sporadic and familial behavioral variant frontotemporal dementia (FTD) in a North American cohort
- Heuer, Hilary W;
- Wang, P;
- Rascovsky, K;
- Wolf, A;
- Appleby, B;
- Bove, J;
- Bordelon, Y;
- Brannelly, P;
- Brushaber, DE;
- Caso, C;
- Coppola, G;
- Dickerson, B;
- Dickinson, S;
- Domoto‐Reilly, K;
- Faber, K;
- Ferrall, J;
- Fields, J;
- Fishman, A;
- Fong, J;
- Foroud, T;
- Forsberg, LK;
- 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, JH;
- Kraft, R;
- Kremers, WK;
- Kukull, W;
- Litvan, I;
- Ljubenkov, P;
- Mackenzie, IR;
- Maldonado, M;
- Manoochehri, M;
- McGinnis, S;
- McKinley, E;
- Mendez, MF;
- Miller, BL;
- Onyike, C;
- Pantelyat, A;
- Pearlman, R;
- Petrucelli, L;
- Potter, M;
- Rademakers, R;
- Ramos, EM;
- Rankin, KP;
- Roberson, ED;
- Rogalski, E;
- Sengdy, P;
- Shaw, L;
- Syrjanen, J;
- Tartaglia, MC;
- Tatton, N;
- Taylor, J;
- Toga, A;
- Trojanowski, J;
- Weintraub, S;
- Wong, B;
- Wszolek, Z;
- Boeve, BF;
- Rosen, HJ;
- Boxer, AL;
- consortia, on behalf of the ARTFL and LEFFTDS
- et al.
Published Web Location
https://www.ncbi.nlm.nih.gov/pubmed/?term=Comparison+of+sporadic+and+familial+behavioral+variant+frontotemporal+dementia+(FTD)+in+a+North+American+cohortNo data is associated with this publication.
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.Many UC-authored scholarly publications are freely available on this site because of the UC's open access policies. Let us know how this access is important for you.