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
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+cohortAbstract
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.
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