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Comparison of sporadic and familial behavioral variant frontotemporal dementia (FTD) in a North American cohort.

  • Author(s): 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
  • ARTFL and LEFFTDS consortia
  • et al.
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.

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