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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 D.E.,
Caso C.,
Coppola G.,
Dickerson B.,
Dickinson S.,
DomotoReilly K.,
Faber K.,
Ferrall J.,
Fields J.,
Fishman A.,
Fong J.,
Foroud T.,
Forsberg L.K.,
Gearhart D.,
Ghazanfari B.,
Ghoshal N.,
Goldman J.,
GraffRadford J.,
GraffRadford 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.
Publication year - 2020
Publication title -
alzheimer's and dementia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 6.713
H-Index - 118
eISSN - 1552-5279
pISSN - 1552-5260
DOI - 10.1002/alz.12046
Subject(s) - frontotemporal dementia , c9orf72 , irritability , generalizability theory , medicine , cohort , depression (economics) , family history , clinical psychology , psychology , psychiatry , dementia , disease , oncology , anxiety , developmental psychology , economics , macroeconomics
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.