Citations 2
Comparison of sporadic and familial behavioral variant frontotemporal dementia (FTD) in a North American cohort
Heuer, Hilary W. , Wang, P., Rascovsky, Katya, Bove, Jessica, Bordelon, Yvette , Brannelly, Patrick , Brushaber, Danielle , Caso, Christine, Coppola, Giovanni , Dickerson, Brad C., Dickinson, Susan, Domoto‐Reilly, Kimiko , Faber, Kelley , Ferrall, Jessica, Fields, Julie A. , Fishman, Ann, Forsberg, Leah K. , Gearhart, Debra, Ghazanfari, Behnaz, Ghoshal, Nupur , Graff‐Radford, Jonathan , Graff-Radford, N. R., Grant, Ian , Haley, Dana , Hsiung, Ging‐Yuek Robin , Huey, Edward D. , Irwin, David J. , Kantarci, Kejal , Karydas, Anna, Kaufer, Daniel , Kornak, John , Kramer, Joel H. , Kraft, Ruth, Kremers, Walter K. , Kukull, Walter A. , Ljubenkov, Peter A. , Mackenzie, Ian R. , Maldonado, Miranda, Manoochehri, Masood , McGinnis, Scott, McKinley, Emily , Mendez, Mario F. , Miller, Bruce L. , Onyike, Chiadi U. , Pantelyat, Alexander , Pearlman, Rodney , Petrucelli, Leonard , Rademakers, Rosa , Ramos, Eliana Marisa , Rankin, Katherine P. , Roberson, Erik D. , Rogalskı, Emily , Sengdy, Pheth, Shaw, Leslie M. , Syrjanen, Jeremy A. , Tartaglia, Maria Carmela , Tatton, Nadine , Taylor, Joanne, Trojanowski, John Q. , Weıntraub, Sandra , Wong, Bonnie , Rosen, Howard J. , Boxer, Adam L. , consortia, on behalf of the ARTFL and LEFFTDS, Irwin, David J , Hsiung, Gink-Yuek , Roberson, Erik , Miller, Bruce L , Kantarci, Kejal , Pantelyat, Alex , Tartaglia, Maria Carmela , Kremers, Walter K , Domoto-Reilly, Kimiko , Wolf, A., Appleby, Brian S. , Fong, Jamie , Foroud, Tatiana , Goldman, Jill , Grossman, Murray , Jones, David T. , Kerwin, Diana , Knopman, David S. , Litvan, Irene , Potter, M., Toga, Arthur W. , Wszołek, Zbigniew K. , Boeve, Bradley F. , consortia, on behalf of the ARTFL and LEFFTDS
Alzheimer's & Dementia | January 16, 2020 | Volume: 16 | Issue: 1 | Pages: 60-70
AbstractIntroductionBehavioral 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.MethodsA 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.ResultsParticipants 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.Discussionf‐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.
Clinical and volumetric changes with increasing functional impairment in familial frontotemporal lobar degeneration
Olney, Nicholas , Ong, Elise, Goh, Sheng‐Yang M., Bajorek, Lynn, Dever, Reilly, Staffaroni, Adam M. , Bock, Meredith , Chiang, Kevin, Ljubenkov, Peter A. , Kornak, John , Heuer, Hilary W. , Rascovsky, Katya, Wolf, Amelia, Bove, Jessica, Bordelon, Yvette , Brannelly, Patrick , Brushaber, Danielle , Caso, Christine, Coppola, Giovanni , Dickinson, Susan, Domoto‐Reilly, Kimiko , Faber, Kelly, Ferrall, Jessica, Fields, Julie A. , Fishman, Ann, Foroud, Tatiana M., Forsberg, Leah K. , Gearhart, Debra, Ghazanfari, Behnaz, Ghoshal, Nupur , Graff‐Radford, Jonathan, Graff‐Radford, Neill R. , Grant, Ian , Haley, Dana , Hsiung, Ging‐Yuek Robin , Huey, Edward D. , Irwin, David J. , Kantarci, Kejal , Karydas, Anna M., Kaufer, Daniel , Kramer, Joel H. , Kraft, Ruth, Kremers, Walter K. , Kukull, Walter A. , Mackenzie, Ian R. , Maldonado, Miranda, Manoochehri, Masood , McGinnis, Scott M., McKinley, Emily , Mendez, Mario F. , Miller, Bruce L. , Onyike, Chiadi U. , Pantelyat, Alexander , Pearlman, Rodney , Petrucelli, Len, Potter, Madeleine, Rademakers, Rosa , Ramos, Eliana Marisa , Rankin, Katherine P. , Roberson, Erik D. , Rogalskı, Emily , Sengdy, Pheth, Shaw, Leslie M. , Syrjanen, Jeremy A. , Tartaglia, Maria Carmela , Tatton, Nadine , Taylor, Joanne, Trojanowski, John Q. , Weıntraub, Sandra , Wong, Bonnie , Boxer, Adam L. , Rosen, Howard J. , consortia, on behalf of the ARTFL and LEFFTDS, Gearhart, Debra J, Boeve, Brad F, Irwin, David J , Hsiung, Gink-Yuek , Roberson, Erik , Miller, Bruce L , Kantarci, Kejal , Pantelyat, Alex , Tartaglia, Maria Carmela , Kremers, Walter K , Domoto-Reilly, Kimiko , Cobigo, Yann , Wang, Ping , Appleby, Brian S. , Dickerson, Bradford Clark , Fong, Jamie , Goldman, Jill , Graff‐Radford, Jonathan , Grossman, Murray , Jones, David T. , Kerwin, Diana , Knopman, David S. , Lapid, Maria I. , Litvan, Irene , Potter, Madeleine, Toga, Arthur W. , Wszołek, Zbigniew K. , Boeve, Bradley F. , consortia, on behalf of the ARTFL and LEFFTDS
Alzheimer's & Dementia | November 26, 2019 | Volume: 16 | Issue: 1 | Pages: 49-59
AbstractIntroductionThe Advancing Research and Treatment in Frontotemporal Lobar Degeneration and Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects longitudinal studies were designed to describe the natural history of familial‐frontotemporal lobar degeneration due to autosomal dominant mutations.MethodsWe examined cognitive performance, behavioral ratings, and brain volumes from the first time point in 320 MAPT, GRN, and C9orf72 family members, including 102 non–mutation carriers, 103 asymptomatic carriers, 43 mildly/questionably symptomatic carriers, and 72 carriers with dementia.ResultsAsymptomatic carriers showed similar scores on all clinical measures compared with noncarriers but reduced frontal and temporal volumes. Those with mild/questionable impairment showed decreased verbal recall, fluency, and Trail Making Test performance and impaired mood and self‐monitoring. Dementia was associated with impairment in all measures. All MAPT carriers with dementia showed temporal atrophy, but otherwise, there was no single cognitive test or brain region that was abnormal in all subjects.DiscussionImaging changes appear to precede clinical changes in familial‐frontotemporal lobar degeneration, but specific early clinical and imaging changes vary across individuals.