title: Amyloid in dementia associated with familial FTLD: not an innocent bystander.
abstract: Patients with frontotemporal lobar degeneration (FTLD) can show superimposed amyloid pathology, though the impact of amyloid on the clinical presentation of FTLD is not well characterized. This cross-sectional case-control study compared clinical features, fluorodeoxyglucose-positron emission tomography metabolism and gray matter volume loss in 30 patients with familial FTLD in whom amyloid status was confirmed with autopsy or Pittsburgh compound B-PET. Compared to the amyloid-negative patients, the amyloid-positive patients performed significantly worse on several cognitive tests and showed hypometabolism and volume loss in more temporoparietal regions. Our results suggest that in FTLD amyloid positivity is associated with a more Alzheimer's disease-like pattern of neurodegeneration.
authors.affiliation: a Memory and Aging Center, Department of Neurology , University of California , San Francisco , CA , USA.
authors.forename: Georges
authors.initials: G
authors.lastname: Naasan
authors.affiliation: a Memory and Aging Center, Department of Neurology , University of California , San Francisco , CA , USA.
authors.forename: Gil D
authors.initials: GD
authors.lastname: Rabinovici
authors.affiliation: a Memory and Aging Center, Department of Neurology , University of California , San Francisco , CA , USA.
authors.forename: Pia
authors.initials: P
authors.lastname: Ghosh
authors.affiliation: a Memory and Aging Center, Department of Neurology , University of California , San Francisco , CA , USA.
authors.forename: Jonathan D
authors.initials: JD
authors.lastname: Elofson
authors.affiliation: a Memory and Aging Center, Department of Neurology , University of California , San Francisco , CA , USA.
authors.forename: Bruce L
authors.initials: BL
authors.lastname: Miller
authors.affiliation: d Department of Psychiatry, Semel Institute for Neuroscience and Human Behavior, David Geffen School of Medicine , University of California , Los Angeles , CA , USA.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Coppola
authors.affiliation: a Memory and Aging Center, Department of Neurology , University of California , San Francisco , CA , USA.
authors.forename: Anna
authors.initials: A
authors.lastname: Karydas
authors.affiliation: a Memory and Aging Center, Department of Neurology , University of California , San Francisco , CA , USA.
authors.forename: Jamie
authors.initials: J
authors.lastname: Fong
authors.affiliation: a Memory and Aging Center, Department of Neurology , University of California , San Francisco , CA , USA.
authors.forename: David
authors.initials: D
authors.lastname: Perry
authors.affiliation: a Memory and Aging Center, Department of Neurology , University of California , San Francisco , CA , USA.
authors.forename: Suzee E
authors.initials: SE
authors.lastname: Lee
authors.affiliation: a Memory and Aging Center, Department of Neurology , University of California , San Francisco , CA , USA.
authors.forename: Jennifer S
authors.initials: JS
authors.lastname: Yokoyama
authors.affiliation: a Memory and Aging Center, Department of Neurology , University of California , San Francisco , CA , USA.
authors.forename: William W
authors.initials: WW
authors.lastname: Seeley
authors.affiliation: a Memory and Aging Center, Department of Neurology , University of California , San Francisco , CA , USA.
authors.forename: Joel H
authors.initials: JH
authors.lastname: Kramer
authors.affiliation: b Department of Radiology , University of California , San Francisco , CA , USA.
authors.forename: Michael W
authors.initials: MW
authors.lastname: Weiner
authors.affiliation: b Department of Radiology , University of California , San Francisco , CA , USA.
authors.forename: Norbert
authors.initials: N
authors.lastname: Schuff
authors.affiliation: c School of Public Health , University of California Berkeley and Lawrence Berkeley National Laboratory , Berkeley , CA , USA.
authors.forename: William J
authors.initials: WJ
authors.lastname: Jagust
authors.affiliation: a Memory and Aging Center, Department of Neurology , University of California , San Francisco , CA , USA.
authors.forename: Lea T
authors.initials: LT
authors.lastname: Grinberg
authors.affiliation: d Department of Psychiatry, Semel Institute for Neuroscience and Human Behavior, David Geffen School of Medicine , University of California , Los Angeles , CA , USA.
authors.forename: Mochtar
authors.initials: M
authors.lastname: Pribadi
authors.affiliation: d Department of Psychiatry, Semel Institute for Neuroscience and Human Behavior, David Geffen School of Medicine , University of California , Los Angeles , CA , USA.
authors.forename: Zhongan
authors.initials: Z
authors.lastname: Yang
authors.affiliation: d Department of Psychiatry, Semel Institute for Neuroscience and Human Behavior, David Geffen School of Medicine , University of California , Los Angeles , CA , USA.
authors.forename: Renee
authors.initials: R
authors.lastname: Sears
authors.affiliation: d Department of Psychiatry, Semel Institute for Neuroscience and Human Behavior, David Geffen School of Medicine , University of California , Los Angeles , CA , USA.
authors.forename: Eric
authors.initials: E
authors.lastname: Klein
authors.affiliation: d Department of Psychiatry, Semel Institute for Neuroscience and Human Behavior, David Geffen School of Medicine , University of California , Los Angeles , CA , USA.
authors.forename: Kevin
authors.initials: K
authors.lastname: Wojta
authors.affiliation: a Memory and Aging Center, Department of Neurology , University of California , San Francisco , CA , USA.
authors.forename: Howard J
authors.initials: HJ
authors.lastname: Rosen
mesh_terms: D000368:Aged; D000682:Amyloid / Q000378:metabolism*; D016229:Amyloid beta-Peptides / Q000378:metabolism*; D001921:Brain / Q000378:metabolism / Q000473:pathology*; D016022:Case-Control Studies; D003430:Cross-Sectional Studies; D005260:Female; D057180:Frontotemporal Dementia / Q000378:metabolism / Q000473:pathology*; D057174:Frontotemporal Lobar Degeneration / Q000378:metabolism / Q000473:pathology*; D066128:Gray Matter / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged
chemical_list: D000682:Amyloid; D016229:Amyloid beta-Peptides
keywords: Alzheimer’s disease; FDG-PET; MRI; amyloid; frontotemporal dementia