title: Positive florbetapir PET amyloid imaging in a subject with frequent cortical neuritic plaques and frontotemporal lobar degeneration with TDP43-positive inclusions.
abstract: Abnormal neuronal accumulation and modification of TAR DNA binding protein 43 (TDP-43) have recently been discovered to be defining histopathological features of particular subtypes of frontotemporal dementia and amyotrophic lateral sclerosis, and are also common in aging, particularly coexisting with hippocampal sclerosis and Alzheimer's disease pathology. This case report describes a 72 year old Hispanic male with no family history of neurological disease, who presented at age 59 with obsessive behavior, anxiety, agitation, and dysphasia. Positron emission tomography imaging using the amyloid ligand 18F florbetapir (Amyvid) was positive. Postmortem examination revealed frequent diffuse and neuritic amyloid plaques throughout the cerebral cortex, thalamus, and striatum, Braak stage II neurofibrillary degeneration, and frequent frontal and temporal cortex TDP-43-positive neurites with rare nuclear inclusions. The case is unusual and instructive because of the co-existence of frequent cortical and diencephalic amyloid plaques with extensive TDP-43-positive histopathology in the setting of early-onset dementia and because it demonstrates that a positive cortical amyloid imaging signal in a subject with dementia does not necessarily establish that Alzheimer's disease is the sole cause.
authors.affiliation: Banner Sun Health Research Institute, Sun City, AZ, USA.
authors.forename: Geidy E
authors.initials: GE
authors.lastname: Serrano
authors.affiliation: Banner Sun Health Research Institute, Sun City, AZ, USA.
authors.forename: Marwan N
authors.initials: MN
authors.lastname: Sabbagh
authors.affiliation: Banner Sun Health Research Institute, Sun City, AZ, USA.
authors.forename: Lucia I
authors.initials: LI
authors.lastname: Sue
authors.affiliation: Banner Sun Health Research Institute, Sun City, AZ, USA.
authors.forename: Jose A
authors.initials: JA
authors.lastname: Hidalgo
authors.affiliation: Rush University Medical Center, Chicago, IL, USA.
authors.forename: Julie A
authors.initials: JA
authors.lastname: Schneider
authors.affiliation: Biospective Inc. and Montreal Neurological Institute, McGill University, Montreal, Canada.
authors.forename: Barry J
authors.initials: BJ
authors.lastname: Bedell
authors.affiliation: University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Vivianna M
authors.initials: VM
authors.lastname: Van Deerlin
authors.affiliation: University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Eunran
authors.initials: E
authors.lastname: Suh
authors.affiliation: Tokyo Institute of Psychiatry, Tokyo, Japan.
authors.forename: Haruhiko
authors.initials: H
authors.lastname: Akiyama
authors.affiliation: Avid Radiopharmaceuticals, Philadelphia, PA, USA.
authors.forename: Abhinay D
authors.initials: AD
authors.lastname: Joshi
authors.affiliation: Avid Radiopharmaceuticals, Philadelphia, PA, USA.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Pontecorvo
authors.affiliation: Avid Radiopharmaceuticals, Philadelphia, PA, USA.
authors.forename: Mark A
authors.initials: MA
authors.lastname: Mintun
authors.affiliation: Banner Sun Health Research Institute, Sun City, AZ, USA.
authors.forename: Thomas G
authors.initials: TG
authors.lastname: Beach
mesh_terms: D000368:Aged; D000814:Aniline Compounds; D004268:DNA-Binding Proteins / Q000235:genetics*; D005026:Ethylene Glycols; D057174:Frontotemporal Lobar Degeneration / Q000150:complications / Q000000981:diagnostic imaging* / Q000235:genetics; D006801:Humans; D002479:Inclusion Bodies / Q000378:metabolism; D008297:Male; D058225:Plaque, Amyloid / Q000150:complications / Q000000981:diagnostic imaging* / Q000235:genetics / Q000473:pathology; D049268:Positron-Emission Tomography / Q000379:methods*; D014057:Tomography, X-Ray Computed
chemical_list: D000814:Aniline Compounds; D004268:DNA-Binding Proteins; D005026:Ethylene Glycols; C545186:florbetapir
keywords: Alzheimer's disease; frontotemporal dementia; neuritic amyloid plaques; neurofibrillary degeneration