title: [(11)C]PiB PET in Gerstmann-Sträussler-Scheinker disease.
abstract: Gerstmann-Sträussler-Scheinker Disease (GSS) is a familial neurodegenerative disorder characterized clinically by ataxia, parkinsonism, and dementia, and neuropathologically by deposition of diffuse and amyloid plaques composed of prion protein (PrP). The purpose of this study was to evaluate if [(11)C]Pittsburgh Compound B (PiB) positron emission tomography (PET) is capable of detecting PrP-amyloid in PRNP gene carriers. Six individuals at risk for GSS and eight controls underwent [(11)C]PiB PET scans using standard methods. Approximately one year after the initial scan, each of the three asymptomatic carriers (two with PRNP P102L mutation, one with PRNP F198S mutation) underwent a second [(11)C]PiB PET scan. Three P102L carriers, one F198S carrier, and one non-carrier of the F198S mutation were cognitively normal, while one F198S carrier was cognitively impaired during the course of this study. No [(11)C]PiB uptake was observed in any subject at baseline or at follow-up. Neuropathologic study of the symptomatic individual revealed PrP-immunopositive plaques and tau-immunopositive neurofibrillary tangles in cerebral cortex, subcortical nuclei, and brainstem. PrP deposits were also numerous in the cerebellar cortex. This is the first study to investigate the ability of [(11)C]PiB PET to bind to PrP-amyloid in GSS F198S subjects. This finding suggests that [(11)C]PiB PET is not suitable for in vivo assessment of PrP-amyloid plaques in patients with GSS.
authors.affiliation: Center for Neuroimaging, Department of Radiology and Imaging Sciences, Indiana University School of MedicineIndianapolis, IN, USA; Indiana Alzheimer Disease Center, Indiana University School of MedicineIndianapolis, IN, USA; Program in Medical Neuroscience, Paul and Carole Stark Neurosciences Research Institute, Indiana University School of MedicineIndianapolis, IN, USA.
authors.forename: Kacie D
authors.initials: KD
authors.lastname: Deters
authors.affiliation: Center for Neuroimaging, Department of Radiology and Imaging Sciences, Indiana University School of MedicineIndianapolis, IN, USA; Indiana Alzheimer Disease Center, Indiana University School of MedicineIndianapolis, IN, USA.
authors.forename: Shannon L
authors.initials: SL
authors.lastname: Risacher
authors.affiliation: Center for Neuroimaging, Department of Radiology and Imaging Sciences, Indiana University School of MedicineIndianapolis, IN, USA; Indiana Alzheimer Disease Center, Indiana University School of MedicineIndianapolis, IN, USA.
authors.forename: Karmen K
authors.initials: KK
authors.lastname: Yoder
authors.affiliation: Indiana Alzheimer Disease Center, Indiana University School of MedicineIndianapolis, IN, USA; Department of Pathology and Laboratory Medicine, Indiana University School of MedicineIndianapolis, IN, USA.
authors.forename: Adrian L
authors.initials: AL
authors.lastname: Oblak
authors.affiliation: Indiana Alzheimer Disease Center, Indiana University School of MedicineIndianapolis, IN, USA; Department of Psychiatry, Indiana University School of MedicineIndianapolis, IN, USA.
authors.forename: Frederick W
authors.initials: FW
authors.lastname: Unverzagt
authors.affiliation: Indiana Alzheimer Disease Center, Indiana University School of MedicineIndianapolis, IN, USA; Department of Pathology and Laboratory Medicine, Indiana University School of MedicineIndianapolis, IN, USA.
authors.forename: Jill R
authors.initials: JR
authors.lastname: Murrell
authors.affiliation: Indiana Alzheimer Disease Center, Indiana University School of MedicineIndianapolis, IN, USA; Department of Pathology and Laboratory Medicine, Indiana University School of MedicineIndianapolis, IN, USA.
authors.forename: Francine
authors.initials: F
authors.lastname: Epperson
authors.affiliation: Center for Neuroimaging, Department of Radiology and Imaging Sciences, Indiana University School of MedicineIndianapolis, IN, USA; Indiana Alzheimer Disease Center, Indiana University School of MedicineIndianapolis, IN, USA.
authors.forename: Eileen F
authors.initials: EF
authors.lastname: Tallman
authors.affiliation: Indiana Alzheimer Disease Center, Indiana University School of MedicineIndianapolis, IN, USA; Department of Medical and Molecular Genetics, Indiana University School of MedicineIndianapolis, IN, USA.
authors.forename: Kimberly A
authors.initials: KA
authors.lastname: Quaid
authors.affiliation: Indiana Alzheimer Disease Center, Indiana University School of MedicineIndianapolis, IN, USA; Department of Neurology, Indiana University School of MedicineIndianapolis, IN, USA.
authors.forename: Martin R
authors.initials: MR
authors.lastname: Farlow
authors.affiliation: Center for Neuroimaging, Department of Radiology and Imaging Sciences, Indiana University School of MedicineIndianapolis, IN, USA; Indiana Alzheimer Disease Center, Indiana University School of MedicineIndianapolis, IN, USA; Department of Psychiatry, Indiana University School of MedicineIndianapolis, IN, USA; Department of Medical and Molecular Genetics, Indiana University School of MedicineIndianapolis, IN, USA; Department of Neurology, Indiana University School of MedicineIndianapolis, IN, USA.
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Saykin
authors.affiliation: Indiana Alzheimer Disease Center, Indiana University School of MedicineIndianapolis, IN, USA; Department of Psychiatry, Indiana University School of MedicineIndianapolis, IN, USA; Department of Pathology and Laboratory Medicine, Indiana University School of MedicineIndianapolis, IN, USA; Department of Medical and Molecular Genetics, Indiana University School of MedicineIndianapolis, IN, USA; Department of Neurology, Indiana University School of MedicineIndianapolis, IN, USA.
authors.forename: Bernardino
authors.initials: B
authors.lastname: Ghetti
keywords: Gerstmann-Sträussler-Scheinker disease (GSS); [11C]Pittsburgh compound B (PiB) positron emission tomography (PET); amyloid; neuroimaging; prion disease