PET of Brain Prion Protein Amyloid in Gerstmann–Sträussler–Scheinker Disease
dc.contributor.author | Kepe, Vladimir | |
dc.contributor.author | Ghetti, Bernardino | |
dc.contributor.author | Farlow, Martin R. | |
dc.contributor.author | Bresjanac, Mara | |
dc.contributor.author | Miller, Karen | |
dc.contributor.author | Huang, Sung-Cheng | |
dc.contributor.author | Wong, Koon-Pong | |
dc.contributor.author | Murrell, Jill R. | |
dc.contributor.author | Piccardo, Pedro | |
dc.contributor.author | Epperson, Francine | |
dc.contributor.author | Repovš, Grega | |
dc.contributor.author | Smid, Lojze M. | |
dc.contributor.author | Petrič, Andrej | |
dc.contributor.author | Siddarth, Prabha | |
dc.contributor.author | Liu, Jie | |
dc.contributor.author | Satyamurthy, Nagichettiar | |
dc.contributor.author | Small, Gary W. | |
dc.contributor.author | Barrio, Jorge R. | |
dc.contributor.department | Pathology and Laboratory Medicine, School of Medicine | en_US |
dc.date.accessioned | 2023-03-13T16:35:09Z | |
dc.date.available | 2023-03-13T16:35:09Z | |
dc.date.issued | 2010-03 | |
dc.description.abstract | In vivo amyloid PET imaging was carried out on six symptomatic and asymptomatic carriers of PRNP mutations associated with the Gerstmann-Sträussler-Scheinker (GSS) disease, a rare familial neurodegenerative brain disorder demonstrating prion amyloid neuropathology, using 2-(1-{6-[(2-[F-18]fluoroethyl)(methyl)amino]-2-naphthyl}ethylidene)malononitrile ([F-18]FDDNP). 2-Deoxy-2-[F-18]fluoro-d-glucose PET ([F-18]FDG) and magnetic resonance imaging (MRI) scans were also performed in each subject. Increased [F-18]FDDNP binding was detectable in cerebellum, neocortex and subcortical areas of all symptomatic gene carriers in close association with the experienced clinical symptoms. Parallel glucose metabolism ([F-18]FDG) reduction was observed in neocortex, basal ganglia and/or thalamus, which supports the close relationship between [F-18]FDDNP binding and neuronal dysfunction. Two asymptomatic gene carriers displayed no cortical [F-18]FDDNP binding, yet progressive [F-18]FDDNP retention in caudate nucleus and thalamus was seen at 1- and 2-year follow-up in the older asymptomatic subject. In vitro FDDNP labeling experiments on brain tissue specimens from deceased GSS subjects not participating in the in vivo studies indicated that in vivo accumulation of [F-18]FDDNP in subcortical structures, neocortices and cerebellum closely related to the distribution of prion protein pathology. These results demonstrate the feasibility of detecting prion protein accumulation in living patients with [F-18]FDDNP PET, and suggest an opportunity for its application to follow disease progression and monitor therapeutic interventions. | en_US |
dc.eprint.version | Final published version | en_US |
dc.identifier.citation | Kepe V, Ghetti B, Farlow MR, et al. PET of brain prion protein amyloid in Gerstmann-Sträussler-Scheinker disease. Brain Pathol. 2010;20(2):419-430. doi:10.1111/j.1750-3639.2009.00306.x | en_US |
dc.identifier.uri | https://hdl.handle.net/1805/31861 | |
dc.language.iso | en_US | en_US |
dc.publisher | Wiley | en_US |
dc.relation.isversionof | 10.1111/j.1750-3639.2009.00306.x | en_US |
dc.relation.journal | Brain Pathology | en_US |
dc.rights | Attribution 4.0 International | * |
dc.rights.uri | http://creativecommons.org/licenses/by/4.0/ | * |
dc.source | PMC | en_US |
dc.subject | Amyloid | en_US |
dc.subject | Familial prion disease | en_US |
dc.subject | Positron emission tomography | en_US |
dc.subject | PRNP gene mutation | en_US |
dc.subject | Tau | en_US |
dc.subject | Transmissible spongiform encephalopathy | en_US |
dc.title | PET of Brain Prion Protein Amyloid in Gerstmann–Sträussler–Scheinker Disease | en_US |
dc.type | Article | en_US |