التفاصيل البيبلوغرافية
| العنوان: |
PET of Brain Prion Protein Amyloid in Gerstmann–Sträussler–Scheinker Disease. |
| المؤلفون: |
Kepe, Vladimir, Ghetti, Bernardino, Farlow, Martin R., Bresjanac, Mara, Miller, Karen, Sung-Cheng Huang, Koon-Pong Wong, Murrell, Jill R., Piccardo, Pedro, Epperson, Francine, Repovš, Grega, Smid, Lojze M., Petrič, Andrej, Siddarth, Prabha, Jie Liu, Satyamurthy, Nagichettiar, Small, Gary W., Barrio, Jorge R. |
| المصدر: |
Brain Pathology; Mar2010, Vol. 20 Issue 2, p419-430, 12p, 2 Color Photographs, 1 Diagram, 3 Charts, 1 Graph |
| مصطلحات موضوعية: |
POSITRON emission tomography, DIAGNOSTIC imaging, RADIONUCLIDE imaging, MAGNETIC resonance imaging, CEREBELLUM, BASAL ganglia, NEOCORTEX |
| مستخلص: |
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. [ABSTRACT FROM AUTHOR] |
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| قاعدة البيانات: |
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