Deters Kacie D, Risacher Shannon L, Yoder Karmen K, Oblak Adrian L, Unverzagt Frederick W, Murrell Jill R, Epperson Francine, Tallman Eileen F, Quaid Kimberly A, Farlow Martin R, Saykin Andrew J, Ghetti Bernardino
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.
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.
Am J Nucl Med Mol Imaging. 2016 Jan 28;6(1):84-93. eCollection 2016.
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.
格斯特曼-施特劳斯勒-谢inker病（GSS）是一种家族性神经退行性疾病，临床特征为共济失调、帕金森综合征和痴呆，神经病理学特征为朊蛋白（PrP）组成的弥漫性淀粉样斑块沉积。本研究的目的是评估[（11）C]匹兹堡化合物B（PiB）正电子发射断层扫描（PET）是否能够检测PRNP基因携带者中的PrP淀粉样蛋白。6名有GSS风险的个体和8名对照者采用标准方法进行了[（11）C]PiB PET扫描。在初次扫描约一年后，三名无症状携带者（两名携带PRNP P102L突变，一名携带PRNP F198S突变）中的每人均接受了第二次[（11）C]PiB PET扫描。三名P102L携带者、一名F198S携带者和一名F198S突变非携带者认知正常，而一名F198S携带者在本研究过程中出现认知障碍。在基线或随访时，任何受试者均未观察到[（11）C]PiB摄取。对有症状个体的神经病理学研究显示，大脑皮质、皮质下核和脑干中有PrP免疫阳性斑块和tau免疫阳性神经原纤维缠结。小脑皮质中PrP沉积也很多。这是第一项研究[（11）C]PiB PET结合GSS F198S受试者中PrP淀粉样蛋白能力的研究。这一发现表明，[（11）C]PiB PET不适用于GSS患者体内PrP淀粉样斑块的评估。
