Amyloid imaging in distinguishing atypical prion disease from Alzheimer disease
Amyloid imaging in distinguishing atypical prion disease from Alzheimer disease
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DOI：
10.1212/01.wnl.0000265815.38958.b6
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发表时间：
2007-07-17
期刊：
影响因子：
9.9
通讯作者：
Miller, B. L.
中科院分区：
文献类型：
--
作者：
Boxer, A. L.;Rabinovici, G. D.;Miller, B. L.
目的：比较朊蛋白（PrP）基因（PRNP）突变导致类似阿尔茨海默病（AD）临床综合征的人类受试者体内两种淀粉样蛋白结合PET药物（PIB和FDDNP）的摄取。背景：特异性PET配体的淀粉样蛋白成像为AD的早期检测和鉴别诊断提供了巨大的希望。朊病毒病的遗传形式可以呈现类似于AD的临床特征，并且在尸检时可以显示突变的PrP-淀粉样蛋白的沉积。FDDNP结合PrP-淀粉样蛋白在死后的人类标本，但还没有报告在体内朊病毒疾病。PIB结合PrP-淀粉样蛋白的能力尚不清楚。研究方法：在PRNP基因中有6个八肽重复插入突变（6-OPRI）的两兄弟接受了临床、结构MRI和FDG-PET评价。一个兄弟接受了PIB-PET评估，另一个接受了FDDNP-PET扫描。PET结果与5名正常受试者和5名AD患者进行了比较。结果：一个兄弟的PIB摄取与对照组相似，而另一个兄弟的FDDNP摄取介于AD和对照组之间。结论：不同的淀粉样蛋白结合剂可能对朊病毒相关的脑病理具有不同的敏感性。淀粉样蛋白显像剂的组合可能有助于早发性痴呆的诊断。
Objective: To compare the in vivo uptake of two amyloid-binding PET agents, PIB and FDDNP, in human subjects with a prion protein (PrP) gene (PRNP) mutation that produces a clinical syndrome similar to Alzheimer disease (AD). Background: Amyloid imaging with specific PET ligands offers great promise for early detection and differential diagnosis of AD. Genetic forms of prion disease can present with clinical features that resemble AD, and at autopsy may show deposition of mutant PrP-amyloid. FDDNP binds to PrP-amyloid in postmortem human specimens, but has not been reported in vivo in prion disease. The ability of PIB to bind PrP-amyloid is not known. Methods: Two brothers with a 6 octapeptide repeat insertion mutation (6-OPRI) in the PRNP gene underwent clinical, structural MRI, and FDG-PET evaluations. One brother received a PIB-PET evaluation, while the other received an FDDNP-PET scan. PET results were compared with five normal subjects and five individuals with AD scanned with either agent. Results: PIB uptake was similar to controls in one brother, while FDDNP uptake was intermediate between AD and controls in the other brother. Conclusions: Different amyloid-binding agents may have differential sensitivity to prion-related brain pathology. A combination of amyloid imaging agents may be useful in the diagnosis of early-onset dementia.