Llorens Franc, Karch André, Golanska Ewa, Schmitz Matthias, Lange Peter, Sikorska Beata, Liberski Pawel P, Zerr Inga
Department of Neurology, University Medical Center Göttingen, and German Center for Neurodegenerative Diseases (DZNE)-site Göttingen, Göttingen, Germany.
Dement Geriatr Cogn Disord. 2017;43(1-2):71-80. doi: 10.1159/000454802. Epub 2017 Jan 6.
Several biomarkers have been proposed to discriminate sporadic Creutzfeldt-Jakob disease (sCJD) from other dementias and control cases. However, their clinical accuracy depends on the PRNP codon 129 genotype, leaving it unclear how well established markers behave in untested conditions.
We analyzed 14-3-3, tau, p-tau levels, and the p-tau/tau ratio in a population sample collected from Polish hospitals including nondementia, dementia, and definite sCJD cases and validated their parameters according to previously established cutoffs. Additionally, the correlation between biomarkers and disease duration as well as the influence of the PRNP129 polymorphism are reported.
The tau levels and p-tau/tau ratios differed considerably between sCJD and clinically characterized non-CJD cases (p < 0.001). p-tau was only elevated in sCJD when compared to cases without dementia (p < 0.05). Tau and the p-tau/tau ratio showed a sensitivity of 95 and 100%, respectively, in detecting sCJD cases. A negative correlation between tau levels and disease duration, but not the timing of lumbar puncture was observed.
The present findings confirmed the value of the p-tau/tau ratio as a robust sCJD biomarker and suggest a role for tau as prognostic marker.
已经提出了几种生物标志物来区分散发性克雅氏病（sCJD）与其他痴呆症及对照病例。然而，它们的临床准确性取决于PRNP密码子129基因型，尚不清楚既定标志物在未经测试的情况下表现如何。
我们分析了从波兰医院收集的人群样本中的14-3-3、tau、p-tau水平以及p-tau/tau比值，该样本包括非痴呆、痴呆和确诊的sCJD病例，并根据先前确定的临界值验证了它们的参数。此外，报告了生物标志物与疾病持续时间之间的相关性以及PRNP129多态性的影响。
sCJD与具有临床特征的非CJD病例之间的tau水平和p-tau/tau比值存在显著差异（p < 0.001）。与无痴呆病例相比，p-tau仅在sCJD中升高（p < 0.05）。Tau和p-tau/tau比值在检测sCJD病例时的敏感性分别为95%和100%。观察到tau水平与疾病持续时间呈负相关，但与腰椎穿刺时间无关。
本研究结果证实了p-tau/tau比值作为一种可靠的sCJD生物标志物的价值，并表明tau作为预后标志物的作用。
