Zerr I, Polyakova T A
National Center of Neurodegenerative and Prion Diseases, Georg-August Gottingen University, Gottingen, Germany.
Russian Medical Academy of Postgraduate Education, Moscow.
Zh Nevrol Psikhiatr Im S S Korsakova. 2015;115(6. Vyp. 2):9-16. doi: 10.17116/jnevro2015115629-16.
In this article, authors analyzed a modern approach to the diagnosis of Creutzfeldt-Jakob disease (CJD) based on the clinical signs, cerebrospinal fluid markers, electroencephalography and magnetic resonance imaging. It was demonstrated for the first time that patients with late-onset CJD differed from younger CJD patients with respect to MRI profiles and initial clinical presentation. To date, cerebrospinal fluid (CSF) analysis, particularly protein 14-3-3 testing, presents an important approach to the identification of disease cases. A spectrum of differential diagnosis of rapid progressive dementia varied from neurodegenerative dementias to dementia due to acute neurological conditions. Real-time quaking-induced conversion (RT-QuIC) allows the amplification of miniscule amounts of scrapie prion protein. Recent studies applied the RT-QuIC methodology to CSF for the diagnosis of human prion diseases.
在本文中，作者分析了一种基于临床体征、脑脊液标志物、脑电图和磁共振成像的克雅氏病（CJD）现代诊断方法。首次证明，晚发型CJD患者在MRI特征和初始临床表现方面与年轻的CJD患者不同。迄今为止，脑脊液（CSF）分析，尤其是蛋白14-3-3检测，是识别病例的重要方法。快速进展性痴呆的鉴别诊断范围从神经退行性痴呆到急性神经系统疾病所致痴呆。实时震颤诱导转化（RT-QuIC）可扩增微量的瘙痒病朊病毒蛋白。最近的研究将RT-QuIC方法应用于脑脊液以诊断人类朊病毒疾病。
