Annus Ádám, Csáti Anett, Vécsei László
Department of Neurology, University of Szeged, Semmelweis u. 6, H-6725 Szeged, Hungary.
Department of Neurology, University of Szeged, Semmelweis u. 6, H-6725 Szeged, Hungary; MTA-SZTE Neuroscience Research Group, Szeged, Hungary.
Clin Neurol Neurosurg. 2016 Nov;150:125-132. doi: 10.1016/j.clineuro.2016.09.006. Epub 2016 Sep 17.
The transmissible spongiform encephalopathies, which include Creutzfeldt-Jakob disease, are fatal neurodegenerative disorders caused by the pathological accumulation of abnormal prion protein. The diagnosis of Creutzfeldt-Jakob disease is complex. The electroencephalogram, magnetic resonance imaging, lumbar puncture and genetic testing findings can help in the differential diagnosis of rapidly progressive dementia. There has recently been considerable debate as to whether proteins involved in the development of neurodegenerative diseases should be regarded as prions or only share prion-like mechanisms. Two recent reports described the detection of abnormal prion protein in the nasal mucosa and urine of patients with Creutzfeldt-Jakob disease. These findings raise major health concerns regarding the transmissibility of human prion diseases. We set out to address this neurological hot topic and to draw conclusions on the basis of what is known in the literature thus far.
包括克雅氏病在内的传染性海绵状脑病是由异常朊病毒蛋白的病理性积累引起的致命性神经退行性疾病。克雅氏病的诊断很复杂。脑电图、磁共振成像、腰椎穿刺和基因检测结果有助于快速进展性痴呆的鉴别诊断。最近，关于神经退行性疾病发展过程中涉及的蛋白质应被视为朊病毒还是仅具有类朊病毒机制存在相当大的争议。最近的两份报告描述了在克雅氏病患者的鼻黏膜和尿液中检测到异常朊病毒蛋白。这些发现引发了对人类朊病毒疾病传播性的重大健康担忧。我们着手探讨这个神经学热点话题，并根据迄今为止文献中的已知内容得出结论。
