Zaborowski Adam
Z I Kliniki Psychiatrycznej AM w Lodzi.
Psychiatr Pol. 2004 Mar-Apr;38(2):283-96.
In the first part of this work the main problems of prion diseases--also called transmissible cerebral amyloidoses (TCA) or subacute (transmissible) encephalopathies (SSE, TSE)--and clinical symptoms of Creutzfeldt-Jakob disease are presented. Some problems of neuropathology of Creutzfeldt-Jakob disease and basic informations about other human prion diseases will be presented in the second part. The growth of the interest in prion diseases during last years is caused by the problem of bovine spongiform encephalopathy (BSE or "mad cow disease") and its transmission into a human. The new variant of Creutzfeldt-Jakob disease (nvCJD) has appeared. Prion diseases: Gerstmann-Sträussler-Scheinker syndrome (GSS), kuru, fatal familial insomnia (FFI) and particularly the most frequent of them--Creutzfeldt-Jakob disease (CJD)--have nonspecific, sometimes variable clinical (psychopathological and neurological) symptoms. The imaging, EEG, cerebrospinal fluid tests and other laboratory tests are not specific either and their diagnostic value is limited. Neuropathological studies are needed but their interpretation is often difficult. The only certain diagnostic marker for TSE is the presence of PrP(Sc), the prion protein, which is presently believed to be a direct cause for all transmissible cerebral amyloidoses (TCA).
在本研究的第一部分，介绍了朊病毒疾病（也称为传染性脑淀粉样变性病（TCA）或亚急性（传染性）脑病（SSE、TSE））的主要问题以及克雅氏病的临床症状。克雅氏病神经病理学的一些问题以及其他人类朊病毒疾病的基本信息将在第二部分介绍。近年来对朊病毒疾病兴趣的增长是由牛海绵状脑病（BSE或“疯牛病”）及其传播给人类的问题引起的。克雅氏病的新变种（nvCJD）已经出现。朊病毒疾病：格斯特曼-施特劳斯勒-谢inker综合征（GSS）、库鲁病、致死性家族性失眠症（FFI），尤其是其中最常见的克雅氏病（CJD），具有非特异性的、有时多变的临床（精神病理学和神经学）症状。影像学、脑电图、脑脊液检查和其他实验室检查也不具有特异性，其诊断价值有限。需要进行神经病理学研究，但其解读往往很困难。TSE唯一确定的诊断标志物是朊病毒蛋白PrP(Sc)的存在，目前认为它是所有传染性脑淀粉样变性病（TCA）的直接病因。
