Johnson Richard T
Pathology 627, The Johns Hopkins University School of Medicine & Bloomberg School of Public Health, Baltimore, MD 21287, USA.
Lancet Neurol. 2005 Oct;4(10):635-42. doi: 10.1016/S1474-4422(05)70192-7.
Prion diseases are degenerative disorders of the nervous system caused by transmissible particles that contain a pathogenic isoform of the prion protein, a normal constituent of cell membranes. The most common human prion disease is Creutzfeldt-Jakob disease (CJD). Most cases are sporadic with unknown mode of transmission, 10-15% of cases are inherited, and a small number have been transmitted by medical procedures. The spread of human prion diseases through consumption of infected material has been implicated historically in kuru and recently in variant CJD. Animal prion diseases (scrapie of sheep, transmissible mink encephalopathy, chronic wasting disease of cervids, and bovine spongiform encephalopathy) all seem to be laterally transmitted by contact with infected animals or by consumption of infected feed. The different modes of transmission of different prion diseases, the unpredictable species barriers, the variable distribution of infectivity in tissues, and strain variations found in some diseases all make risk assessment and predictions of future events difficult.
朊病毒疾病是由可传播颗粒引起的神经系统退行性疾病，这些颗粒含有朊病毒蛋白的致病异构体，而朊病毒蛋白是细胞膜的正常组成成分。最常见的人类朊病毒疾病是克雅氏病（CJD）。大多数病例是散发性的，传播方式不明，10% - 15%的病例是遗传性的，少数病例是通过医疗程序传播的。人类朊病毒疾病通过食用受感染物质传播，历史上曾与库鲁病有关，最近又与变异型克雅氏病有关。动物朊病毒疾病（绵羊瘙痒病、传染性水貂脑病、鹿慢性消耗病和牛海绵状脑病）似乎都通过与受感染动物接触或食用受感染饲料而横向传播。不同朊病毒疾病的不同传播方式、不可预测的种间屏障、组织中传染性的可变分布以及某些疾病中发现的毒株变异，都使得风险评估和对未来事件的预测变得困难。
