Ramasamy I
Department of Chemical Pathology, Newham General Hospital, Glen Road, Plaistow, London E13 8RU, UK.
Public Health. 2004 Sep;118(6):409-20. doi: 10.1016/j.puhe.2003.12.016.
The transmissible spongiform encephalopathies (TSEs), thought to be caused by prions, are fatal neurodegenerative disorders of humans and animals. Despite their rarity, human prion diseases have received prominence because the consumption of prion-contaminated meat from cattle with bovine spongiform encephalopathy (BSE) is thought to be responsible for the emergence of variant Creutzfeldt-Jakob disease (vCJD) in humans. Clinical criteria for the diagnosis of vCJD is now available. Recent, more startling evidence suggests that the clinical presentation of vCJD may vary and that patients may present as classical (sporadic) CJD or may have subclinical infection and be apparently healthy. These patients may still pose a risk of iatrogenic transmission through surgical or medical (blood transfusion) procedures. The aim of future work is to develop preclinical screening tests for the identification of infected but still healthy individuals. The future course of vCJD is still uncertain. Modelling studies to predict the cases of vCJD depend on the date of origin of BSE and time of infection, which is, at best, only approximated. As the number of cases of BSE in the UK declines, the risk of BSE in other countries from imported cattle or meat and bone meal from the UK has been increasing. It is also recognized that other animal species (farmed, domestic and wild animals) other than cows are susceptible to TSEs. The possibility of interspecies transmission of TSEs and the global presence of the disease suggests a need for a co-ordinated worldwide risk management approach to eradicate TSEs.
传染性海绵状脑病（TSEs）被认为是由朊病毒引起的，是人和动物的致命性神经退行性疾病。尽管其发病率很低，但人类朊病毒疾病受到了广泛关注，因为食用患有牛海绵状脑病（BSE）的牛的受朊病毒污染的肉被认为是人类变异型克雅氏病（vCJD）出现的原因。目前已有vCJD的临床诊断标准。最近，更令人震惊的证据表明，vCJD的临床表现可能有所不同，患者可能表现为经典（散发性）克雅氏病，也可能有亚临床感染且表面健康。这些患者仍可能通过手术或医疗（输血）程序造成医源性传播风险。未来工作的目标是开发临床前筛查测试，以识别已感染但仍健康的个体。vCJD的未来病程仍不确定。预测vCJD病例的模型研究取决于BSE的起源日期和感染时间，而这充其量也只是大致估算。随着英国BSE病例数量的下降，其他国家因进口英国的牛或肉骨粉而感染BSE的风险一直在增加。人们还认识到，除了牛之外，其他动物物种（养殖动物、家畜和野生动物）也易感染TSEs。TSEs的种间传播可能性以及该疾病在全球的存在表明，需要一种协调一致的全球风险管理方法来根除TSEs。
