Ironside J W
CJD Surveillance Unit, University of Edinburgh, Scotland, UK.
Arch Virol Suppl. 2000(16):143-51. doi: 10.1007/978-3-7091-6308-5_13.
Variant Creutzfeldt-Jakob disease (vCJD) is a novel prion disease in man which was first described in 1996 in the UK. There is substantial evidence to indicate that vCJD represents the effects of the bovine spongiform encephalopathy (BSE) agent in man. The neuropathology of vCJD is characterised by the florid plaque, composed of a central amyloid core with a fibrillary periphery, surrounded by a rim of spongiform change in an intact neuropil. Unique patterns of PrP accumulation in vCJD are revealed by immunocytochemistry in the cerebral and cerebellar cortices, the basal ganglia, thalamus and brainstem. The neuropathology of the thalamus and midbrain is also characterised by severe neuronal loss and gliosis. vCJD is distinct from other human prion diseases in that disease-associated PrP accumulates within follicular dendritic cells in lymphoid tissue, and consistently in peripheral sensory ganglia. All vCJD patients so far have been methionine homozygotes at codon 129 in the PrP gene. There is no evidence to indicate that cases of BSE infection have occurred in individuals in the UK who are MV or VV at codon 129 in the PrP gene. It is conceivable that BSE incubation periods in these groups may be longer than in methionine homozygotes, hence the precise numbers of future cases of vCJD are difficult to estimate at present.
变异型克雅氏病（vCJD）是一种人类新型朊病毒病，于1996年在英国首次被描述。有大量证据表明，vCJD是牛海绵状脑病（BSE）病原体在人类身上产生的影响。vCJD的神经病理学特征是出现特征性斑块，由中央淀粉样核心和纤维状周边组成，周围是完整神经纤维网中的海绵状改变边缘。免疫细胞化学显示，vCJD在大脑和小脑皮质、基底神经节、丘脑和脑干中PrP积累具有独特模式。丘脑和中脑的神经病理学特征还包括严重的神经元丧失和胶质细胞增生。vCJD与其他人类朊病毒病不同，在于疾病相关的PrP在淋巴组织的滤泡树突状细胞内积累，并始终存在于外周感觉神经节中。迄今为止，所有vCJD患者在PrP基因第129密码子处均为甲硫氨酸纯合子。没有证据表明在英国PrP基因第129密码子处为MV或VV的个体中发生过BSE感染病例。可以想象，这些群体中BSE的潜伏期可能比甲硫氨酸纯合子更长，因此目前难以准确估计未来vCJD病例的具体数量。
