Schulz-Schaeffer W J, Fatzer R, Vandevelde M, Kretzschmar H A
Institute of Neuropathology, Georg-August University, Göttingen, Germany.
Arch Virol Suppl. 2000(16):173-80. doi: 10.1007/978-3-7091-6308-5_16.
The appearance of a new variant of CJD (vCJD) in young patients has caused considerable public concern and there is evidence that this novel disease is caused by the same agent as BSE. BSE is a prion disease that became epidemic in the UK, with a peak incidence in January 1993. New test systems should aim to identify BSE-infected cattle early in the incubation period. We compared the established histological and immunohistochemical methods and the Western blot method used by Prionics with the PET blot method that detects prion PrP(Sc) deposits in formalin-fixed and paraffin-embedded tissue. Investigating the obex region with the PET blot, all BSE cases were detectable and no false positive cases occurred. From the Swiss culling program, five clinically healthy cattle out of 1761 were identified as incubating BSE. With the PET blot method four of them showed the same PrP(Sc) deposition pattern that was seen in clinical BSE, though less conspicuous. In one of the five cases, PrP(Sc) was restricted to two brain stem nuclei, a pattern that was reported to be the first manifestation of PrP(Sc) deposits in the brain after peripheral infection and one that occurs after half of the incubation time. In this case, histology and Western blot were negative.
新型克雅氏病（vCJD）在年轻患者中出现引起了公众的广泛关注，且有证据表明这种新型疾病与牛海绵状脑病（BSE）由同一病原体引起。BSE是一种朊病毒疾病，曾在英国流行，1993年1月发病率达到峰值。新的检测系统应旨在在潜伏期早期识别出感染BSE的牛。我们将已确立的组织学和免疫组化方法以及Prionics公司使用的蛋白质印迹法与能检测福尔马林固定石蜡包埋组织中朊病毒PrP(Sc)沉积物的PET印迹法进行了比较。用PET印迹法研究延髓区时，所有BSE病例均可检测到，且未出现假阳性病例。在瑞士的扑杀计划中，1761头临床健康的牛中有5头被确定为处于BSE潜伏期。用PET印迹法检测，其中4头呈现出与临床BSE相同的PrP(Sc)沉积模式，不过不太明显。在这5例中的1例中，PrP(Sc)局限于两个脑干核团，据报道这种模式是外周感染后大脑中PrP(Sc)沉积的首次表现，且在潜伏期一半时间后出现。在该病例中，组织学检查和蛋白质印迹法结果均为阴性。
