Ironside J W
CJD Surveillance Unit, Western General Hospital, Edinburgh, UK.
FEMS Immunol Med Microbiol. 1998 Jun;21(2):91-5. doi: 10.1111/j.1574-695X.1998.tb01153.x.
The diagnosis of new variant Creutzfeldt-Jakob disease is dependent on the neuropathological examination of brain tissue following brain biopsy or autopsy. The characteristic neuropathological features are multiple 'florid' plaques in the cerebral and cerebellar cortex, spongiform change most marked in the basal ganglia, severe thalamic gliosis and marked accumulation of the disease-associated prion protein in diffuse or pericellular deposits in the cerebrum and cerebellum. These features allow distinction from cases of sporadic, familial and iatrogenic Creutzfeldt-Jakob disease in neuropathological terms, new variant Creutzfeldt-Jakob disease also differs from sporadic Creutzfeldt-Jakob disease in terms of prion protein accumulation in lymphoid tissue outside the central nervous system. This has given rise to the possibility that prion protein in new variant Creutzfeldt-Jakob disease might be transported to the brain by circulating lymphocytes in the blood. Experimental strain typing of new variant Creutzfeldt-Jakob disease has shown that the transmissible agent responsible for this disorder is identical to that identified in bovine spongiform encephalopathy, confirming the hypothesis that exposure to the bovine spongiform encephalopathy agent, presumably through the diet, is the cause of new variant Creutzfeldt-Jakob disease.
新型变异型克雅氏病的诊断依赖于脑活检或尸检后对脑组织进行的神经病理学检查。其典型的神经病理学特征包括大脑和小脑皮质出现多个“显著”斑块、基底神经节海绵状改变最为明显、丘脑严重胶质增生以及大脑和小脑弥漫性或细胞周围沉积物中疾病相关朊蛋白的显著积聚。从神经病理学角度来看，这些特征有助于将其与散发性、家族性和医源性克雅氏病区分开来。新型变异型克雅氏病在中枢神经系统外淋巴组织中的朊蛋白积聚方面也与散发性克雅氏病不同。这就引发了一种可能性，即新型变异型克雅氏病中的朊蛋白可能通过血液中循环的淋巴细胞被转运至大脑。新型变异型克雅氏病的实验菌株分型表明，导致这种疾病的可传播病原体与在牛海绵状脑病中鉴定出的病原体相同，证实了如下假设：接触牛海绵状脑病病原体（推测是通过饮食接触）是新型变异型克雅氏病的病因。
