Deptartment of Molecular Pathology and Neuropathology, Medical University of Lodz, Poland.
J Neuropathol Exp Neurol. 2012 Feb;71(2):92-103. doi: 10.1097/NEN.0b013e3182444efd.
Kuru was the first human transmissible spongiform encephalopathy (TSE) or prion disease identified, occurring in the Fore linguistic group of Papua New Guinea. Kuru was a uniformly fatal cerebellar ataxic syndrome, usually followed by choreiform and athetoid movements. Kuru imposed a strong balancing selection on the Fore population, with individuals homozygous for the 129 Met allele of the gene (PRNP) encoding for prion protein (PrP) being the most susceptible. The decline in the incidence of kuru in the Fore has been attributed to the exhaustion of the susceptible genotype and ultimately by discontinuation of exposure via cannibalism. Neuropathologically, kuru-affected brains were characterized by widespread degeneration of neurons, astroglial and microglial proliferation, and the presence of amyloid plaques. These early findings have been confirmed and extended by recent immunohistochemical studies for the detection of the TSE-specific PrP (PrP). Confocal laser microscopy also showed the concentration of glial fibrillary acidic protein-positive astrocytic processes at the plaque periphery. The fine structure of plaques corresponds to that described earlier by light microscopy. The successful experimental transmission of kuru led to the awareness of its similarity to Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease and formed a background against which the recent epidemics of iatrogenic and variant Creutzfeldt-Jakob disease could be studied.
库鲁病是首例被发现的人类传染性海绵状脑病（TSE）或朊病毒病，发生于巴布亚新几内亚的福雷族语言群体中。库鲁病是一种均匀致命的小脑共济失调综合征，通常随后出现舞蹈病和手足徐动症运动。库鲁病对福雷人群施加了强烈的平衡选择，个体携带朊病毒蛋白（PrP）编码基因（PRNP）中 129 位蛋氨酸等位基因（Met）的纯合子，最易受感染。福雷人群中库鲁病发病率的下降归因于易感基因型的耗尽，最终通过停止食人行为而终止感染。神经病理学上，受库鲁病影响的大脑表现为神经元广泛退化、星形胶质细胞和小胶质细胞增生，以及淀粉样斑块的存在。这些早期发现已被最近用于检测 TSE 特异性 PrP（PrP）的免疫组织化学研究证实和扩展。共聚焦激光显微镜还显示了斑块周边区胶质纤维酸性蛋白阳性星形胶质细胞过程的浓度。斑块的精细结构与早期通过光学显微镜描述的结构相对应。库鲁病的成功实验传播导致人们意识到其与克雅氏病和格斯特曼-施塔雷克病的相似性，并为研究最近发生的医源性和变异型克雅氏病流行提供了背景。
