Scrimgeour E M, Masters C L, Alpers M P, Kaven J, Gajusek D C
J Neurol Sci. 1983 May;59(2):265-75. doi: 10.1016/0022-510x(83)90044-8.
Kuru was diagnosed in a 42-year-old Melanesian male from the Eastern Highlands Province of Papua New Guinea. The clinical features indicated predominant cerebellar degeneration together with widespread cortical neuronal dysfunction and involvement of the diencephalon, hippocampus and basal ganglia. Dementia was an early and prominent feature. The duration of clinical illness was about 12 months and atypically he spent the last 7 months in hospital allowing continuous assessment. At autopsy, spongiform encephalopathy was demonstrated, and inoculation of brain tissue into 4 squirrel monkeys and 1 capuchin monkey resulted in the development of kuru. This is the longest continuous study of kuru in a hospital setting to be recorded in the adult human subject. The virus isolated from the brain of this patient has been adopted as a standard reference strain of kuru for future use and repository.
库鲁病在一名来自巴布亚新几内亚东部高地省的42岁美拉尼西亚男性身上被确诊。临床特征表明主要为小脑变性，同时伴有广泛的皮质神经元功能障碍以及间脑、海马体和基底神经节受累。痴呆是早期突出特征。临床病程约12个月，非典型的是，他在医院度过了最后7个月，得以持续接受评估。尸检显示为海绵状脑病，将脑组织接种到4只松鼠猴和1只卷尾猴体内导致了库鲁病的发生。这是在医院环境中对成年人类受试者进行的关于库鲁病的最长持续研究记录。从该患者大脑中分离出的病毒已被用作未来使用和储存的库鲁病标准参考菌株。
