Galvez S, Masters C, Gajdusek C
Arch Neurol. 1980 Jan;37(1):11-4. doi: 10.1001/archneur.1980.00500500041004.
Descriptive epidemiological data are presented from a survey of 35 cases of Creutzfeldt-Jakob disease (CJD) that occurred in Chile in the period 1955 to 1977. The average mortality in Chile (0.31 deaths per 1 million) and in urban Santiago (0.73 deaths per 1 million) is compared with data reported from other countries. An increasing incidence in recent years is probably related to a greater awareness of the disease. The familial occurrence of CJD in nine patients from five affected families is described, and its analysis suggests a genetically determined susceptibility with incubation periods of more than 30 years. Horizontal transmission of the disease may have occurred in one patient in whom the disease developed 13 years after the patient married into a family with seven other affected members.
本文呈现了1955年至1977年期间在智利发生的35例克雅氏病（CJD）的描述性流行病学数据。将智利的平均死亡率（每100万人中有0.31人死亡）和圣地亚哥市的平均死亡率（每100万人中有0.73人死亡）与其他国家报告的数据进行了比较。近年来发病率的上升可能与对该疾病的认识提高有关。描述了来自五个受影响家庭的九名患者中CJD的家族性发病情况，对其分析表明存在由基因决定的易感性，潜伏期超过30年。在一名患者中可能发生了该疾病的水平传播，该患者在嫁入一个有其他七名受影响成员的家庭13年后患上了这种疾病。
