Buge A, Escourolle R, Brion S, Rancurel G, Hauw J J, Mehaut M, Gray F, Gajdusek D C
Rev Neurol (Paris). 1978 Mar;134(3):165-81.
We report here a family with three pathologically confirmed cases of Creutzfeldt-Jakob disease in three siblings and with transmission of one case to the squirrel monkey. This family, originating from the South-East of England, settled in France in 1870. Eight members of the family, distributed in three generations, were affected by the disease at the same age (about 50), with similar symptoms and length of evolution. Two cases were confirmed at post-mortem and one case by cerebral biopsy. The genealogic inquiry dealth with 46 members of 5 generations. All the affected members, six men and two women, belonged to the three older generations which were comprised of 15 individuals. All had lived in close relationship in a limited area of the north of Ardennas. The possible mechanisms of dominant inheritance or cross infection are discussed.
我们在此报告一个家族，该家族中有三例经病理确诊为克雅氏病的病例，患者为三兄妹，其中一例还传染给了松鼠猴。这个家族来自英格兰东南部，于1870年定居法国。家族中的八名成员，分布在三代人中，在相同年龄（约50岁）患上这种疾病，症状相似，病程长度相近。两例经尸检确诊，一例经脑活检确诊。家谱调查涉及五代中的46名成员。所有患病成员，六名男性和两名女性，都属于由15人组成的前三代。他们都在阿登纳斯北部的一个有限区域内密切生活。文中讨论了显性遗传或交叉感染的可能机制。
