Brown P, Cathala F, Raubertas R F, Gajdusek D C, Castaigne P
Neurology. 1987 Jun;37(6):895-904. doi: 10.1212/wnl.37.6.895.
During the 15-year period 1968-1982, a total of 329 patients dying of Creutzfeldt-Jakob disease (CJD) were identified in continental France. Annual mortality rates stabilized at 0.5 to 0.6 cases per million (1.1 to 1.2 cases per million in Paris). Six percent of cases were familial. Although the frequency of CJD was related to population density, no contacts could be established among the great majority of patients. No association with socioeconomic factors, preceding trauma or surgery (excepting one iatrogenic neurosurgical case), or exposure to animal sources of infection was identified. Evidence from this and other epidemiologic studies suggests that CJD is a minimally contagious disease that may be principally acquired in early life from presymptomatic patients, asymptomatic carriers, or chance contamination by environmental sources. It is possible that CJD could also occur sporadically as a noncontagious disease by a mechanism akin to oncogenes in carcinogenesis.
在1968年至1982年的15年期间，法国本土共确诊329例死于克雅氏病（CJD）的患者。年死亡率稳定在每百万人口0.5至0.6例（巴黎为每百万人口1.1至1.2例）。6%的病例为家族性。尽管克雅氏病的发病率与人口密度有关，但绝大多数患者之间并未发现有接触史。未发现与社会经济因素、既往创伤或手术（除1例医源性神经外科病例外）或接触动物感染源有关。这项研究及其他流行病学研究的证据表明，克雅氏病是一种传染性极低的疾病，可能主要在早年从症状前患者、无症状携带者或环境源的偶然污染中感染。克雅氏病也有可能以类似于致癌过程中癌基因的机制作为一种非传染性疾病散发性发生。
