Creutzfeldt—Jakob病(CJD)是海绵状脑病的一种。该病极为罕见,据推测发病率为0.09/百万人口,不过这个数字可能估计过低。大多数病例散在发病,但偶有报告整个家庭同时发病。起病通常在40一60岁,男女发病相等。大多数患者显示特征性的临床表现,但少数患者表现不典型,常常呈较慢 Creutzfeldt-Jakob disease (CJD) is a form of spongiform encephalopathy. The disease is extremely rare, with a presumed morbidity rate of 0.09 per million population, although this figure may be underestimated. The majority of cases are scattered, but occasional reports of simultaneous onset of the entire family. The onset is usually 40 to 60 years old, the same incidence of men and women. Most patients show a characteristic clinical manifestation, but a minority of patients show atypical, often slower