Creutzfeldt-Jakob病即亚急性海绵状脑病，又称皮质-纹状体-脊髓变性，是一种罕见病，但在地理上广泛分布。国外文献报告其患病率约为百万分之0.99～1.9。我国尚无发病率或患病率之统计。自1980年报告2例以来，迄今尚不足50例，也广泛分布于十多个省市。近几年我们遇到2例，特 Creutzfeldt-Jakob disease is subacute spongiform encephalopathy, also known as cortex - striatum - spinal degeneration, is a rare disease, but widely distributed geographically. Foreign literature reported a prevalence of about 0.99 to 1.9 parts per million. There is no statistics on morbidity or prevalence in our country. Since the report of 2 cases in 1980, there are still less than 50 cases so far and are widely distributed in more than 10 provinces and cities. In recent years, we have encountered two cases, especially