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Creutzfeldt-Jakob病(简称CID),是一种中枢神经系统广泛变性的疾病。由于病变主要累及灰质,故又称“皮质纹状体脊髓变性”;病理改变主要以广泛的海绵状变性为特征,故又称为“亚急性海绵状脑病”。本病较罕见,自1920年Creutzfeld首次报告以来,国外文献仅有200多例报告,多数病例为散发,少数有家族史。国内我们见有3例报道。本例为散发,其临床表现、脑电图及病理变化均有特点,现报告并讨论如下:
Creutzfeldt-Jakob disease (CID), a widely degenerative disease of the central nervous system. As the lesion is mainly involved in gray matter, it is also known as “corticosteroid spinal cord degeneration”; pathological changes are mainly characterized by a wide range of cavernous degeneration, it is also known as “subacute spongiform encephalopathy.” The disease is rare, since Creutzfeld’s first report in 1920, the foreign literature only 200 reports, the majority of cases were distributed, a small number of family history. We have seen three cases in China. This case is disseminated, and its clinical manifestations, EEG and pathological changes are characterized, are now reported and discussed as follows: