报道了２例ＣＪＤ，均以急性记忆障碍、进行性痴呆起病，病程进展迅速，分别于起病后２７天及３个月死亡。临床表现肌阵挛、锥体外系统体征及去皮质状态，脑活检电镜示神经元肿胀，核浓缩，并见胞浆中大小不一的空泡，髓内轴索肿胀及肿胀变性的线粒体和内质网，胶质细胞轻度增生，未见炎性反应与包涵体。结合文献就其临床、电镜进行讨论。 Two cases of CJD were reported, both of which had acute memory impairment and onset of progressive dementia. The course of disease was rapid and died at 27 days and 3 months respectively after onset. Clinical manifestations Myoclonus, extrapyramidal signs and signs of cortical state, brain biopsy electron microscopy showed neuronal swelling, nuclear concentration, and see the cytoplasm of different sizes of vacuoles, intramedullary axonal swelling and swelling of the mitochondria and the degeneration The cytoplasm, glial cells slightly hyperplasia, no inflammatory reaction and inclusion body. Combined with the literature on its clinical, electron microscope to discuss.