1980年以来。国内陆续报导9例皮质—纹状体—脊髓变性、又称Creutzfeldt—Jakob病。本文结合我院1988年经尸检证实的1例、重点讨论我国有关本病的若干问题。病例报告患者,男,48岁,因进行性智能减退、抽搐2个月入院。二个月前患者开始出现精神淡漠、行为异常、记忆障碍、逐渐智能减退、肢体无力等。患病一个月后语言不清、视物模糊伴有肢体肌阵挛抽搐、震颤和不自主运动、痴呆加重。入院检查;体温38℃、脉博80次/分、呼吸20次/分、BP16/10kPa、浅昏迷、双侧瞳孔等大同圆、对光反射良好,眼球活动自如,右侧中枢性面瘫、右侧上下肢肌力减弱、四肢肌张力增高、腱反射亢进、巴彬斯氏征(+)。脑脊液蛋白含量0.75g/L,余正常。CT扫描示对称性脑室扩大,脑沟 Since 1980. Nine cases of cortical - striatum - spinal cord degeneration were reported in China, also known as Creutzfeldt-Jakob disease. This article combined with our hospital in 1988 confirmed by autopsy in 1 case, focusing on discussing some of the problems of this disease in our country. Case report patients, male, 48 years old, admitted to hospital for convulsions for 2 months due to progressive degeneracy. Two months ago, patients began to appear apathetic, abnormal behavior, memory impairment, progressive intellectual decline, limb weakness and so on. A month after the illness ill-defined language, blurred vision with limb myoclonus convulsions, tremors and involuntary movements, dementia worsened. Admission examination; body temperature 38 ℃, pulse Bo 80 / min, breathing 20 beats / min, BP16 / 10kPa, shallow coma, bilateral pupil and other Datong Yuan, good light reflexes, eye movement freely, right central paralysis, right Lower extremity muscle weakness, limb muscle tension increased, tendon hyperreflexia, Babbitt’s sign (+). Cerebrospinal fluid protein content 0.75g / L, more than normal. CT scan showed symmetrical ventricular enlargement, sulci