克-雅氏病,亦称亚急性海绵状脑病(Subacute Spongi- form Encephalopathy,SSE)或称为皮质-纹状体-脊髓变(cor- ticol-Striatd-Spinal degeneration)。早在1920年和1921年, Creutzfeldtt和Jakob先后描述了一种发生在老年人的累及皮质、基底节和脊髓的进展性疾病。表现为智能进行性减退,伴有小脑、锥体系和锥体外系受损的症状和体征,病变涉及到大脑皮质、丘脑、小脑、脑干和脊髓。是第二个被认识的人朊病毒病(Prion disease),本病预后极差,最终必然死亡。临床确诊需具有典型／标准的神经病理学改变,和／或免疫细胞化学和／或Western印迹法确定存在蛋白酶耐受性PrP。近年来国内 Creutzfeldt-Jakob disease, also known as Subacute Spongi-form Encephalopathy (SSE) or so-called cortical-striatal-spinal cord degeneration. As early as 1920 and 1921, Creutzfeldtt and Jakob successively described a progressive disease that occurs in the elderly involving the cortex, the basal ganglia and the spinal cord. The performance of the intellectual decline, accompanied by cerebellar, pyramidal and extrapyramidal system damage symptoms and signs, lesions involving the cerebral cortex, thalamus, cerebellum, brain stem and spinal cord. Is the second known human prion disease (Prion disease), the prognosis of this disease is extremely poor, eventually inevitable death. Clinical diagnosis requires typical / standard neuropathological changes, and / or immunocytochemistry and / or Western blotting to determine the presence of protease-resistant PrP. In recent years, domestic