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Creutzfeldt－Jakob 病（CJD）又称皮质—纹状体—脊髓变性或克雅病，是一种病情严重的可传播脑病，临床表现差异大，早期诊断困难，虽发病率低，但病死率达到百分之百，该文就近年来有关 CJD 的病因、诊断、治疗研究进展作一综述。“,”Creutzfeldt-Jakob disease (CJD) is also known as the cortex striatum spinal cord degeneration or subacute cavernous encephalopathy , and it is a rare form of transmission of encephalopathy , with 100% of mortality and hard way to di-agnosis early.This thesis is a review about lasted study of pathogeny and case reports .