散发型Creutzfeldt-Jakob病的研究进展

摘要

Creutzfeldt-Jakoh disease( CJD ) is a neurodegenerative disease caused hy variant prion protein, and sporadic CJD( sCJD )is the most common type of CJD. Spongiform degeneration is the dominant neuropathological change of sCJD . with the typical manifestations of rapidly progressive dementia , myoclonus ,visual disturbances and cerehellar ataxia. Periodic sharp-wave complexes are the typical electroencephalogram alteration. 14 -3 -3 protein analysis in the cerebrospinal fluid can be positive. Bilateral syrmmetric abnormal signal in the basal ganglia and/or cerebral cortex has been described on magnetic resonance imaging. sCJD is still lack of specific treatment with a poor prognosis.%Creutzfeldt-Jakob病(CJD)是由变异朊蛋白引起的神经变性病,散发型CJD(sCJD)是CJD最常见的类型.sCJD主要病理改变为脑海绵状变性,典型表现为进行性痴呆、肌阵挛、视觉症状、小脑性共济失调等,典型脑电图改变为周期性尖慢综合波.脑脊液中14-3-3蛋白可阳性,磁共振成像可见基底节和(或)大脑皮质双侧对称性异常信号,预后差,目前仍缺乏特异治疗.