散发型Creutzfeldt-Jakob病的研究进展

Progress in Sporadic Creutzfeldt-Jakob Disease

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。

Creutzfeldt-Jakob disease（CJD） is a neurodegenerative disease caused by 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 cerebellar ataxia. Periodic sharp-wave complexes are the typical, electroencephalogram alteration. 14-3-3 protein analysis in the cerebrospinal fluid can be positive. Bilateral symmetric 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.