散发性克雅氏病12例临床特点分析

发布时间：2018-04-05 16:34

本文选题：克雅氏病　切入点：痴呆　出处：《中国神经精神疾病杂志》2017年07期

【摘要】：目的分析12例可能或很可能散发性克雅氏病(sporadic Creutzfeldt-Jakob disease,sCJD)患者的临床特点及辅助检查结果,以提高对克雅氏病的认识。方法回顾性分析2012年3月至2016年12月至我院神经内科12例疑诊散发性克雅氏病(sCJD)的临床资料。结果 12例患者中亚急性起病11例,慢性起病1例。主要临床特点有进行性痴呆、锥体束征、锥体外系征、小脑症状。磁共振T1WI低信号,T2WI高信号,弥散加权像DWI显示以对称或不对称的沿皮层走行的花边样高信号为特征性磁共振改变,基底节区受累相对较少。脑电图以周期性三相波为主。9例患者行脑脊液14-3-3蛋白检查,其中阳性者7例,阴性者2例。所有患者均于16个月内死亡。结论 sCJD多为亚急性起病,以快速进行性痴呆为特征临床表现,磁共振DWI皮层花边征、脑电图周期性三相波、脑脊液14-3-3蛋白阳性有助于临床医生早期识别和诊断本病。
[Abstract]:Objective to analyze the clinical features and auxiliary examination results of 12 patients with sporadic Creutzfeldt-Jakob disease of Creutzfeldt-Jakob disease (CJDD), so as to improve the understanding of CJD.Methods the clinical data of 12 patients with suspected sporadic CJDD from March 2012 to December 2016 were retrospectively analyzed.Results there were 11 cases of subacute onset and 1 case of chronic onset.The main clinical features are progressive dementia, pyramidal beam sign, extrapyramidal system sign, cerebellar symptoms.Magnetic resonance imaging (T1WI) was characterized by high signal intensity on T _ 2WI and diffusion-weighted imaging (DWI), which was characterized by symmetrical or asymmetric lacy like hyperintensity along the cortex, and the basal ganglia was less involved.Electroencephalogram (EEG) was mainly composed of periodic three-phase wave in 9 patients with cerebrospinal fluid 14-3-3 protein examination, in which 7 cases were positive and 2 cases were negative.All patients died within 16 months.Conclusion sCJD is mostly subacute onset, characterized by rapid progressive dementia. Magnetic resonance (DWI) cortical lace sign, EEG periodic three-phase wave, cerebrospinal fluid 14-3-3 protein positive are helpful for the early identification and diagnosis of the disease.
【作者单位】：郑州大学人民医院;
【分类号】：R742

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