散发型克-雅病的临床特点分析

发布时间：2018-03-20 09:13

本文选题：克雅病　切入点：朊蛋白病　出处：《中国神经精神疾病杂志》2016年11期 　论文类型：期刊论文

【摘要】：目的探讨散发型克-雅病(sporadic Creutzfeldt-Jakob disease,s CJD)的临床特点,以期更好地指导临床医师早期发现该病。方法纳入s CJD患者17例,分析其临床特点、早期症状、辅助检查结果以及生存期等。结果男女比例为1:1.83,平均发病年龄为(60±8.8)岁,首发症状以行走不稳(82.4%)、记忆力减退(64.7%)为主,其中,肌阵挛、磁共振"绸带征"、基底节异常信号、脑电图三相波、脑脊液14-3-3蛋白的阳性率分别为82.4%、76.5%、58.8%、82.4%、70%。随访过程中,12例患者已死亡,平均生存期为(12±7.7)个月。结论本研究中,s CJD患者多数中老年发病,首发症状无特异性,磁共振"绸带征"及脑电图三相波阳性出现的几率较高。
[Abstract]:Objective to investigate the clinical features of sporadic sporadadic Creutzfeldt-Jakob disease (CJDD) in order to better guide clinicians in the early detection of the disease. Methods 17 patients with s CJD were included and their clinical characteristics and early symptoms were analyzed. Results the ratio of male to female was 1: 1.83, the average age of onset was 60 卤8.8 years old. The first symptom was walking instability (82.4%), memory loss (64.7%), myoclonus, magnetic resonance "silk band sign", abnormal signal of basal ganglia. The positive rates of 14-3-3 protein in EEG three-phase wave and cerebrospinal fluid were 82.4 and 76.5, respectively. 12 patients had died during the follow-up, and the average survival time was 12 卤7.7months. Conclusion in this study, the majority of patients with CJD have developed disease, but the initial symptoms are not specific. MRI "silk band sign" and EEG three-phase wave positive probability is higher.
【作者单位】：福建医科大学附属第一医院神经内科;
【基金】：国家临床重点专科建设项目经费资助(编号:2012-GJCZD) 福建省科技计划重点项目(编号:2014Y0022)
【分类号】：R742.9

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