散发型克-雅病的临床特点分析

Clinical features of sporadic Creutzfeldt-Jakob disease

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。

Objective We characterized the clinical features of sporadic Creutzfeldt-Jakob disease （sCJD）in order to diagnose it at the early stage. Methods Seventeen patients with sCJD were enrolled in the study. The clinical data, symptoms at the early stage, result of auxiliary examinations and survival time were analyzed. Results The ratio of male to female was 1：1.83 and the average age of onset was 60±8.8 years old. Most of them presented with walking unstable （82.4%） and hypomnesia （64.7%） as the initial symptom. The occurrence rate was 82.4%, 76.5% and 58.8% for myoclonus, colored-ribbon-shaped high signals in cerebral cortex and high signals in basal ganglia of MRI. Periodic synchronous discharge （PSD） of electroencephalography（EEG） was seen in 82.4% cases, while cerebrospinal fluid analysis revealed positive results for 14-3-3 protein in 70% cases. Twelve patients had been dead in our study. The median survival time was 12±7.7 months. Conclusions sCJD is more frequently occurred in mid-aged and older without specific symptoms in early stage and positive rate of high signals in cerebral cortex of MRI and PSD of EEG is high.