【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。更多还原

【Abstract】 ObjectiveWe characterized the clinical features of sporadic Creutzfeldt-Jakob disease(s CJD)in order to diagnose it at the early stage.MethodsSeventeen patients with s CJD were enrolled in the study. The clinical data,symptoms at the early stage, result of auxiliary examinations and survival time were analyzed.ResultsThe 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.Conclusionss CJD 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.更多还原