Clinical features of sporadic Creutzfeldt-Jakob disease

VernacularTitle:散发型克-雅病的临床特点分析
Author: Xuan WU ; Aiyu LIN ; Zhiwen LI ; Zhiqiang WANG ; Ning WANG
Keywords: Creutzfeldt-Jakob disease; prion desease; Clinical features
From: Chinese Journal of Nervous and Mental Diseases 2016;42(11):676-679
CountryChina
Language:Chinese
Abstract: Objective We characterized the clinical features of sporadic Creutzfeldt-Jakob disease(sCJD)in or?der 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 myoclo?nus, colored-ribbon-shaped high signals in cerebral cortex and high signals in basal ganglia of MRI. Periodic synchro?nous discharge (PSD) of electroencephalography(EEG) was seen in 82.4% cases, while cerebrospinal fluid analysis re?vealed positive results for 14-3-3 protein in 70%cases. Twelve patients had been dead in our study. The median surviv?al time was 12±7.7 months. Conclusions sCJD is more frequently occurred in mid-aged and older without specific symp?toms in early stage and positive rate of high signals in cerebral cortex of MRI and PSD of EEG is high.