Creutzfeldt-Jakob病的临床和影像学特点

目的探讨Creutzfeldt-Jakob病(CJD)的临床及影像学特征。方法回顾性分析29例CJD患者的临床资料。结果本组有12例CJD患者以快速进展性痴呆起病;典型的临床表现为进行性痴呆(100%)、共济失调(93.1%)、肌阵挛(89.6%)。EEG均异常,出现典型三相波17例、不典型三相波8例。头颅MRI表现双侧皮质高信号或基底节区T2WI对称性高信号15例,单侧皮质高信号6例,单侧基底节区高信号4例;20例行MR弥散加权成像(DWI)扫描,双侧皮质或基底节区出现高信号14例,一侧皮质或基底节区高信号各3例。14例双侧DWI高信号的患者均出现EEG典型三相波。3例行脑脊液14-3-3蛋白检查,2例阳性。结论 CJD患者多以快速进展性认知功能障碍起病,典型的临床表现为痴呆、共济失调、肌阵挛;EEG、DWI、CSF 14-3-3检测是诊断CJD的重要检查手段;DWI双侧皮质及基底节高信号可能与EEG出现三相波有关。

Clinical and imaging features of Creutzfeldt-Jakob disease

Objective To investigate the clinical and imaging features of Creutzfeldt-Jakob disease(CJD).Methods The clinical data of 29 CJD patients were analyzed retrospectively.Results In this group,12 cases were onset by rapid progressed dementia.The typical clinical manifestation were progressive dementia(100%),incoordination(93.1%),myoclonia(89.6%).EEG was abnormal in all the patients,17 cases appeared typical triphasic wave and 8 cases appeared atypical triphasic wave.On MRI T2WI,the symmetry high signal was observed in bilateral cortex or basal ganglia in 15 cases,in unilateral cortex in 6 cases and in singel basal ganglia in 4 cases.Twenty cases carried out MRI diffusion weighted images(DWI),the high signal in bilateral cortex or basal ganglia was observed in 14 cases,and in unilateral cortex or singal in basal ganglia was 3 cases respectively.Fourteen cases with high signal in bilateral cortex or basal ganglia on MRI DWI appeared the typical triphasic wave on EEG.The level of CSF 14-3-3 protein was detecled in 3 cases,and 2 cases showed positive.Conclusions Most CJD patients onset with rapidly progressive dementia.The typical clinical features are dementia,incoordination and myoclonia.EEG,DWI and CSF 14-3-3 protein test are the major methods of examination for diagnosis of CJD.High signal in bilateral cortex or basal ganglia in DWI may be related to typical triphasic wave on EEG.