1例Creutzfeldt-Jakob病的神经影像学与脑电图长期跟踪研究  

Neuroimaging and electroencephalogram features of a patient with Creutzfeldt-Jakob disease: a follow-up study

作　　者：张家堂[1] 蒲传强[1] 郎森阳[1] 贾渭泉[1] 黄旭升[1] 于生元[1] 吴卫平[1] 

机构地区：[1]解放军总医院神经内科,北京100853

出　　处：《解放军医学杂志》2008年第6期743-746,共4页Medical Journal of Chinese People's Liberation Army

摘　　要：目的探讨散发性Creutzfeldt-Jakob病(sCJD)患者神经影像学、脑电图的特点和动态规律性。方法利用CT、MRI以及动态脑电图设备先后对1例病理确诊的sCJD患者行5次神经影像学和6次脑电图监测,并分析其规律性。结果①在辅助诊断CJD方面,弥散加权像(DWI)较头颅CT、常规MRI的T1加权像(T1WI)、T2加权像(T2WI)、FLAIR像以及MRI增强扫描更敏感;②CJD患者DWI表现为双侧底节区及皮层的异常高信号;病程晚期,皮层DWI异常信号可以消失;③CJD患者脑电图周期性尖慢复合波(PSD)的出现有明显的病程区间性及正弦曲线性特征;PSD在病程早期可以不出现,病程晚期可以消失。结论反复监测DWI和脑电图,对于确诊CJD是十分重要的。Objective To explore the characteristics of neuroirnaging and electroencephalogram （EEG） features of sporadic Creutzfeldt-Jakob disease （sCJD）. Methods CT scan was performed at the 6th week, MRI was performed at the 7th, 9th, 17th and 64th week, regular EEG recording was performed at the 9th week, contineous 24-hour EEG recording was performed at the llth, 14th, 16th, 37th and 64th week after onset in a patient with the pathological diagnosis of CJD. Regular pattern was analyzed according to the neuroimaging results and EEG. Results a） Diffusion-weighted imaging （DWI） was more sensitive in diagnosis of sCJD than cranial CT, T1- weighted MRI （T1 WI）, T2-weighted MRI （T2 WI）, fiNd-attenuated inversion recovery （FLAIR） MRI or contrast-enhanced MRI. Abnor malities would not be found in the early stage of CJD in CT, T1 weighted MRI, T2-weighted MRI, FLAIR or contrast-enhanced MRI, but they could be found in the middle stage of CJD. The CT scan and routine MRI might not show any specific feature for the sCJD. b） DWI abnormalities appeared in bilateral cortex and basal ganglia, and changed along with the progression of disease. In late stage of the disease, abnormal aignals in the cortex would disappear, but it was still present in basal ganglia, c） The appearance of periodic discharge of sharp wave complexes （PSD）, which could be mathematically described by a sine curve, might be absent in the early and late stage of the dis ease. Along with the progression of CJD, PSD would be present in a part of a lobe at first and then involve more lobes, and finally the whole brain would be involved. Conclusion Repeated monitoring of DWI and EEG is significant for early diagnosis of CJD.

关 键 词：CREUTZFELDT-JAKOB病 磁共振弥散加权像 脑电图 

分 类 号：R741.044[医药卫生—神经病学与精神病学] R742[医药卫生—临床医学]