散发型Creutzfeldt-Jakob病的临床、病理及头颅核磁DWI影像特点研究

Clinical Manifestations,Neuropathology and DWI Characters of Sporadic Creutzfeldt-Jakob Disease

目的探讨散发型Creutzfeldt-Jakob病(sCJD)的临床、病理、头颅核磁影像学特点和内在关系,分析弥散加权成像(DWI)的诊断价值。方法对3例sCJD患者的临床特征、病理改变和头颅核磁DWI异常信号进行分析。结果3例患者DWI病变主要分布于皮层及纹状体。皮层异常信号首先出现与临床先有智能下降符合。DWI对于早期发现特异的皮层和基底节高信号优于传统核磁的T1WI、T2WI及FLAIR技术。DWI异常发现早于典型脑电图改变。在DWI异常信号显著部位所取活检组织病理改变显著。在DWI提示下,早期行脑活检可明显缩短确诊时间。结论头颅核磁DWI技术可早期发现皮层及基底节异常信号,具有无创、快捷优点,有助于sCJD早期诊治。

Objective To investigate the characters and internal relationships of clinical manifestations, neuropathology and diffusion-weighted MRI （DWI） in patients of sporadic Creutzfeldt-Jakob disease （sCJD）. Methods Systemic study was performed on the clinical features, pathological changes and abnormal signals of cranial DWI of 3 sCJD cases. Results DWI abnormal signals were mainly distributed in cortexes and striated bodies. Cortex involvement first of all was consistent with dementia clinically. DWI technique was much better than conventional T1WI, T2WI and FLAIR technique in definding specific cortical and basal ganglionic abnormalities. Typical DWI abnormality appeared earlier than that of electroencephalograghy. Biopsy tissues taken from the dominantly abnormal DWI regions demonstrated typical neuropathological changes. Early cerebral biopsy should prompt the early diagnosis. Conclusions DWI technique was good for the early detection of cortical and striatal changes. It was untraumatic and convenient for the early diagnosis of sCJD.