散发性Creutzfeldt-Jakob病的头颅MRI特点与鉴别诊断

发布时间：2018-09-17 09:25

【摘要】：目的总结散发型克-雅氏病(Sporadic Creutzfeldt-Jakob Disease,s CJD)的头颅MR特点及鉴别诊断要点。方法搜集我院自2009年1月至2014年6月诊断的很可能的s CJD 8例患者的临床及头颅MRI资料,回顾性分析其头颅MRI特点,随访其诊治经过和疾病转归,探讨其鉴别诊断要点。结果 8例患者均以迅速进展的痴呆和精神行为异常为主要临床表现;8例患者常规脑电图均异常,其中5例可见周期性三相波,首次头颅MR检查均表现为大脑皮质受累为主的对称或不对称性花边裙样DWI高信号,其中5例伴尾状核和(或)壳核受累,病灶在头颅MR-DWI病灶出现最早,表现为"彩带样"高信号;其次是FLAIR表现为高信号;病灶在T2WI上为高信号、T1WI上为低信号。8例患者中有5例患者经历误诊,其中误诊为脑梗死2例,误诊为病毒性脑炎、阿尔兹海默病、眩晕症、皮质基底节变性各1例。结论 s CJD患者头颅MRI表现具有一定的特征性。DWI反应病变最敏感,可能有助于本病的早期诊断。s CJD需要与缺血性脑血管病、脑炎和其他进展性痴呆鉴别。
[Abstract]:Objective to summarize the cranial MR features and differential diagnosis of sporadic Creutzfeldt-Jakob disease (Sporadic Creutzfeldt-Jakob Disease,s CJD). Methods from January 2009 to June 2014, the clinical and cranial MRI data of 8 patients with s CJD diagnosed in our hospital from January 2009 to June 2014 were collected. The characteristics of head MRI were analyzed retrospectively. The diagnosis and treatment and the outcome of the disease were followed up, and the main points of differential diagnosis were discussed. Results the main clinical manifestations of 8 cases were rapid progression of dementia and abnormal mental behavior. The routine EEG of 8 cases were abnormal. Among them, 5 cases showed periodic three-phase waves. All of the first cranial MR showed high signal intensity of symmetric or asymmetric lace skirt DWI, which was mainly involved in cerebral cortex. In 5 cases, caudate nucleus and / or putamen nucleus were involved, and the lesions appeared first in the lesions of MR-DWI. Among the 8 patients with high signal intensity on T2WI, 5 cases were misdiagnosed as cerebral infarction, 2 cases were misdiagnosed as viral encephalitis, 2 cases were misdiagnosed as cerebral infarction, 2 cases were misdiagnosed as cerebral infarction, and 2 cases were misdiagnosed as viral encephalitis. Alzheimer's disease, vertigo, cortical basal ganglia degeneration in 1 case. Conclusion the cranial MRI features of the patients with s CJD are the most sensitive, which may be helpful to the early diagnosis of the disease. S CJD should be distinguished from ischemic cerebrovascular disease, encephalitis and other progressive dementia.
【作者单位】：徐州医学院附属医院神经内科;
【分类号】：R445.2;R742.9

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