散发性Creutzfeldt-Jakob病的头颅MRI特点与鉴别诊断

The brain MR features and differential diagnosis of sporadic Creutzfeldt-Jakob Disease

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。

Objective To summarize brain magnetic resonance imaging（MRI） features and differential diagnosis of sporadic Creutzfeldt-Jakob disease.Methods The clinic data of 8 probable Creutzfeldt-Jakob disease cases in our hospital were analyzed retrospectively from January 2009 to June 2014. We mainly analysed the characteristics of brain MR and the causes of misdiagnosis. We had followed up the family members of these patients on the progress and prognosis of disease. We also analyzed the differential diagnosis of s CJD.Results All the patients presented with rapid progressive dementia and mental abnormal behavior as the main clinical manifestations. All the patients had abnormal routine EEG and five of them had a periodical sharp wave complexes. The brain MRI of the 8 patients showed high signal intensities in cerebral cortex（and/or basal ganglia） on diffusion weighted images（DWI） and 5 of them had caudate nucleus and（or） putamen involvement. The lesions were first appeared in DWI imaging as＂ribbon-like＂high signal, followed by Flair as high signal intensities. lesions were low signal intensities on T1 WI and high signal intensities on T2 WI. Five patients were misdiagnosed. 2 cases were misdiagnosed as having cerebral infarction,1 case was misdiagnosed as having viral encephalitis, 1 case was misdiagnosed as having Alzheimer＇s disease, 1 case was misdiagnosed as having vertigo and1 case was misdiagnosed as having corticobasal degeneration.Conclusion sThe brain MRI of the s CJD patients showed a certain characteristic. DWI is the most sensitive tool in the detection of lesions which is useful in the early diagnosis of this disease. We should distinguish s CJD form ischemic cerebrovascular disease, encephalitis, and other progressive de-mentia identification.