散发型克雅病头颅磁共振影像异常分析

Analysis of brain MRI abnormalities in patients with sporadic Creutzfeldt Jakob disease

目的总结散发型克雅病(sCJD)患者头颅磁共振(MRI)特征,探讨MRI对sCJD诊断的意义。方法对25例sCJD患者的头颅MRI、临床资料、遗传学资料进行总结,主要分析sCJD患者头颅MRI上异常信号在不同序列上的显示情况、受累部位分布及其他指标。结果本组25例sCJD患者中,头颅MRI异常者24例,阳性率96%。异常信号在MRI不同序列上的阳性率DWI高信号者占96%,FLAIR高信号者占56%,ADC低信号者占80%。受累部位、皮层受累者占52%,皮层和基底节同时受累者占48%。皮层肥大征的阳性率为28%。另有1例病人丘脑受累,为国内外罕见。结论头颅MRI异常在sCJD诊断中具有重要价值,以DWI序列最为敏感,病灶模式以单独皮层和皮层/基底节同时受累为主。中国sCJD病人中皮层肥大征和丘脑受累情况,值得进一步观察研究。

Objective To summarize the brain magnetic resonance imaging(MRI)abnormalities in patients with sporadic Creutzfeldt Jakob disease(sCJD)and to explore the significance of MRI in the diagnosis of sCJD.Methods The brain MRI,clinical data,genetic data of 25 sCJD patients were analyzed.The positive rate of abnormal signals in different MRI sequences and the distribution of involved lesions were analyzed.Results Among the 25 patients with sCJD,24 presented brain MRI abnormalities,with the positive rate of 96%.The positive rates of abnormal signals on different MRI sequences were 96%on DWI,56%on Flair and 80%on ADC.52%of the patients showed cortical lesions alone while 48%of the patients showed both cortex and basal ganglia involvement.The positive rate of cortical hypertrophy was 28%.One patient presented the rare thalamus involvement.Conclusion This study suggests that MRI is of great value in the diagnosis of sCJD,and DWI sequence is most sensitive.The distribution pattern of lesions was mainly cortical involvement alone and cortex plus basal ganglia involvement.In Chinese patients with sCJD,cortical hypertrophy and thalamus involvement warrant further investigations.