散发性Creutzfeldt-Jakob病头部磁共振表现

目的 探讨散发性Creutzfeldt-Jakob病(sCJD)头部磁共振的表现及其与临床的关系。方法 对10例sCJD于病后第2—12个月进行头部磁共振扫描，其中脑活检证实6例，14-3-3蛋白检测8例，脑电特异性改变8例及朊蛋白基因分析8例。结果5例双侧尾状核、壳核于T2，加权像或Flair像呈对称性高信号，苍白球与丘脑正常，T1加权像无改变；2例脑萎缩，1例少许脑腔隙梗死，另有2例正常。结论 (1)4例甲硫氨酸纯合型(129Met／Met)底节T2异常信号发现时间平均2．5个月，存活时间平均10．5个月；1例甲硫氨酸杂合型(129Met／Val)底节异常信号发现时间为12个月，存活时间为16个月；(2)底节T2异常信号者平均病程为12．2个月，长于底节无异常信号者(平均5．5个月)；(3)双侧尾核、壳核T2加权像对称性高信号是sCJD重要影像学改变，在特定的临床背景下为sCJD临床诊断依据之一。

The MRI findings of sporadic Creutzfeldt-Jakob disease

Objective To study the MRI findings of sporadic Creutzfeldt-Jakob disease and its clinical relations. Methods MRI of 10 cases CJD patients were examined 2-12 months after the onset. 6 cases were diagnosed using cerebral biopsy, 8 cases received CSF analysis for 14-3-3 protein, 8 cases showed special changes of electroencephalogram, PrP gene of 9 cases were analyzed. Results Symmetric bilateral high signals were observed in caudate nucleus and Putamen in T 2-weighted imaging and Flair imaging in 5 cases, but the pallidum and thalamus were normal. No changes were found in T1-weighted imaging. 2 cases showed brain atrophy, 1 case showed mild lacunar infarction,and the other 2 were normal. Conclusions Abnormal signals in basal ganglia of 4 patients of 129Met/Met homozygote occurred after 2.5 months averagely, they survived for 10.5 months at average. 1 patient of 129 Met/Val heterozygote showed abnormal signals in basal ganglia after 12 months, and survived for 16 months. The mean duration of patient with abnormal signals in basal ganglia (12.2 months) is longer than those without changes in basal ganglia (5.5 months). Symmetric high signal in bilateral caudate nucleus and Putamen is an important imaging feature of sCJD. It might be served as a diagnostic index in some circumstances.