肝豆状核变性脑部磁敏感加权成像表现

Susceptibility weighted imaging features of brain in Wilson’s disease patients

目的观察肝豆状核变性(WD)脑部SWI表现。方法收集20例临床确诊的WD患者(WD组)及20名健康志愿者(正常对照组),对所有研究对象进行SWI扫描,于校正相位图上手动绘制ROI,分别测量双侧尾状核头、苍白球、壳核、红核、黑质、丘脑相位值。比较WD组与正常对照组各核团相位值差异,分析WD组各核团相位值与血清铜和铜蓝蛋白间相关性。结果WD组各核团均见异常低信号,其中尾状核头、苍白球、壳核低信号分布不均匀,多位于核团边缘,红核、黑质、丘脑低信号分布均匀。与正常对照组比较,WD组各核团相位值明显降低,差异均有统计学意义(P<0.05),以壳核差异最为显著(左侧:-0.41±0.25 vs-0.04±0.03,右侧:-0.40±0.26 vs-0.04±0.03)。WD组各核团相位值与血清铜和铜蓝蛋白均无明显相关性(P均>0.05)。结论 WD患者脑内核团存在异常顺磁性物质沉积,可通过SWI进行检测。

Objective To observe the susceptibility-weighted imaging （SWI） features of brain in Wilson＇s disease （WD） patients. Methods Twenty clinically confirmed WD patients （WD group） and 20 healthy volunteers （control group） underwent SWI. On the corrected phase images, the ROI was mannally drawn and the phase values of caudate nucleus, globus pallidus, putamen, red nucleus, substantia nigra and thalamus were measured and compared between the two groups. The correlations between phase value and serum copper and ceruloplasmin were analyzed in WD group. Results Abnormal low signals were observed in all the nucleus, which was unevenly distributed on the edge of the caudate nucleus, globus pallidus and putamen, and evenly distributed in the red nucleus, substantia nigra and thalamus. The phase values of all the nucleus in WD group were significantly lower than those in control group （P〈0.05） and the difference was the largest in the putamen （left： —0.41±0.25 vs —0.04±0.03, right： —0.40±0.26 vs —0.04±0.03）. There was no significant correlations between phase value and serum copper and ceruloplasmin in WD patients （both P〉0.05）. Conclusion There is paramagnetic mineralization deposition in brain gray nuclei in WD patients, which can be detected by SWI.