肝豆状核变性合并中央桥脑髓鞘溶解症的MRI表现

MRI manifestation of Wilson's disease accompanied with central pontine myelinolysis

目的 探讨肝豆状核变性合并中央桥脑髓鞘溶解症 (CPM)的MRI表现及弥散加权成像(DWI)的应用价值。方法 采用 1 5TMR仪对 2例肝豆状核变性合并CPM患者进行T1WI、T2 WI、液体衰减反转恢复成像 (FLAIR)、DWI和增强扫描MR检查。结果 桥脑中央部、双侧豆状核、尾状核、丘脑等部位出现对称性长T1、T2 信号 ,增强MRI无明显强化反应。DWI显示桥脑病变呈明显高信号 ,表观弥散系数 (ADC)值在 10 0× 10 -6mm2 /s～ 30 0× 10 -6mm2 /s之间 ,FLAIR仅显示为略微高信号 ,表明病变伴有明显的细胞毒性水肿 ;同时 ,双侧肝豆状核亦表现出DWI高信号 ,ADC值在 30 0× 10 -6mm2 /s～ 6 0 0× 10 -6mm2 /s之间 ,FLAIR呈不均匀较高信号 ,其中 1例尚出现尾状核头对称性DWI略低信号 ,表明在缓慢的病程进展中同时伴有细胞毒性水肿和血管源性水肿等改变。结论 肝豆状核变性和CPM具有较为特征的MRI表现 ,DWI对其具有较大的诊断和鉴别诊断价值。

Objective To discuss MRI manifestation of Wilson′s disease accompanied with central pontine myelinolysis (CPM),and to especially detect the value of diffusion-weighted MRI (DWI) in this disease. Methods Two cases of Wilson′s disease accompanied with CPM were performed with sequent MR examination including T_1WI,T_2WI,FLAIR,DWI,and contrast-enhanced MRI (CE MRI) by a 1.5 T magnetic resonance system (Philips gyroscan master).Results Symmetric long T_1 and T_2 signals were found in central pontine region,lenticular nucleus,caudate nucleus,and thalamus.CE MRI showed no apparent enhanced lesions.DWI showed obviously high signal intensities in central pontine region with apparent diffusion coefficient values fluctuated between (100～300)×10 -6 mm 2/s while FLAIR images just showed slightly high signal in the same area,which suggested that cytotoxic edema did in fact exist in CPM.Meanwhile,DWI also showed high signals in bilateral lenticular nucleus with apparent diffusion coefficient values fluctuated between (300～600)×10 -6 mm 2/s ,however,FLAIR images showed heterogeneous high signals and one case presented low DWI signals in bilateral head of caudate nucleus,all these signs might be explained by mixed-changes of cytotoxic edema and vasogenic edema in long period of Wilson′s disease.Conclusion Wilson′s disease and CPM have characteristic MRI manifestation and DWI may be a very useful way to confirm a correct diagnosis.