肝豆状核变性的脑部MRI表现(附5例报告)

MRI findings of hepatolenticular degeneration:Analysis of 5 cases

目的评价MRI对诊断肝豆状核变性(HLD)的意义。方法5例由临床神经学与生化学证实的HLD患者均经脑部T1加权和T2加权自旋回波序列磁共振成像(MRI)。1例患者还经Gd-DTPA增强扫描。记录了每个序列形态学特征和MRI信号特征。结果在T2加权像上,所有异常病灶均呈高信号,例如,全部病例两侧豆状核和3例的两侧尾状核均呈对称性分布的高信号,2例的中脑见对称性分布的腔隙性高信号,1例右侧颞叶深部白质内见局限性高信号,而在T1加权像上,所有异常病灶均呈低信号,1例经Gd-DTPA增强扫描后,病灶无强化。脑萎缩改变见于3例,表现为脑沟、脑裂、脑池加宽,及脑室系统扩张。尾状核轻度萎缩见于1例。结论肝豆状核变性的特征性MRI表现是其诊断的重要依据,尤其是T2加权SE序列应推荐为诊断HLD的首选方法。

Objective To assess the significance of MRI in the diagnosis of hepatolenticular degeneration(HLD).Methods Five patients with clinic-neurologically and biochemically proved HLD underwent T1 weigheted and T2 weighted Spin-echo(SE)sequence MRI of brain,of them,one case underwent additional Gd-DTPA enhanced scan.MR signal characteristics and morphologic features were recorded for each sequence.Results On T2 weighted SE images.all the lesions were of hyperintense,for example,bilateral lenticulas in all patients and bilateral caudate nucleus in 3 patients were of symmetric hyperintense.There were symmetric cavitary hyperintense in midbrain of 2 cases and local hyperintense in deep white matter of right temporal lobe in 1 case.respectively.Following Gd-DTPA enhancement scan in 1 case the lesions were of unenhanced.An extensive cortical atrophy and a widening of the venticules were found in 3 cases but mild atrophy of caudate nucleus was found only in 1 case.Conclusion The characteristic MRI findings of HLD are important evidence for the diagnosis of HLD,Particularly T2 WI SE sequence should be prefered as a first choice of diagnostic methods for HLD.