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目的探讨肝豆状核变性的脑MRI特征。方法分析归纳16例肝豆状核变性患者脑MRI图像,并与其临床表现进行对照研究。结果基本特征是对称性基底节区和脑干长T1长T2信号,铜沉积多者出现短T2信号,病程长者可见脑萎缩,尤其后颅窝结构萎缩。病灶部位与神经系统表现有较好的一致性。结论脑MRI是肝豆状核变性诊断、鉴别诊断和疗效评价的一项重要手段。
Objective To investigate the brain MRI features of hepatolenticular degeneration. Methods The brain MRI images of 16 patients with hepatolenticular degeneration were analyzed and compared with the clinical manifestations. Results The basic characteristics of the symmetrical basal ganglia and brain stem long T1 long T2 signal, copper deposition more short T2 signal appears, the longer course of disease visible brain atrophy, especially after atrophy of the posterior fossa structure. Lesions and nervous system performance has good consistency. Conclusion Brain MRI is an important method for the diagnosis, differential diagnosis and evaluation of hepatolenticular degeneration.