【摘要】目的：分析肝豆状核变性的临床表现与脑CT征象。方法：回顾分析36例肝豆状核变性患者临床资料及CT检查资料。结果：病变分布于豆状核13例(36.11%)，丘脑内囊后肢7例(19.44%)，苍白球6例(16.67%)，尾状核头部6例(16.67%)，豆状核尾状核联合病变2例(5.56%)，丘脑内囊后肢苍白球联合病变2例(5.56%)。脑萎缩30例(83.33%)，脑室扩大28例(77.78%)。脑基底节区见双侧对称性低密度影24例(66.67%)。脑白质脱髓鞘6例(16.67%)，脑干低密度影3例(8.33%)，小脑齿状核低密度影3例(8.33%)。结论：CT诊断肝豆状核变性均有显著的阳性征象，有助于临床早期诊断。

    【关键词】肝豆状核变性；CT；诊断

    Analysis of clinical manifestations and brain CT of hepatolenticular degeneration

    LI Zhanjun,LI Kunwei ,QIN Peixin

    【Abstract】Objective To analyse clinical manifestations and brain CT of hepatolenticular degeneration . Method Retrospectively analysed the clinical date CT sign of 36 cases with hepatolenticular degeneration. Results Lesions of 13cases(36.11%)were in beans shape distribution nuclear, 7cases(19.44%)were in internal capsule thalamus hind legs, 6 cases(16.67%)were in globus pallidus, 6 cases(16.67%)were in head of caudate nucleus, 2 cases(5.56%)were in bean shape nuclear joint caudate nucleus, 2 cases(5.56%)were in globus pallidus joint capsule thalamus hind legs. 30 cases(83.33%)were encephalatrophy, 28 cases(77.78%)were ventriculomegaly, brain basal ganglia of 24 cases(66.67%)saw bilateral symmetry low density, 6 cases(16.67%)were brain demyelinated white matter, 3 cases(8.33%)were brainstem low density, 3 cases(8.33%)were cerebellum dentate nuclear low density. Conclusion CT diagnosis of liver degeneration showed a significant positive signs, contribute to early clinical diagnosis. ......