肝豆状核变性14例临床与脑CT分析

The analysis of clinic and encephalon CT in 14 patients with hepatolenticular degeneration

目的 分析肝豆状核变性的临床症状特点和CT表现特征以及二者间的关系。方法观察14例肝豆状核变性病人的临床表现,查相关血生化指标,行脑CT扫描。结果 9例以神经系统症状首发,1例以肝损害症状首发,1例以肝损害伴精神症状首发,1例以关节肿痛首发,2例无症状。14例血清铜检查均提示代谢障碍。CT见11例双侧基底节低密度灶,2例脑萎缩,1例阴性。结论 肝豆状核变性多以神经系统症状首发,且脑CT提示的损害部位与临床症状相符。

Objective To analyse the symptom characters and the characteristic representation of CT for hep-atolenticular degeneration as well as relationship between both. Methods Clinical representation, blood biochemical index and CT scan of head were observed in 14 patients with hepatolenticular degeneration. Results Symptoms of nerve system were found first in 9 cases. Hepatic lesion or hepatic lesion with mental symptom were found first in 1 case respectively. 1 case was found according to arthrosis symptom. Symptom was not found in 2 cases. Copper metabolic disturbance was proved by searching serum cuprum in all of 14 cases. CT examination found that profile expressed low density focuc of sides basel ganglion in 11 cases,encephalatrophy in 2 cases and negative in 1 case. Conclusion Those results indicate that nerve system symptom is more,and CT representation corresponds to clinical symptom.