肝豆状核变性患者的执行功能障碍

Executive dysfunction in patients with hepatolenticular degeneration

目的通过神经心理学测验评估肝豆状核变性（HLD）患者的执行功能变化特点，探讨其发生机制。方法选取102例HLD患者为研究对象，根据颅脑影像学有无异常分为CT／MRI阳性组（70例）和CT／MRI阴性组（32例），另选择30名健康志愿者做为正常对照组。分别记录测试者瑞文标准推理测验（R’SPM）、Stroop色-字干扰测验（CWT）、连线测验（TMT）的结果。结果3组比较在R’SPM总分、知觉辨别、比较推理、系列关系、抽象推理，CWT的字义干扰时间、颜色干扰时间，TMT的B项耗时数及干扰量上差异有统计学意义（P〈0．05）。结论HLD患者存在执行功能障碍，CT／MRI阳性组的损害严重程度和范围更为明显。额叶一纹状体环路上的破坏可能是HLD患者执行功能障碍的重要机制之一。

Objective To assess the characteristics of executive dysfunction in patients with hepatolenticular degeneration （HLD） through neuropsychological tests and explore its mechanism. Methods One hundred and two patients with HLD were chosen and divided into CT/MRI positive （n=70） and CT/MRI negative （n=32） groups in accordance with cerebral imaging and the other 30 healthy subjects were selected as normal control group. These three groups were recorded the results of such imaging data as Raven＇s standard progressive matrices （R＇SPM） test, Stroop color-word test （CWT）, trail making test （TMT）. Results Imaging data indicated a statistical change of frontal-striatal loops in patients with HLD. Significant differences of the total scores, perception discrimination, serial relationship and abstract reasoning of R＇SPM test were showed in the three groups and the word interference time of CWT, B items time-consuming and interference effects of TMT in these groups were statistically different （P〈0.05）. Conclusion Executive dysfimetion appears commonly in patients with HLD. The CT/MRI positive group showed a more serious degree and area of the damage as compared to that in the CT/MRI negative group. Damages of frontal-striatal loops may be one of the important mechanisms of executive dysfunction in patients with HLD.