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目的:通过神经心理学测验评估肝豆状核变性(HLD)患者的流体智力变化特点,探讨HLD患者流体智力变化的发生机制。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);另选正常对照组30例。记录瑞文标准推理测验(R’SPM)总分和分项结果。结果:3组间比较在R’SPM总分、知觉辨别、系列关系及抽象推理上差异有显著统计学意义(P<0.01);在R’SPM比较推理上差异有统计学意义(P<0.05)。与对照组比较,CT/MRI阳性组在R’SPM总分、知觉辨别、系列关系及抽象推理上差异有显著统计学意义(P<0.01),在R’SPM类同比较、比较推理上差异有统计学意义(P<0.05);与CT/MRI阴性组比较,CT/MRI阳性组在R’SPM系列关系上差异有显著统计学意义(P<0.01),在总分、知觉辨别、抽象推理上差异有统计学意义(P<0.05)。CT/MRI阳性组R’SPM与病程呈负相关(P<0.05,P<0.01)。结论:CT/MRI异常HLD患者存在流体智力缺陷;基底神经节器质性破坏及大脑广泛性萎缩可能是HLD患者流体智力缺陷的重要机制之一。
Objective: To evaluate the neuropsychological test in patients with hepatolenticular degeneration (HLD), the characteristics of fluid intelligence to explore the mechanism of fluid intelligence in patients with HLD. Methods: A total of 102 patients with HLD were enrolled in this study. According to the presence or absence of abnormal brain imaging, they were divided into CT / MRI positive group (70 cases) and CT / MRI negative group (32 cases). Record the total and partial results of the Raven Standard Inference Test (R’SPM). Results: There were significant differences in R’SPM score, perceptual discrimination, series relationship and abstract reasoning between the three groups (P <0.01), and there was significant difference in R’SPM comparative reasoning (P <0.05 ). Compared with the control group, there were significant differences in R’SPM score, perceived discrimination, series relationship and abstract reasoning between CT / MRI positive group (P <0.01). In the same comparison of R’SPM and comparative reasoning (P <0.05). Compared with CT / MRI negative group, CT / MRI positive group had significant difference in R’SPM series (P <0.01) Reasoning difference was statistically significant (P <0.05). The R’SPM in CT / MRI positive group was negatively correlated with the course of disease (P <0.05, P <0.01). CONCLUSION: There is fluid mental retardation in patients with abnormal HLD on CT / MRI. The organic destruction of basal ganglia and extensive brain atrophy may be one of the important mechanisms of fluid mental retardation in patients with HLD.