摘要
目的明确非脑型肝豆状核变性(HLD)成人患者智商(IQ)情况。方法使用龚氏修订的韦氏成人智力量表(WAIS-RC)对40例非脑型HLD成人患者进行IQ测定,并与30位正常人对照组进行比较。对该组非脑型HLD成人患者进行神经系统检查和辅助检查。结果非脑型HLD患者WAIS-RC11项测试中有相似、数字广度、填图、图片排列、图形拼凑5项结果高于正常对照组(P<0.05),余6项结果与正常对照组无显著性差异(P>0.05)。非脑型HLD患者组言语智商(VIQ)、操作智商(PIQ)、及全量表智商(FIQ)均值分别为111.6±10.85、101.6±10.5、106.7±10.39,正常对照组VIQ、PIQ、及FIQ均值分别为112.1±10.97、100.5±9.6、107.3±10.5;两组比较差异无显著性。非脑型HLD患者IQ120~129者4例(10%),110~119者13例(32.5%),90~109者21例(51%),均高于正常人韦氏量表实际分布百分数。非脑型HLD患者虽然临床上均尚未出现明显锥体外系损害表现、精神障碍等,但神经系统检查、化验、B超及神经电生理等检查结果表明依然存在着全身组织器官的铜沉积表现,头颅CT和MRI检查却基本上尚未产生脑组织明显异常的结构性改变。结论非脑型HLD患者不存在智力障碍,IQ总体水平与正常成人无差异,并且部分测试项目高于正常对照组。考虑非脑型HLD患者脑组织处于轻微的铜沉积阶段,可能存在着与铜相关酶的活性增强有关。
Objectives To clarify the level of adult non- cerebral type hepatolenticular degeneration (HLD) patients' intelligence quotient (IQ). Methods forty adult non- cerebral type HLD patients were selected as research group and tested by Wechsler adult intelligence scale-revised by china (WAIS-RC), and thirty normal people were selected as control group.The patients in research group were given neurologic check and auxiliary examination. Results compared with control group , the levels of five results out of eleven tests such as resembiance,the extent of figure, completion of drawing,picture's arrangement, picture's puting together were higher (P〈0.05). There were no significant difference in the remaining six test results when compared with control group(P〉0.05). The average score of non- cerabral type HLD patients' verbal intelligence quotient (VIQ), performance intelligence quotient (PIQ) and full measuring scale intelligence quotien(FIQ) were 11 1.6±10.85,101.6±10.5,106.7±10.39,respectively; The average score of VIQ, PIQ,and FIQ in nor- mal control group were 112.1±10.97, 100.5±9.6, 107.3±10.5, respectively; There were no significant difference between two groups.Among research group,there were four patients (10%)whose IQ rang from120 to129,thirteen patients (32.5%) whose IQ rang from 110 to119,and twenty-one patients (51%)whose IQ rang from 90 to 109, respectively;And all the practical percentile were higher than those in normal people.Ahhough there were no appearing obviously extracorticospinal tract damage ormental disorder and so on in these non- cerabral type HLD patients, the results of neurologic check,chemical examination,B ultrasonic wave and Electrophysiology show that it also exist deposition of copper in all body's tissue and organic. Othermore,they brain tissue was no discovered existing structural changes by using CT and MIR. Conclusions non- cerabral type HLD patients were no exsiting intelligence damage;the were no significant difference with the overall level of IQ when compared with normal people;moreover, some test objects' levels were higher than those in control group.we consider that non- cerabral type HLD patients' brain tissue was in the stage of slight deposition of copper, which probably exist to strengthen copper correlated enzymatic activity.
出处
《安徽医学》
2009年第2期97-100,共4页
Anhui Medical Journal
基金
安徽省自然科学基金项目(050430803)
安徽省教育厅自然科学研究项目(2006KJ070C)