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患亨廷顿舞蹈症高/低风险人群的智力指数

Intelligence indices in people with a high/low risk for developing Huntington's disease.

作者信息

de Boo G M, Tibben A, Lanser J B, Jennekens-Schinkel A, Hermans J, Vegter-van der Vlis M, Roos R A

机构信息

Department of Neurology, Leiden University Hospital, The Netherlands.

出版信息

J Med Genet. 1997 Jul;34(7):564-8. doi: 10.1136/jmg.34.7.564.

Abstract

Intelligence in 20 presymptomatic subjects with an increased risk (> 95%) for carrying the gene for Huntington's disease (HD) was studied in a prospective, case-control, single blind study. No significant differences between the groups were detected for intelligence indices and subtest scores (Wechsler Adult Intelligence Scale). The high level of the performance IQ and the significant discrepancy between performance IQ and verbal IQ found in both the high risk and the low risk groups contrasted with our expectations based on anamnestic information, general clinical opinion, and the results of previously conducted studies. We propose that psychosocial circumstances could explain the test results and discuss the consequences of our findings for clinical genetics practice.

摘要

在一项前瞻性、病例对照、单盲研究中,对20名携带亨廷顿舞蹈症(HD)基因风险增加(>95%)的症状前受试者的智力进行了研究。在智力指数和分测验分数(韦氏成人智力量表)方面,未检测到两组之间存在显著差异。高风险组和低风险组中表现智商的高水平以及表现智商与言语智商之间的显著差异,与我们基于既往信息、一般临床意见和先前进行的研究结果的预期形成对比。我们提出社会心理环境可以解释测试结果,并讨论了我们的发现对临床遗传学实践的影响。

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本文引用的文献

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Psychiatric syndromes in Huntington's disease.
Am J Psychiatry. 1983 Jun;140(6):728-33. doi: 10.1176/ajp.140.6.728.
9
Language functions in Huntington's disease.亨廷顿舞蹈症中的语言功能。
Brain. 1988 Dec;111 ( Pt 6):1475-503. doi: 10.1093/brain/111.6.1475.
10

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