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亨廷顿舞蹈症中的趋近现象:额叶/执行功能障碍的神经心理学标志物

Closing-in Phenomenon in Huntington's Disease: A Neuropsychological Marker of Frontal/Executive Dysfunction.

作者信息

De Lucia Natascia, Peluso Silvio, Roca Alessandro, Russo Cinzia Valeria, Massarelli Marco, De Michele Giovanna, Di Maio Luigi, Salvatore Elena, De Michele Giuseppe

机构信息

Department of Neurosciences, Reproductive and Odontostomatological Sciences, "Federico II" University, Naples, Italy.

出版信息

Arch Clin Neuropsychol. 2019 Feb 1;34(1):24-30. doi: 10.1093/arclin/acy020.

Abstract

OBJECTIVE

In visuo-constructional tasks, patients may reproduce drawings near-to or superimposed on a model, showing the so-called "Closing-in" (CI), often ascribed to a defect in inhibitory control. CI has been described in neurological conditions, but no studies have explored CI in Huntington's disease (HD), a neurodegenerative disorder often involving the frontal cortical-subcortical circuits. We searched for the occurrence of CI in HD patients and systematically investigated its correlates to find a clinical marker of the frontal/executive dysfunctions in the early examination of HD patients.

METHOD

We assessed 130 HD participants, who performed a graphic coping task and a neuropsychological, psychiatric, motor, and functional assessment.

RESULTS

CI occurred in 52/130 (40%) HD patients, with 43/52 (82.7%) superimposing their copy directly on the model. MANOVA showed that HD patients with CI scored significantly poorer on Symbol digit modality test, Stroop-color word - reading test, Stroop-color word - interference test, Trail making test - part B, and Phonological verbal fluency test. However, a logistic regression analysis revealed that the significant predictor of the occurrence of CI was the score on Stroop-color word - interference test.

CONCLUSIONS

HD patients may show CI in graphic tasks, and it could be related to a defect in inhibitory control impeding the switch of attention from the model to the copying space, and releasing a default tendency which causes an attraction of hand movement towards the focus of visual attention. CI might be a useful clinical marker for the early detection of frontal/executive defects in HD patients.

摘要

目的

在视觉构建任务中,患者可能会临摹出与模型相近或叠加在模型上的图形,表现出所谓的“向心性”(CI),这通常被归因于抑制控制缺陷。CI已在神经疾病中有所描述,但尚无研究探讨亨廷顿舞蹈病(HD)中的CI情况,HD是一种常累及额叶皮质 - 皮质下回路的神经退行性疾病。我们探究HD患者中CI的发生情况,并系统地研究其相关因素,以寻找HD患者早期检查中额叶/执行功能障碍的临床标志物。

方法

我们评估了130名HD参与者,他们完成了一项图形临摹任务以及神经心理学、精神病学、运动和功能评估。

结果

130名HD患者中有52名(40%)出现CI,其中43名(82.7%)将其临摹的图形直接叠加在模型上。多变量方差分析显示,出现CI的HD患者在符号数字模式测验、斯特鲁普颜色词阅读测验、斯特鲁普颜色词干扰测验、连线测验B部分和语音流畅性测验中的得分显著更低。然而,逻辑回归分析显示,CI发生的显著预测因素是斯特鲁普颜色词干扰测验的得分。

结论

HD患者在图形任务中可能表现出CI,这可能与抑制控制缺陷有关,该缺陷阻碍了注意力从模型向临摹空间的转换,并释放了一种默认倾向,导致手部动作向视觉注意力焦点吸引。CI可能是HD患者早期检测额叶/执行缺陷的有用临床标志物。

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