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目的:探讨肝豆状核变性患者的认知情绪调节方式的特征及其与病变脑区的关系。方法:采用认知情绪调节策略问卷中文版(cognitive emotion regulation,CERQ-C)对78例肝豆状核变性患者(WD患者组)以及84例健康成年人(健康对照组)进行问卷测查,并对78例患者进行颅脑3.0 T磁共振(MRI)检查。分析WD患者在遭遇负性生活事件时采用的认知情绪调节方式的特征,以及与健康人的差异性。结合头颅MRI进一步分析不同脑结构损害与认知情绪调节方式的关系。结果:(1)WD患者组在自我责难、沉思、灾难化3个分量表上的得分[分别为(11.41±2.46)分、(11.27±3.09)分、(9.53±2.85)分]显著高于健康对照组[分别为(9.57±2.81)分、(9.79±2.63)分、(8.05±2.42)分](n t=4.417,3.409,3.563,n P<0.01),在重新关注计划、积极重新评价2个分量表上的得分[分别为(13.26±3.41)分、(13.49±3.14)分]显著低于健康对照组[分别为(14.88±2.57)分、(14.42±2.41)分](n t=-3.404,-2.103,n P<0.05)。(2)多元线性回归分析显示,额叶损伤是分量表重新积极关注因子分的影响因素(n B=-2.142,n P=0.035);丘脑损伤(n B=2.058,n P=0.037)和壳核损伤(n B=-2.348,n P=0.011)是分量表沉思因子分的影响因素。n 结论:WD患者在遭遇负性生活事件时较健康人群更多地使用自我责难、沉思、灾难化这三种非适应性调节方式,不同脑区损伤对WD患者认知情绪调节方式的选择有不同程度的影响。“,”Objective:To explore the characteristics of cognitive emotion regulation in patients with hepatolenticular degeneration, and the relationship with brain lesions.Methods:The Chinese version of cognitive emotion regulation (CERQ-C) was used to investigate 78 patients with hepatolenticular degeneration(WD group) and 84 healthy adults, and all patients were examined with brain 3.0 T magnetic resonance.The characteristics of cognitive and emotional regulation in WD group were analyzed when they encounterd negative life events, and the differences between WD group and healthy people.The relationship was further analyzed between different brain structure damage and cognitive emotion regulation combined with brain MRI.Results:(1)The scores of self censure, contemplation and catastrophes in WD group((11.41±2.46), (11.27±3.09), (9.53±2.85), respectively) were significantly higher than those in healthy control group(respectively(9.57±2.81), (9.79±2.63), (8.05±2.42))(n t=4.417, 3.409, 3.563, n P<0.01). The scores of refocus planning and positive reevaluation ((13.26±3.41), (13.49±3.14)) in MD group were significantly lower than those of the healthy control group ((14.88±2.57), (14.42±2.41))(n t=-3.404, -2.103, n P<0.05). (2)Multiple linear regression analysis showed that frontal lobe injury was the influencing factor of the score of positive re-evaluation (n B=-2.142, n P=0.035), thalamic injury (n B=2.058, n P=0.037) and putamen injury (n B=-2.348, n P=0.011) were the influencing factors of the score of contemplation.n Conclusion:WD patients are more likely to choose self censure, contemplation and catastrophes than healthy people when they encounter negative life events.Different brain regions have different effects on the choice of cognitive emotion regulation in WD patients.