Duchenne型肌营养不良患者认知功能调查

Cognition of Patients with Duchenne Muscular Dystrophy

目的了解Duchenne型肌营养不良（DMD）患者认知功能损害情况。方法 2013年4月~2015年12月,127例诊断明确的6~14岁DMD患者均行韦氏儿童智力量表第Ⅳ版检测,分析言语智商（VIQ）、操作智商（PIQ）以及总智商（FSIQ）。结果中国DMD患者FSIQ（93.2±18.6）分,VIQ（91.5±18.9）分,PIQ（96.6±19.5）分,均低于正常人群的100分（P〈0.001）;显著高于国外DMD患者（P〈0.001）。FSIQ〈70分的DMD患者13例（10.24%）。VIQ-PIQ差值得分（-5.0±16.6）。结论约1/10的中国DMD患者有认知功能损害,显著低于国外。言语智力比操作智力受损更重。

Objective To investigate the cognitive condition in patients with Duchenne muscular dystrophy（DMD）.Methods Wechsler Intelligence Scale for Children IV（WISC-IV） was administered for 127 DMD patients aged 6~14 years from April,2013 to December,2015.The verbal intelligence quotient（VIQ）,performance intelligence quotient（PIQ） and full-scale intelligence quotient（FSIQ） scores were analyzed.Results The FSIQ,VIQ and PIQ were（93.2±18.6）,（91.5±18.9）,and（96.6±19.5）,respectively,which were lower than 100 in normal population（P〈0.001）,and higher than that of patients abroad in the previous studies（P〈0.001）.There were 13 patients（10.24%）whose scores of FSIQ were less than 70.The VIQ-PIQ discrepancy score was（-5.0±16.6）.Conclusion About 1/10 of Chinese DMD patients impaire in cognitive function,which is significantly lower than that abroad.VIQ is more affected than PIQ.