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.