Investigation of Poor Academic Achievement in Children with Duchenne Muscular Dystrophy.
Investigation of Poor Academic Achievement in Children with Duchenne Muscular Dystrophy.
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DOI：
10.1111/j.1540-5826.2004.00098.x
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发表时间：
2004-08-01
期刊：
影响因子：
--
通讯作者：
Stern, Y
中科院分区：
文献类型：
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作者：
Hinton, V J;De Vivo, D C;Stern, Y
Duchenne肌营养不良症(DMD)是一种神经遗传性发育障碍，表现为进行性肌肉无力。它是由一种基因突变引起的，这种突变导致缺乏通常定位于肌肉细胞和中枢神经系统(CNS)的特定产物。大多数受影响的人的智商在正常范围内，通常言语智商低于操作智商。先前的研究已经证明，在言语广度和即刻记忆测试中存在选择性缺陷。在目前的研究中，对26名患有DMD(智力功能正常)的男孩及其未受影响的兄弟姐妹进行了评估。配对比较表明，患有DMD的儿童的学习成绩明显低于他们的兄弟姐妹，尽管他们的词汇水平以及家庭和教育环境是相似的。患有DMD的儿童也有更多的行为问题、身体残疾和较差的语言记忆广度。线性回归表明，行为关注、执行功能和身体残疾对学习成绩没有实质性影响，而言语广度方面的表现对学习成绩有显著影响。DMD在言语广度上表现为选择性发育异常，对学习技能有广泛的影响。
Duchenne Muscular Dystrophy (DMD) is a neurogenetic developmental disorder that presents with progressive muscular weakness. It is caused by a mutation in a gene that results in the absence of specific products that normally localize to muscle cells and the central nervous system (CNS). The majority of affected individuals have IQs within the normal range, generally with lower verbal than performance IQ scores. Prior work has demonstrated selective deficits on tests of verbal span and immediate memory. For the current study, 26 boys with DMD (and normal intellectual function) and their unaffected siblings were evaluated. Paired comparisons demonstrated that the children with DMD had significantly poorer academic achievement scores than their siblings, even though their vocabulary levels and home and educational environments were comparable. Children with DMD also had more behavioral concerns, physical disabilities, and poorer verbal memory spans. Linear regression indicated that behavioral concerns, executive function, and physical disability did not contribute substantially to academic performance, whereas performance on verbal span did. DMD presents with a selective developmental aberration in verbal span that has wide-ranging consequences on learning skills.