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Investigation of Poor Academic Achievement in Children with Duchenne Muscular Dystrophy

机译:杜氏肌营养不良症儿童学业不良的调查

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摘要

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.
机译:杜兴肌营养不良症(DMD)是一种神经遗传性发育障碍,表现为进行性肌无力。它是由基因突变引起的,该基因突变导致通常位于肌肉细胞和中枢神经系统(CNS)的特定产物缺失。大多数受影响的个体的智商都在正常范围内,通常口头表达低于表现智商得分。先前的工作已经证明了对语言跨度和即时记忆的选择性缺陷。在本研究中,对26名DMD(及正常智力功能)男孩及其未受影响的兄弟姐妹进行了评估。配对比较表明,尽管DMD儿童的词汇水平,家庭和教育环境具有可比性,但他们的学习成绩却比其兄弟姐妹差得多。 DMD儿童还具有更多的行为问题,肢体残疾和较差的言语记忆力。线性回归表明,行为方面的关注,执行功能和肢体残疾对学习成绩没有实质性贡献,而口头跨度的表现却有很大帮助。 DMD在言语跨度方面表现出选择性的发育畸变,对学习技能产生广泛的影响。

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