Department of General Psychology , University of Padova, Via Venezia 8, Padua, Italy.
Clin Neuropsychol. 2011 Nov;25(8):1359-77. doi: 10.1080/13854046.2011.617782. Epub 2011 Oct 17.
It has been shown that children with Duchenne muscular dystrophy (DMD) exhibit specific cognitive deficits. However, the neuropsychological profile has not yet been fully characterized. In order to control for the contribution of motor impairments as a confounding variable that is usually present when assessing children with muscular pathologies, we compared children with DMD to a group of children with an autoimmune pathology that does not entail either brain or cognitive dysfunction but does imply motor impairment: juvenile rheumatoid arthritis (JRA). An extensive neuropsychological evaluation was administered, including intelligence, perception, language, memory and learning, attention, executive function, praxis and reasoning. As the main result, in children with DMD we found significantly lower performances in verbal IQ, verbal short-term memory and phonological abilities, as well as in praxis and executive functioning domains. A wide range of cognitive domains, including verbal abilities, executive functioning, and praxis, must be taken into account when assessing neuropsychological functioning in children with DMD. In particular, subtle executive function impairment could be considered as a subclinical marker of cognitive impairment in developmental disorders.
已有研究表明，患有杜氏肌营养不良症（DMD）的儿童存在特定的认知缺陷。然而，神经心理学特征尚未完全确定。为了控制运动障碍的影响，因为在评估患有肌肉疾病的儿童时，运动障碍通常是一个混杂变量，我们将患有 DMD 的儿童与一组患有自身免疫性疾病的儿童进行了比较，这种疾病既不会导致脑或认知功能障碍，也不会导致运动障碍：幼年特发性关节炎（JRA）。进行了广泛的神经心理学评估，包括智力、知觉、语言、记忆和学习、注意力、执行功能、动作和推理。作为主要结果，我们发现 DMD 患儿在言语智商、言语短期记忆和语音能力方面，以及在动作和执行功能方面的表现明显较低。在评估 DMD 患儿的神经心理功能时，必须考虑到广泛的认知领域，包括语言能力、执行功能和动作。特别是，轻微的执行功能障碍可以被认为是发育障碍中认知障碍的亚临床标志物。
