Billard C, Gillet P, Barthez M, Hommet C, Bertrand P
Neurosurgery and Neurology Department, Hôpital Clocheville, Tours, France.
Dev Med Child Neurol. 1998 Jan;40(1):12-20. doi: 10.1111/j.1469-8749.1998.tb15351.x.
We analysed the reading abilities and processing of 21 children with Duchenne muscular dystrophy (DMD), 11 matched children suffering from spinal muscular atrophy (SMA) and 42 children receiving normal education. The principal result observed was that the DMD children exhibited a reading age which was significantly lower than the SMA children compared with their chronological age. These learning disabilities were not related to a deficit in non-verbal performance intelligence, but psycholinguistic evaluation showed a deficit in verbal intelligence, especially in the Similarities and Arithmetic WISC-R subtests, in phonological abilities, oral word repetition, and in digit span score. The results for the DMD children were heterogeneous, and ranged from normal to greater or lesser involvement. In an attempt to clarify the nature of this reading impairment in DMD children, the three groups (DMD, SMA, and normal control children) were tested by reading aloud a list of single words and non-words. The DMD children were significantly impaired in reading non-words, suggesting reading disability similar to dysphonetic dyslexia, the most frequent subtype of developmental dyslexia. These results are discussed in the light of psychometric data available for our DMD population and in the light of previous studies. The practical consequences of diagnosis on rehabilitation are very important. The precise description of the cognitive deficits seen in DMD is of value for future clinical and genetic studies.
我们分析了21名杜氏肌营养不良症（DMD）患儿、11名匹配的脊髓性肌萎缩症（SMA）患儿以及42名接受正常教育的儿童的阅读能力和处理过程。观察到的主要结果是，与实际年龄相比，DMD患儿的阅读年龄明显低于SMA患儿。这些学习障碍与非言语表现智力缺陷无关，但心理语言学评估显示言语智力存在缺陷，尤其是在韦氏儿童智力量表修订版（WISC-R）的相似性和算术子测试、语音能力、口语单词重复以及数字广度得分方面。DMD患儿的结果存在异质性，程度从正常到或多或少的受累不等。为了阐明DMD患儿这种阅读障碍的性质，对三组（DMD、SMA和正常对照儿童）进行了大声朗读单字和非字列表的测试。DMD患儿在阅读非字方面明显受损，表明存在类似于语音性阅读障碍的阅读障碍，这是发育性阅读障碍最常见的亚型。根据我们DMD人群的心理测量数据以及先前的研究对这些结果进行了讨论。诊断对康复的实际影响非常重要。对DMD中所见认知缺陷的精确描述对未来的临床和基因研究具有价值。
