Reading ability and processing in Duchenne muscular dystrophy and spinal muscular atrophy

Abstract

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.