No abstract

Duchenne muscular dystrophy (DMD) is a fatal recessive X-linked muscular disease affecting about 1 in 3500 live-born human males, characterized by severe muscle degeneration and responsible for muscular weakness. *Correspondence to first nictbor at address nbotieWe analysed the reading avities 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 DIM) children exhibited a reading age which was siWcantly lower than the SMA children compared with their chronological age. These learning disabilities were not related to adeficit in non-verbal performance intelligence, but psycholinguistic evaluation Simildties and Arithmetic WISO-R subtests, in phonological abilities, oral word repetition, and in digit span score. The results for the DWD children were heterogeneous, and-ranged from normal to greater or lesser involvement. X n an attempt to c l a m 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 r e e g non-words, suggesting reading disability to dysphonetic dyslexia, the most freqbent 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 consequenees of diagnosis on rehabilitation are very important. The precise description of the cognitive deflcits seen in DMD js of value for futpre clinical and genetic studies.

The major aim of this study was to assess whether the syndrome of nonverbal learning disabilities described in hydrocephalic children is observed in adulthood. Eleven adults shunted for congenital hydrocephalus related to spina bifida and eight adults shunted for hydrocephalus related to aqueductal stenosis were administered an extensive neuropsychologic battery to investigate discrepancies between verbal and visuospatial cognition, verbal and visuospatial long-term memory, and psycho-social adaptive abilities. The results showed no discrepancies between Wechsler Performance IQ or Verbal IQ in either hydrocephalic group. Nevertheless, the subjects with spina bifida appeared more cognitively impaired than the subjects with aqueductal stenosis, who performed normally on the Wechsler Adult Intelligence Scale-Revised. Memory assessment using Signoret's Memory Battery revealed no discrepancy between verbal and visuospatial memory in the hydrocephalic group. Nevertheless, the subjects with spina bifida had poorer verbal and visuospatial memory performance than the subjects with aqueductal stenosis. There were no differences on the Vineland Adaptive Behavioral Scale between subjects with spina bifida and those with aqueductal stenosis in autonomy, socialization, and daily living skills. These results suggest that shunted congenital hydrocephalus is not characterized by nonverbal learning disabilities syndrome in adolescence or in adulthood.

In order to clarify the relationship between developmental dysphasia and EEG abnormalities, paroxysmal activities during sleep were studied in a series of 24 children with expressive developmental dysphasia (mean age 8 years) and compared to a control group of 39 children (mean age 9 years). The children of both groups were selected excluding cases with prior history of neurological disease or epilepsy. In the control group, 37 children had normal sleep EEG while 2 children had paroxysmal abnormalities. In the dysphasic group, epileptic abnormalities were observed in 9 cases, rare in 4 cases and frequent in 5 cases (density: 2.5 to 66.2% of total sleep time). Nevertheless, paroxysmal abnormalities did not reach the frequency described in the Landau-Kleffner syndrome, and it is unlikely that EEG abnormalities could have produced dysphasia.