Résumé :
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Background Previous studies have shown variable degree of cognitive impairment in the childhood form of DM1 (Steayert et al., 1997; Gossens et al., 2000). It ranges from mental retardation to subnormal intelligence but dysfunction in verbal working memory and visuospatial abilities. Negative correlation with the CTG expansion size has also been reported (Angeard et al., 2007). Method We present here the results of a neuropsychological study on 20 subjects aged from 7 to 17 years with the childhood phenotype and no mental retardation (inclusion criteria). Results Concerning global cognitive abilities, the mean FSIQ was in the subnormal range with a significant dissociation between verbal (VIQ = 91) and non-verbal scores (PIQ = 78). In neuropsychological measures focusing on mental flexibility, cognitive inhibition and planning, the sample study scored significantly lower than the normative reference population. Concerning memory, we observed a dissociation depending on the nature of the information to be treated: in the memorization of a list of words or a story, subject obtain normal scores in immediate and delayed recall but impaired performances for the geometrical figure. Conclusion The pattern of dysfunction in speed of processing and planning observed in the children and adolescents could be related to those observed in the adult classical form of DM1 (Meola et al., 2007). Short-term and long-term verbal memory preservation could be used to elaborate well-adapted rehabilitation.
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