Résumé :
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There are clinical, neurophysiological, neuropsychological and neuroimaging evidences of brain dysfunctions in DM1 (see Meola et al., 2007 for a review). Hypotheses of developmental abnormalities in the congenital form and neurodegenerative changes in the classical adult phenotype are suggested (Di Costanzo et al., 2002). By contrast, not much is known about the neurocognitive impairment in the childhood phenotypic expression of DM1.We will present here a review of the last 14 years from the first published study of Steayert et al., (1997) to the more recent ones (Angeard et al., 2007; Ekstrm et al., 2008; Douniol et al., 2009; Echenne et al., 2009) pointing out a conceptual change from a global cognitive impairment to the notion of heterogeneous cognitive profile within Childhood DM1 population. At least, we will focus on Angeard and al. (2010) research showing, in a context of normal intelligence, higher verbal than spatial episodic memory (with no impairment between short and long-term memory), poor visuoconstructive abilities and greater deficits in processing speed than in mental flexibility, inhibition or working memory.This pattern of deficits could implicate a frontoparietal circuit (Diamond, 2005) in accordance with the neural network involved in the adult form of DM1 (Meola et al., 2003; Caramia et al., 2010; Romeo et al., 2010). Finally, the question of a continuum between childhood and adulthood neurocognitive impaiments will be discussed
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