Résumé :
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Communication n° 132. Background : Impairment of intelligence in Duchenne muscular dystrophy (DMD) was described by Duchenne de Boulogne himself in 1868.Further studies reported intelligence disorders with major impairment of memory and language functions. Memory deficit mainly involves long-term memory; short-term memory impairment was also present, even if less frequently. Some studies tried to establish a correlation between the distal deletion of the dystrophin gene and neuropsychological performance. Methods: The present study investigated the neuropsychological performance in a group of DMD children (20 subjects);neuropsychological tests included: Wechsler intelligence scale for children-revised; Raven's progressive matrices test; Zazzo's deux barrages test; Benton's visual retention form D; Frostig's developmental test of visual perception; Rey's complex figure test.These patients did not have any brain anomaly evident on neuroimaging. Results : Only 6 subjects had a total IQ below average.In case 6, a mild and non-significant impairment in the long-term verbal memory was detected as was minor attention deficit; this patient also had slight difficulties in the immediate auditive memory, and in recent memory. Discussion and Conclusions : The present study was therefore aimed to assess, through a battery of multisectorial tests, some neuropsychological functions and the presence of sectorial defects in DMD.DMD alters not only psychomotor development, but also neuropsicological disorders, causing dramatic changes in the subject's life.
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