Résumé :
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Duchenne muscular dystrophy is a neuromuscular disease genetically inherited X-linked condition with progressive physical disability. The exact functional role of the dystrophin in the brain is unknown, but their absence is associated with a downward shift in intelligence quotients (IQ). P300 makes an objective estimation of the cognition disorders related with central nervous system Objective : To examine the relation between cognitive evoked potentials (P300 potentials) and neuropsychological dysfunction in patients with Duchenne muscular dystrophy (DMD). Method : this was a prospective, comparative and descriptive study. P300 potentials and neuropsychological test (Standford- Binet ) results were obtained from 31 DMD patients and 30 healthy control boys. Mean age was 9.44 (2.25). Full Intelligence Quotients (IQ) were estimated for patients and control group. Statistic Analysis were made with Man Whitney Test and Chi square to compare IQ and P300, significance (p>0.05). Results : The mean IQ values were 93.21 (10.39) DMD patient group and 107.54 (9.24) in the control group (p=0.000) both are in the normal IQ. Mean P300 values in Pz were 314.75 in the DMD group and 311.50 in the control group (p=0. 730). There was no significant correlation between parameters in each group. The most afected area in the was numeric reasoning (p=0.001), conceptual thinking (p=0.002), memory (p=0.003), reasoning (p=0.005), social intelligence (p=0.033) and visuomotor area (p=0.051). Conclusion : there is no correlation statistically significant between the P300 component and the IQ. The values were normal in both groups. The mayority of the patients in the Duchenne group had normal intelligence. Systematic alterations in neuropsychological test results were found to be statistically significant.
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