Cognitive Event-Related Potentials (P300) and Cognitive Impairment in Duchenne Muscular Dystrophy
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Duchenne muscular dystrophy (DMD) is a progressing disorder characterized by muscle wasting and weakness due to the absence or alteration of the function of dystrophin that protects muscle cells from mechanical stress induced by a movement during contraction. The function of dystrophin isoforms expressed in the brain is not fully understood, but the presence of non-progressing cognitive impairment (including disorders of learning and memory) is a common feature in patients with DMD. To establish correlation between the cognitive event-related potential P300 and psychological evaluation with an intelligence test based on the Stanford Binet Intelligence Quotient (IQ) in patients with DMD and a control group, the respective tests were performed in 31 patients with DMD and 30 controls. The mean age of the group with DMD was 9.35 ± 2.88 years, while that in control children was 9.43 ± 2.69 years (P = 0.89). The IQ was 90.77 ± 12.62 in the DMD group and 106.77 ± 9.62 in the controls (P < 0.0001). The amplitude of the cognitive potential P300 in leads Fz, Cz, and Pz showed no statistically significant differences between the groups. Thus, parameters of the P300 potential and cognitive assessment showed nearly no relationship in patients with DMD vs. controls.
KeywordsDuchenne muscular dystrophy cognition event-related potentials P300 Intelligence Quotient (IQ)
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- 1.W. D. Biggar, “Duchenne muscular dystrophy,” Pediat. Rev., 27, No. 3, 83-88 (2006).Google Scholar
- 9.K. D. Mathews, C. Cunniff, J. R. Kantamneni, et al., “Muscular dystrophy surveillance tracking and research network (MD STARnet): case definition in surveillance for childhood-onset Duchenne/Becker muscular dystrophy,” J. Child Neurol., 25, No. 9, 1098-1102 (2010).CrossRefPubMedPubMedCentralGoogle Scholar
- 11.H. J. Heinze, T. F. Munte, M. Kutas, et al., “Cognitive event-related potentials. The International Federation of Clinical Neurophysiology,” Electroencephalogr. Clin. Neurophysiol., Suppl., 52, 91-95 (1999).Google Scholar
- 12.T. R. Bashore and M. W. van der Molen, “Discovery of the P300: a tribute,” Biol. Psychol., 32, Nos. 2/3, 155-171 (1991).Google Scholar
- 18.J. Polich, “Meta-analysis of P300 normative aging studies,” Psychophysiology, 33, No. 4, 334-353 (1996).Google Scholar
- 24.J. L. Anderson, S. I. Head, C. Rae, et al., “Brain function in Duchenne muscular dystrophy,” Brain, 125, Part 1, 4–13 (2002).Google Scholar
- 25.R. Kreis, K. Wingeier, P. Vermathen, et al., “Brain metabolite composition in relation to cognitive function and dystrophin mutations in boys with Duchenne muscular dystrophy,” NMR Biomed., 24, No. 3, 253-262 (2011).Google Scholar