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Observational studies that determined the prevalence of IDD in the population with BMD or DMD were included. Meta\u2010analyses of IDD prevalence and prevalence ratios of the IDD\u2013genotype association were conducted.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Forty\u2010nine studies were included. The prevalence of IDD in BMD was 8.0% (95% confidence interval 5.0\u201311.0), and in DMD it was 22.0% (18.0\u201327.0). Meta\u2010analyses of IDD\u2013genotype association showed a deleterious association between IDD and the number of isoforms affected in DMD, with a prevalence ratio\u00a0=\u00a00.43 (0.28\u20130.64) and 0.17 (0.09\u20130.34) for <jats:italic>Dp140<\/jats:italic><jats:sup>+<\/jats:sup>\/<jats:italic>Dp71<\/jats:italic><jats:sup>+<\/jats:sup> versus <jats:italic>Dp140<\/jats:italic><jats:sup>\u2212<\/jats:sup>\/<jats:italic>Dp71<\/jats:italic><jats:sup>+<\/jats:sup> and <jats:italic>Dp140<\/jats:italic><jats:sup>+<\/jats:sup>\/<jats:italic>Dp71<\/jats:italic><jats:sup>+<\/jats:sup> versus <jats:italic>Dp140<\/jats:italic><jats:sup>\u2212<\/jats:sup>\/<jats:italic>Dp71<\/jats:italic><jats:sup>\u2212<\/jats:sup> comparisons respectively. However, in BMD, there was no association for <jats:italic>Dp140<\/jats:italic><jats:sup>+<\/jats:sup>\/<jats:italic>Dp71<\/jats:italic><jats:sup>+<\/jats:sup> versus <jats:italic>Dp140<\/jats:italic><jats:sup>\u2212<\/jats:sup>\/<jats:italic>Dp71<\/jats:italic><jats:sup>+<\/jats:sup>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>There is a high prevalence of IDD in BMD and DMD. Moreover, the number of isoforms affected is strongly and negatively associated with the prevalence of IDD in DMD.<\/jats:p><\/jats:sec><jats:sec><jats:title>What this paper adds<\/jats:title><jats:p>\n<jats:list list-type=\"bullet\">\n<jats:list-item><jats:p>The global prevalence of intellectual developmental disorder (IDD) was 8% in Becker muscular dystrophy and 22% in Duchenne muscular dystrophy (DMD).<\/jats:p><\/jats:list-item>\n<jats:list-item><jats:p>The global prevalence of IDD in DMD was 12%, 29%, and 84% in participants with <jats:italic>Dp427<\/jats:italic><jats:sup>\u2212<\/jats:sup>\/<jats:italic>Dp140<\/jats:italic><jats:sup>+<\/jats:sup>\/<jats:italic>Dp71<\/jats:italic><jats:sup>+<\/jats:sup>, <jats:italic>Dp427<\/jats:italic><jats:sup>\u2212<\/jats:sup>\/<jats:italic>Dp140<\/jats:italic><jats:sup>\u2212<\/jats:sup>\/<jats:italic>Dp71<\/jats:italic><jats:sup>+<\/jats:sup>, and <jats:italic>Dp427<\/jats:italic><jats:sup>\u2212<\/jats:sup>\/<jats:italic>Dp140<\/jats:italic><jats:sup>\u2212<\/jats:sup>\/<jats:italic>Dp71<\/jats:italic><jats:sup>\u2212<\/jats:sup> genotypes respectively.<\/jats:p><\/jats:list-item>\n<jats:list-item><jats:p>In DMD, 12% and 22% of participants had abnormal performance IQ and verbal IQ values respectively.<\/jats:p><\/jats:list-item>\n<\/jats:list><\/jats:p><\/jats:sec>","DOI":"10.1111\/dmcn.15481","type":"journal-article","created":{"date-parts":[[2022,11,28]],"date-time":"2022-11-28T09:31:52Z","timestamp":1669627912000},"page":"734-744","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Global prevalence of intellectual developmental disorder in dystrophinopathies: A systematic review and 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