• Media type: E-Article
  • Title: Wechsler Scale Intelligence Testing in Males with Dystrophinopathies: A Review and Meta-Analysis
  • Contributor: Weerkamp, Pien M. M.; Mol, Eva M.; Sweere, Dirk J. J.; Schrans, Debby G. M.; Vermeulen, R. Jeroen; Klinkenberg, Sylvia; Hurks, Petra P. M.; Hendriksen, Jos G. M.
  • Published: MDPI AG, 2022
  • Published in: Brain Sciences, 12 (2022) 11, Seite 1544
  • Language: English
  • DOI: 10.3390/brainsci12111544
  • ISSN: 2076-3425
  • Origination:
  • Footnote:
  • Description: Background: Intelligence scores in males with Duchenne Muscular Dystrophy (DMD) and Becker Muscular Dystrophy (BMD) remain a major issue in clinical practice. We performed a literature review and meta-analysis to further delineate the intellectual functioning of dystrophinopathies. Method: Published, peer-reviewed articles assessing intelligence, using Wechsler Scales, of males with DMD or BMD were searched from 1960 to 2022. Meta-analysis with random-effects models was conducted, assessing weighted, mean effect sizes of full-scale IQ (FSIQ) scores relative to normative data (Mean = 100, Standard Deviation = 15). Post hoc we analysed differences between performance and verbal intelligence scores. Results: 43 studies were included, reporting data on 1472 males with dystrophinopathies; with FSIQ scores available for 1234 DMD (k = 32) and 101 BMD (k = 7). DMD males score, on average, one standard deviation below average (FSIQ = 84.76) and significantly lower than BMD (FSIQ = 92.11). Compared to a previous meta-analysis published in 2001, we find, on average, significantly higher FSIQ scores in DMD. Conclusion: Males with Duchenne have, on average, significantly lower FSIQ scores than BMD males and the general population. Clinicians must consider lower intelligence in dystrophinopathies to ensure good clinical practice.
  • Access State: Open Access