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Review
. 2022 Nov 14;12(11):1544.
doi: 10.3390/brainsci12111544.

Wechsler Scale Intelligence Testing in Males with Dystrophinopathies: A Review and Meta-Analysis

Pien M M Weerkamp  1   2 , Eva M Mol  1   3 , Dirk J J Sweere  1   2 , Debby G M Schrans  4 , R Jeroen Vermeulen  2   5 , Sylvia Klinkenberg  1   2   5 , Petra P M Hurks  2   5 , Jos G M Hendriksen  1   2
Affiliations
Review

Wechsler Scale Intelligence Testing in Males with Dystrophinopathies: A Review and Meta-Analysis

Pien M M Weerkamp et al. Brain Sci. .

Abstract

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.

Keywords: Wechsler Intelligence Scales; dystrophinopathies; meta-analysis.

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Conflict of interest statement

The authors declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article.

Figures

Figure 1
Figure 1
Flowchart of the selected articles.
Figure 2
Figure 2
Forest plot of full-scale intelligence scores in the DMD population. Abbreviations used: CI = confidence interval; p = significance level; SMD = standard mean difference; SD = standard deviation [10,13,24,26,41,96,97,98,99,101,102,103,104,107,108,109,110,113,114,115,117,118,121,122,123,124,125,126,127,128,129].
Figure 3
Figure 3
Forest plot of full-scale intelligence scores in the BMD population. Abbreviations used: CI = confidence interval; p = significance level; SMD = standard mean difference; SD = standard deviation [,,,,,,,].
Figure 4
Figure 4
Funnel plot of full-scale intelligence scores in the DMD population.
Figure 5
Figure 5
Funnel plot of full-scale intelligence scores in the BMD population.
See this image and copyright information in PMC

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