Intellectual function in muscular dystrophies

Glaub, T.; Mechler, Ferenc

doi:10.1007/bf00377429

Cited by 7 publications

(3 citation statements)

References 19 publications

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“… 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 ]. …”

Section: Figurementioning

confidence: 99%

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

et al. 2022

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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.

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Section: Figurementioning

confidence: 99%

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

et al. 2022

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“…problems with phonic analysis and synthesis of words as well as perception of the visual shape of words (Billard et al 1992(Billard et al , 1998Cotton et al 2001). A number of investigators have hypothesized that the cerebellum plays an important role in the manifestation of cognitive deficits in dystrophinopathies ( Individual studies finding a difference between verbal and performance IQ include Karagan & Zellweger (1976), Karagan & Zellweger (1978), Karagan (1979), Glaub & Mechler (1987), Bresolin et al (1994), Roccella et al (2003), andOgasawara (1989a). In a more recent metaanalysis VIQ and PIQ data was available for >800 children with VIQ = 80.4 +/-18.8 (SD, n=881) and PIQ 85.4 +/-16.9 (SD, n=878), both significantly different from the normal population.…”

Section: Functional Evidence Of Cns Aberrations In Dystrophinopathymentioning

confidence: 99%

Duchenne Muscular Dystrophy and Brain Function

Anderson¹,

Head²,

Morley³

2012

Muscular Dystrophy

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“…35 There is only one study in which a positive mean VIQ-PIQ discrepancy (0.09) has been reported. 39 Figure 4 shows the frequency distribution of VIQ-PIQ discrepancies for 877 children with DMD. The mean VIQ-PIQ discrepancy score was -5.1 (SD 14.4), with discrepancies ranging from -44 to +46.…”

Section: Verbal and Non-verbal Intelligencementioning

confidence: 99%