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

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...

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Autores principales: 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.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9688319/
https://www.ncbi.nlm.nih.gov/pubmed/36421868
http://dx.doi.org/10.3390/brainsci12111544
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author 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.
author_facet 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.
author_sort Weerkamp, Pien M. M.
collection PubMed
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.
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spelling pubmed-96883192022-11-25 Wechsler Scale Intelligence Testing in Males with Dystrophinopathies: A Review and Meta-Analysis 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. Brain Sci Review 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. MDPI 2022-11-14 /pmc/articles/PMC9688319/ /pubmed/36421868 http://dx.doi.org/10.3390/brainsci12111544 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
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.
Wechsler Scale Intelligence Testing in Males with Dystrophinopathies: A Review and Meta-Analysis
title Wechsler Scale Intelligence Testing in Males with Dystrophinopathies: A Review and Meta-Analysis
title_full Wechsler Scale Intelligence Testing in Males with Dystrophinopathies: A Review and Meta-Analysis
title_fullStr Wechsler Scale Intelligence Testing in Males with Dystrophinopathies: A Review and Meta-Analysis
title_full_unstemmed Wechsler Scale Intelligence Testing in Males with Dystrophinopathies: A Review and Meta-Analysis
title_short Wechsler Scale Intelligence Testing in Males with Dystrophinopathies: A Review and Meta-Analysis
title_sort wechsler scale intelligence testing in males with dystrophinopathies: a review and meta-analysis
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9688319/
https://www.ncbi.nlm.nih.gov/pubmed/36421868
http://dx.doi.org/10.3390/brainsci12111544
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