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

Pien M M Weerkamp; Eva M Mol; Dirk J J Sweere; Debby G M Schrans; R Jeroen Vermeulen; Sylvia Klinkenberg; Petra P M Hurks; Jos G M Hendriksen

doi:10.3390/brainsci12111544

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

Pien M M Weerkamp^*, Eva M Mol, Dirk J J Sweere, Debby G M Schrans, R Jeroen Vermeulen, Sylvia Klinkenberg, Petra P M Hurks, Jos G M Hendriksen

^*Corresponding author for this work

Research output: Contribution to journal › (Systematic) Review article › peer-review

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.

Original language	English
Article number	1544
Number of pages	19
Journal	Brain Sciences
Volume	12
Issue number	11
DOIs	https://doi.org/10.3390/brainsci12111544
Publication status	Published - 14 Nov 2022

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@article{69bc13676b094fde9c0f963bfd6db667,

title = "Wechsler Scale Intelligence Testing in Males with Dystrophinopathies: A Review and Meta-Analysis",

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.",

author = "Weerkamp, {Pien M M} and Mol, {Eva M} and Sweere, {Dirk J J} and Schrans, {Debby G M} and Vermeulen, {R Jeroen} and Sylvia Klinkenberg and Hurks, {Petra P M} and Hendriksen, {Jos G M}",

note = "Funding Information: We gratefully acknowledge support to P.W. and J.H. from the European Union Horizon 2020 Framework Programme research and innovation programme {\textquoteleft}Brain Involvement iN Dystrophinopathies{\textquoteright} (grant agreement, 847826). Special thanks to I. Gijselhart for her help creating the searches. Publisher Copyright: {\textcopyright} 2022 by the authors.",

year = "2022",

month = nov,

day = "14",

doi = "10.3390/brainsci12111544",

language = "English",

volume = "12",

journal = "Brain Sciences",

issn = "2076-3425",

publisher = "Multidisciplinary Digital Publishing Institute (MDPI)",

number = "11",

}

TY - JOUR

T1 - Wechsler Scale Intelligence Testing in Males with Dystrophinopathies

T2 - A Review and Meta-Analysis

AU - Weerkamp, Pien M M

AU - Mol, Eva M

AU - Sweere, Dirk J J

AU - Schrans, Debby G M

AU - Vermeulen, R Jeroen

AU - Klinkenberg, Sylvia

AU - Hurks, Petra P M

AU - Hendriksen, Jos G M

N1 - Funding Information: We gratefully acknowledge support to P.W. and J.H. from the European Union Horizon 2020 Framework Programme research and innovation programme ‘Brain Involvement iN Dystrophinopathies’ (grant agreement, 847826). Special thanks to I. Gijselhart for her help creating the searches. Publisher Copyright: © 2022 by the authors.

PY - 2022/11/14

Y1 - 2022/11/14

N2 - 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.

AB - 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.

U2 - 10.3390/brainsci12111544

DO - 10.3390/brainsci12111544

M3 - (Systematic) Review article

C2 - 36421868

SN - 2076-3425

VL - 12

JO - Brain Sciences

JF - Brain Sciences

IS - 11

M1 - 1544

ER -

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

Abstract

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