Assessment of Intelligence of Duchenne Muscular Dystrophy (DMD) Children and Adolescents and Parental Stress in a Muscular Dystrophy Center in Nepal

Rana, Mita; Adhikari, Sirjana; Pradhan, Monalisa

doi:10.3126/jnps.v36i3.16357

Cited by 4 publications

(5 citation statements)

References 13 publications

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“…Cognitive stratification based on IQ showed a similar distribution to that in the literature: 28% (normal IQ), 19% (borderline intelligence), 25% (mild ID), and 6% (moderate ID). Severe ID and profound ID has been estimated in 0.4% to 10% and 0.08% of patients [2][3][4][5][6][7][8][9][10], but we did not find these categories in our sample. There was a clear decreasing gradient of mean MMSE total score in four cognitive categories: 24 ± 4.9 (normal IQ), 23.1 ± 4.5 (borderline IQ), 18.6 ± 6 (mild ID), and 12.5 (moderate ID) (r s = 0.52, P < 0.001).…”

Section: Discussioncontrasting

confidence: 75%

“…63%) [2][3][4][5][6][7][8][9][10][11][12], borderline intelligence (10% to 26%) [6][7][8], mild ID (15% to 27%) [2,6,10,11], moderate ID (6.7% to 10%) [2,10], severe ID (0.4% to 10%) [2,10], and profound ID (0.08%) among DMD patients [2]. Several studies have found an overall reduction of intelligence quotient (IQ) in boys with DMD, by the Wechsler scale, recording total scores from 76 to 90.2 [2][3][4][5][6][7]9,[13][14][15].…”

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confidence: 99%

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The Mini-Mental State Examination (MMSE) as a Cognitive Screening Tool in Duchenne Muscular Dystrophy

et al. 2020

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Duchenne muscular dystrophy (DMD), a lethal X-linked recessive neuromuscular disorder, is caused by mutations in the gene encoding the dystrophin protein. Besides muscle weakness, static cogni-Purpose: Given that intellectual disability affects 15% to 63% of boys with Duchenne muscular dystrophy (DMD), it is relevant to evaluate if the Mini-Mental State Examination (MMSE) is a reliable test for screening in these cognitively impaired boys. Methods: A descriptive study of 74 DMD boys was performed. Age, years of schooling, genotype, and performance in the MMSE and Wechsler Intelligence Scale for Children (WISC)-IV were verified. Results: Sixty-seven patients, with a mean of age 12.1±3 years were enrolled. Verbal comprehension (76.9±13.9) and working memory (71.2±16.7) indexes showed the highest impairment. Correlation between the MMSE (20.3±6.1) and WISC-IV (74.4±15.6) was moderate (r=0.556). Conclusion: The MMSE is a good cognitive screening test for DMD patients of 9 years and older, particularly in countries where the public health system does not provide expensive neuropsychological batteries, and has 78% accuracy and 82% sensitivity for scores ≤21. Subtests of the MMSE with better discriminatory power (for orientation, attention, and calculation) can help educators, teachers, and families to develop simple activities or academic games that enhance such mental skills.

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Section: Discussioncontrasting

confidence: 75%

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confidence: 99%

The Mini-Mental State Examination (MMSE) as a Cognitive Screening Tool in Duchenne Muscular Dystrophy

et al. 2020

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“…Of the 2913 records initially identified (Figure S1), 59 were read in full text, of which 49 met the inclusion/exclusion criteria and were included in the systematic review (Table S1) 31–79 (47 of them in the meta‐analyses 32–39,41–79 ), while 10 were excluded for not including the outcome of interest or for other justified reasons (Table S2).…”

Section: Resultsmentioning

confidence: 99%

Global prevalence of intellectual developmental disorder in dystrophinopathies: A systematic review and meta‐analysis

Pascual‐Morena

Cavero‐Redondo

Álvarez‐Bueno

et al. 2022

Develop Med Child Neuro

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Aim To estimate the global prevalence of intellectual developmental disorder (IDD) and the IDD prevalence–genotype association in Becker muscular dystrophy (BMD) or Duchenne muscular dystrophy (DMD) according to the affected isoforms of the DMD gene: Dp427, Dp140, Dp71. Method Systematic searches in MEDLINE, Scopus, Web of Science, and the Cochrane Library were conducted from inception of each database to March 2022. Observational studies that determined the prevalence of IDD in the population with BMD or DMD were included. Meta‐analyses of IDD prevalence and prevalence ratios of the IDD–genotype association were conducted. Results Forty‐nine studies were included. The prevalence of IDD in BMD was 8.0% (95% confidence interval 5.0–11.0), and in DMD it was 22.0% (18.0–27.0). Meta‐analyses of IDD–genotype association showed a deleterious association between IDD and the number of isoforms affected in DMD, with a prevalence ratio = 0.43 (0.28–0.64) and 0.17 (0.09–0.34) for Dp140+/Dp71+ versus Dp140−/Dp71+ and Dp140+/Dp71+ versus Dp140−/Dp71− comparisons respectively. However, in BMD, there was no association for Dp140+/Dp71+ versus Dp140−/Dp71+. Interpretation There is a high prevalence of IDD in BMD and DMD. Moreover, the number of isoforms affected is strongly and negatively associated with the prevalence of IDD in DMD. What this paper adds The global prevalence of intellectual developmental disorder (IDD) was 8% in Becker muscular dystrophy and 22% in Duchenne muscular dystrophy (DMD). The global prevalence of IDD in DMD was 12%, 29%, and 84% in participants with Dp427−/Dp140+/Dp71+, Dp427−/Dp140−/Dp71+, and Dp427−/Dp140−/Dp71− genotypes respectively. In DMD, 12% and 22% of participants had abnormal performance IQ and verbal IQ values respectively.

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“…Children with DMD stop walking (Birnkrant et al, 2018) approximately between the ages of 10 and 12. In some children, their cognitive ability is also compromised (Anikiej, Mánski, & Bidzan, 2018;Naidoo & Anthony, 2020), while in others it is preserved intact (Rana et al, 2016).…”

Section: Introductionmentioning

confidence: 99%

Experiences of caregivers of boys with Duchenne Muscular Dystrophy

Palacios‐Espinosa

Márquez

Mateus

et al. 2021

Interd

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Assessment of Intelligence of Duchenne Muscular Dystrophy (DMD) Children and Adolescents and Parental Stress in a Muscular Dystrophy Center in Nepal

Abstract: Introduction: Duchene Muscular Dystrophy (DMD) is

Cited by 4 publications

References 13 publications

The Mini-Mental State Examination (MMSE) as a Cognitive Screening Tool in Duchenne Muscular Dystrophy

The Mini-Mental State Examination (MMSE) as a Cognitive Screening Tool in Duchenne Muscular Dystrophy

Global prevalence of intellectual developmental disorder in dystrophinopathies: A systematic review and meta‐analysis

Experiences of caregivers of boys with Duchenne Muscular Dystrophy

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