Mathematical learning disabilities in children with 22q11.2 deletion syndrome: A review

Centre for Parenting, Child Welfare and Disabilities, Katholieke Universiteit Leuven, Leuven, Belgium.
Developmental Disabilities Research Reviews (Impact Factor: 0.29). 01/2009; 15(1):4-10. DOI: 10.1002/ddrr.44
Source: PubMed

ABSTRACT Mathematical learning disabilities (MLD) occur frequently in children with specific genetic disorders, like Turner syndrome, fragile X syndrome and neurofibromatosis. This review focuses on MLD in children with chromosome 22q11.2 deletion syndrome (22q11DS). This syndrome is the most common known microdeletion syndrome with a prevalence of at least 1:4000 to 1:6000 live births. Although the clinical presentation of 22q11DS is quite variable, its major characteristics include velopharyngeal abnormalities, congenital cardiac anomalies, mild facial dysmorphism and learning difficulties. Children with 22q11DS show considerable difficulties in mathematics, despite relatively normal reading performance. While fact retrieval seems to be preserved, impairments in procedural calculation and word problem solving are particularly prominent. Children with 22q11DS also have substantial difficulties in understanding and representing numerical quantities, possibly related to poor visuospatial attention, which all might stem from their underlying abnormalities in the inferior parietal cortex. This review ends with a discussion on how research on genetic disorders might aid our understanding of MLD in general.

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    • "A closer examination of specific cognitive abilities reveals deficits in a number of domains, many of which are independent of IQ, including visualspatial processing, executive functioning, attention, verbal learning and working memory (Sobin et al. 2005; Lewandowski et al. 2007a). Decrements in cognitive ability can lead to poor academic performance in these children, and indeed, research has found notable deficits in arithmetic among children with 22q11DS, whereas reading and spelling skills are relatively stronger (Moss et al. 1999; Simon 2008; De Smedt et al. 2009). "
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    ABSTRACT: BACKGROUND: Children with 22q11.2 deletion syndrome (22q11DS) are at risk for social-behavioural and neurocognitive sequelae throughout development. The current study examined the impact of family environmental characteristics on social-behavioural and cognitive outcomes in this paediatric population. METHOD: Guardians of children with 22q11DS were recruited through two medical genetics clinics. Consenting guardians were asked to complete several questionnaires regarding their child's social, emotional and behavioural functioning, as well as family social environment and parenting styles. Children with 22q11DS were asked to undergo a cognitive assessment, including IQ and achievement testing, and measures of attention, executive function and memory. RESULTS: Modest associations were found between aspects of the family social environment and parenting styles with social-behavioural and cognitive/academic outcomes. Regression models indicated that physical punishment, socioeconomic status, parental control and family organisation significantly predicted social-behavioural and cognitive outcomes in children with 22q11DS. CONCLUSION: Characteristics of the family social environment and parenting approaches appear to be associated with functional outcomes of children with 22q11DS. Understanding the impact of environmental variables on developmental outcomes can be useful in determining more effective targets for intervention. This will be important in order to improve the quality of life of individuals affected by 22q11DS.
    Journal of Intellectual Disability Research 06/2013; 58(1). DOI:10.1111/jir.12054 · 2.41 Impact Factor
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    • "For example, children with 22q11.2DS, appear to be less impaired on number reading and number fact recall tasks, which are strongly verbal, but more impaired on arithmetic problems involving carrying across columns where visuospatial processing is required (De Smedt et al., 2009). An alternative account of magnitude comparison processing (Van Opstal, Gevers, De Moor, & Verguts, 2008) is based on the Verguts and Fias model mentioned earlier. "
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    ABSTRACT: Several neurodevelopmental disorders of known genetic etiology generate phenotypes that share the characteristic of numerical and mathematical cognitive impairments. This article reviews some of the main findings that suggest a possible key role that spatial and temporal information processing impairments may play in the atypical development of numerical cognitive competence. The question of what neural substrate might underlie these impairments is also addressed, as are the challenges for interpreting neural structure/cognitive function mapping in atypically developing populations.
    Developmental Neuropsychology 08/2011; 36(6):788-805. DOI:10.1080/87565641.2010.549879 · 2.67 Impact Factor
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    • "glish et al . [ 2009 ] report a mathematics profile in children with SBM that includes relative preservation of mechanical arithmetic skills , a cardinal deficit in the nonverbal learning disabilities framework . In 22q11DS , the characteristic nonverbal learning disabilities IQ pattern ( Verbal IQ > Performance IQ ) is attenuated or reversed [ De Smedt et al . , 2009 ] . For these and other disorders ( such as Turner syndrome ) , the framework fails to specify within - disorder specificity , much less to differentiate these disorders from each one ."
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    ABSTRACT: What is mathematical learning disability (MLD)? The reviews in this special issue adopt different approaches to defining the construct of MLD. Collectively, they demonstrate the current status of efforts to establish a consensus definition and the challenges faced in this endeavor. In this commentary, we reflect upon the proposed pathways to mathematical learning difficulties and disabilities presented across the reviews. Specifically we consider how each of the reviews contributes to identifying the MLD phenotype by specifying the range of assets and deficits in mathematics, identifying sources of individual variation, and characterizing the natural progression of MLD over the life course. We show how principled comparisons across disorders address issues about the cognitive and behavioral co-morbidities of MLD, and whether commonalities in brain dysmorphology are associated with common mathematics performance profiles. We project the status of MLD research ten years hence with respect to theoretical gains, advances in methodology, and principled intervention studies.
    Developmental Disabilities Research Reviews 01/2009; 15(1):80-9. DOI:10.1002/ddrr.54 · 0.29 Impact Factor
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