Elsevier

Cortex

Volume 41, Issue 2, 2005, Pages 145-155
Cortex

Visuospatial and Numerical Cognitive Deficits in Children with Chromosome 22Q11.2 Deletion Syndrome

https://doi.org/10.1016/S0010-9452(08)70889-XGet rights and content

Abstract

This article presents some of the earliest evidence of visuospatial and numerical cognitive deficits in children with the chromosome 22q11.2 deletion syndrome; a common but ill-understood genetic disorder resulting in medical complications, cognitive impairment, and brain morphologic changes. Relative to a group of typically developing controls, deleted children performed more poorly on tests of visual attentional orienting, visual enumeration and relative numerical magnitude judgment. Results showed that performance deficits in children with the deletion could not be explained by a global deficit in psychomotor speed. Instead, our findings are supportive of the hypothesis that visuospatial and numerical deficits in children with the chromosome 22q11.2 deletion are due, at least in part, to posterior parietal dysfunction.

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      A subgroup of primary school-aged children with 22q11DS shows significantly better verbal (VIQ) than visuospatial abilities (i.e., performance intelligence, PIQ) (Bearden et al., 2001; De Smedt et al., 2007; Swillen et al., 1999). Despite (severe) limitations in visuospatial skills (Bearden et al., 2001; Swillen et al., 2000; Woodin et al., 2001), processing speed (PS) has been reported to be least impaired (Campbell et al., 2009; Simon et al., 2005) on intelligence tests. Goldenberg et al. (2012) and Gur et al. (2014) demonstrated no significant difference for cognitive flexibility and non-verbal reasoning between children with 22q11DS and chronological age-matched typically developing (TD) children.

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      With regard to their mathematical abilities, all studies highlighted very poor performance in general standardized achievement for children with 22q11DS compared to typically developing children (Moss et al., 1999; Wang et al., 2000; Woodin et al., 2001). More in-depth studies provide evidence for poorer calculation abilities compared to control participants matched on age and IQ, especially in calculation tasks requiring the deployment of calculation procedures such as addition and subtraction with a carry, and multi-digit calculations (De Smedt et al., 2006, 2007b, 2008, 2009; Simon et al., 2005). Furthermore, despite demonstrating preserved transcoding abilities (De Smedt et al., 2006, 2007b, 2009) individuals with this disorder often displayed difficulties in the counting range in a dot numerical estimation task (3–8 dots, Simon et al., 2005, 2008).

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