Functional brain imaging study of mathematical reasoning abilities in velocardiofacial syndrome (del22q11.2)

Abstract

Purpose: Children with velocardiofacial syndrome (VCFS) often have deficits in mathematical reasoning. Previous research has suggested that structural abnormalities in the parietal lobe region might underlie these deficits. The present study utilized functional magnetic resonance imaging (fMRI) to explore the relationship between brain function and mathematical performance in VCFS. Methods: Eight children with VCFS and eight comparison subjects underwent fMRI scanning and completed an arithmetic computation task. Results: In the VCFS group, increased activation was observed in the left supramarginal gyrus (LSMG) as the task difficulty increased. Conclusion: Persons with velocardiofacial syndrome (VCFS) have cognitive deficits and typically score in the borderline to mildly mentally retarded range on standardized tests of general cognitive ability. [1][2][3][4][5] In addition to general cognitive disability, children with VCFS also show more specific deficiencies in the area of mathematics ability.The first descriptive study of the cognitive phenotype associated with VCFS 2 showed that children with this condition scored disproportionately lower on math and reading comprehension tests relative to tests of language. For example, an early study demonstrated that math scores from the Wide Range Achievement Test (WRAT) among 6 -11 year olds with VCFS ranged from 81 to 90 (population mean ϭ 100, SD ϭ 10) and from 74 to 86 among affected adolescents. 2 More recent investigations have yielded similar results. 3,6,7 General weaknesses in problem solving and manipulation of numbers have been observed among school-age children with VCFS, as well as particular difficulties in converting language into number sentences, solving problems with decimals and fractions, telling time, and converting money. 6 When examining standardized test scores in clinical samples of 33 subjects with VCFS, Moss et al. 3 reported lower composite math achievement scores relative to composite scores of reading and spelling. In accordance with these findings, Swillen et al. 7 recently reported that children with VCFS had significant decrements in arithmetic performance alongside relatively preserved abilities in reading and spelling.Although the neural substrates that underlie deficiencies in arithmetic reasoning in VCFS are currently unknown, research focused on individuals with neurological dysfunction from other causes has begun to define the specific brain regions that are involved in mathematical performance. For example, human brain lesion studies have demonstrated acalculia among patients with lesions to the parietal cortex. Further, the parietal area is thought to underlie the acalculia component of Gerstmann's syndrome. 8 -13 Functional magnetic resonance imaging (fMRI) studies with "normal" subjects also have implicated several brain regions as being associated with arithmetic performance. 14,15 During "approximate" computation, brain activation has been observed in the bilateral inferior parietal lobule, right precuneus, bilateral prece...

A multilevel analysis of cognitive dysfunction and psychopathology associated with chromosome 22q11.2 deletion syndrome in children

“…Thus, our findings replicated most of those previously reported, which include volume reductions in occipital, parietal, temporal and cerebellar regions (e.g., Eliez, Blasey, Menon, White, Schmitt, & Reiss, 2001;Eliez, Blasey, Schmitt, White, Hu, & Reiss, 2001;Eliez, Schmitt, White, & Reiss, 2000;Kates et al, 2001). In our sample, the gray matter analysis showed that the most extensive areas of reduced volume in the brains of children with DS22q11.2 were found in regions from the anterior aspects of the medial cerebellum through the parahippocampal, fusiform, and lingual regions, up through the cuneus, precuneus, posterior corpus callosum, posterior parietal lobes, and following the cingulum from posterior to a small medial region of its anterior aspect.…”

The neurocognitive phenotype in velo‐cardio‐facial syndrome: A developmental perspective

“…Multiple independent groups have reported that youth with VCFS have difficulty in visual spatial problem solving [Swillen et al, 1999;Bearden et al, 2001;Lajiness-O'Neill et al, 2005], and these deficits are most likely due to the alterations in parietal lobe gray/white volumes [Eliez et al, 2000b;Kates et al, 2001] and frontal-parietal connectivity [Barnea-Goraly et al, 2003] that have been described. For example, Eliez et al [2001] conducted a fMRI study of math reasoning in eight children with VCFS. Their results indicate that children with VCFS showed less activation than did controls in the angular gyrus, a posterior parietal brain region typically activated during such tasks.…”

“…In addition, increased activation was observed in the left supramarginal gyrus as a function of task difficulty. Eliez et al [2001] concluded that youth with VCFS may utilize an atypical and less effective circuit for completing math problems.…”

Overlapping numerical cognition impairments in children with chromosome 22q11.2 deletion or Turner syndromes

“…Although not exclusively the case, recent research has focused on explicitly computational aspects of the numerical domain, namely arithmetic and simple mathematics. Such tasks have been employed in order to understand the neural and, to some extent, the genetic basis of the problems (Eliez et al, 2001;Kesler, Menon, & Reiss, 2006;Molko, Cachia, Riviere, Mangin, Bruandet, LeBihan, Cohen, & Dehaene, 2004;Rivera, Menon, White, Glaser, & Reiss, 2002).…”