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

Abstract

Children with one of two genetic disorders (chromosome 22q11.2 deletion syndrome and Turner syndrome) as well typically developing controls, participated in three cognitive processing experiments. Two experiments were designed to test cognitive processes involved in basic aspects numerical cognition. The third was a test of simple manual motor reaction time. Despite significant differences in global intellectual abilities, as measured by IQ tests, performance on the two numerical cognition tasks differed little between the two groups of children with genetic disorders. However, both performed significantly more poorly than did controls. The pattern of results are consistent with the hypothesis that impairments were not due to global intellectual ability but arose in specific cognitive functions required by different conditions within the tasks. The fact that no group differences were found in the reaction time task, despite significant differences in the standardized processing speed measure, further supports the interpretation that specific cognitive processing impairments and not global intellectual or processing speed impairments explain the pattern of results. The similarity in performance on these tasks of children with unrelated genetic disorders counters the view that numerical cognition is under any direct genetic control. Instead, our findings are consistent with the view that disturbances in foundational spatiotemporal cognitive functions contribute to the development of atypical representations and processes in the domains of basic magnitude comparison and simple numerical enumeration.

Figure 1

Example stimulus for the Enumeration Task.

Figure 2

Mean reaction time (+/−1SEM) for control, DS22q11.2 and 45,X TS groups for each numerosity in the Enumeration Task.

Figure 3

Example stimulus for the Distance Effect Task for both blocks and digits conditions.

Figure 4

Mean reaction time (+/−1SEM) for control, DS22q11.2 and 45,X TS groups on Enumeration Task both the blocks (A) and digits conditions (B).

Figure 4

Mean reaction time (+/−1SEM) for control, DS22q11.2 and 45,X TS groups on Enumeration Task both the blocks (A) and digits conditions (B).