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. 2002 Aug;16(4):206-18.
doi: 10.1002/hbm.10048.

Functional brain activation during arithmetic processing in females with fragile X Syndrome is related to FMR1 protein expression

Susan M Rivera  1 , Vinod Menon, Christopher D White, Bronwyn Glaser, Allan L Reiss
Affiliations

Functional brain activation during arithmetic processing in females with fragile X Syndrome is related to FMR1 protein expression

Susan M Rivera et al. Hum Brain Mapp. 2002 Aug.

Abstract

Arithmetic processing deficits in persons with fragile X Syndrome (fraX), the most common heritable cause of mental retardation, are well known. In this study, we characterize the neural underpinnings of these performance deficits using functional MRI. Given that a single gene defect (FMR1) is known to be responsible for this disorder, we also assess whether brain activation in arithmetic processing areas is related to amount of FMR1 protein expression (FMRP). Subjects included 16 females with fraX, and 16 female age-matched controls. Subjects viewed arithmetic equations with two (1 + 3 = 4) or three (2 + 3 - 1 = 5) operands, and were asked to judge whether the results were correct or not. Subjects with fraX showed significant impairment in behavioral performance on the 3-operand but not the 2-operand arithmetic equations. Significant brain activation was observed bilaterally in the prefrontal and parietal cortices for unaffected subjects, and bilateral prefrontal and left angular gyrus for subjects with fraX, for both trial types. Subjects with fraX exhibited less overall activation than did unaffected subjects in both types of trials; and, unlike the unaffected group, did not show increased extent of activation in association with greater task difficulty. During the 3-operand trials, activation in bilateral prefrontal and motor/premotor, and left supramarginal and angular gyri were positively correlated with FMRP, suggesting that decreased FMR1 protein expression underlies deficits in math performance in persons with fraX. More broadly, this investigation demonstrates a unique bridging of cognitive and molecular neuroscience and represents a useful approach for the study of brain development and function.

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Figures

Figure 1
Figure 1
Percent correct on 2‐ and 3‐operand arithmetic equations for unaffected subjects and subjects with fraX.
Figure 2
Figure 2
Brain areas that show significantly greater activation during 2‐operand arithmetic equations for (a) unaffected subjects and (b) subjects with fraX; and during 3‐operand arithmetic equations for (c) unaffected subjects and (d) subjects with fraX. All activations reported were significant after height (Z > 2.33; P < 0.01) and extent (P < 0.01) thresholding.
Figure 3
Figure 3
Brain areas that show significantly greater activation for unaffected subjects than subjects with fraX during 3‐operand, compared to 2‐operand arithmetic equations.
Figure 4
Figure 4
Brain areas that show, for subjects with fraX, a significant correlation between FMRP and brain activation for (a) 2‐operand and (b) 3‐operand arithmetic equations.
See this image and copyright information in PMC

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