Neurobehavioral correlates of CGG amplification were studied in 17 nonretarded adult female carriers of fragile X syndrome. The results revealed a significant relationship between IQ and the number of CGG repeats in the 5' untranslated region of the FMR1 gene. Women with a full mutation (> 200 CGG repeats) scored below average in IQ, visual-spatial perception, visual-spatial organization, and executive function. There were no differences in fine motor dexterity or memory as a function of CGG amplification status. A history of major depressive disorder was identified in 71% of the sample, but incidence of depression was not associated with the degree of CGG amplification. Schizotypal features were noted in 18%. No intellectual or neuropsychological deficit was found in women with a premutation (< 200 CGG repeats). Decrements in IQ, visual-spatial perception, and executive function appear to arise as a consequence of the CGG amplification.
Quay (1988) put forward a model of childhood mental disorders based on Gray's (1982) theory that there exists within the brain a behavioral inhibition system (BIS), which processes signals related to aversive or punishing stimuli. According to this model, children with attention deficit hyperactivity disorder (ADHD) show lower than optimal levels of activity in this system, which leads to less responsiveness at a physiological level to signals related to punishment. Children with ADHD and controls were compared on a classical conditioning paradigm. Skin conductance and cardiac responses were measured in response to a conditioned stimulus that had been paired with an aversive unconditioned stimulus. There were no differences between the groups, suggesting that, in terms of classical conditioning, ADHD children are equally responsive to signals related to punishment as controls.
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