Behavior phenotype of FG syndrome: Cognition, personality, and behavior ineleven affected boys

In this study we examined several behavioral, personality, and cognitive ch aracteristics of boys with FC syndrome. We confirmed high rates of attentio n and activity level problems, which were described previously. Nine of the 11 patients met criteria for attention deficit/hyperactivity disorder. The boys did not manifest autistic behavior, and none met criteria for an auti sm spectrum disorder, though their parents reported substantial repetitive behavior. The personalities of the participants often were described as fri endly, good-natured, and cheerful, but they did not differ empirically on a standardized measure of personality structure from typically developing co mparison children, even after controlling for the effects of IQ Specificall y, higher rates of agreeableness and extraversion were not confirmed, thoug h these constructs do not correspond perfectly with the traits of affabilit y and gregariousness described in earlier published case studies of FC synd rome. In terms of neuropsychological assessment, the boys had relatively le ss developed language, fine motor, and executive function skills, and visua l-spatial abilities were a relative strength. Limitations and suggestions f or future research are discussed. (C) 2000 Wiley-Liss, Inc.