title: Behavior phenotype of FG syndrome: cognition, personality, and behavior in eleven affected boys.
abstract: In this study we examined several behavioral, personality, and cognitive characteristics of boys with FG syndrome. We confirmed high rates of attention 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 autism spectrum disorder, though their parents reported substantial repetitive behavior. The personalities of the participants often were described as friendly, good-natured, and cheerful, but they did not differ empirically on a standardized measure of personality structure from typically developing comparison children, even after controlling for the effects of IQ. Specifically, higher rates of agreeableness and extraversion were not confirmed, though these constructs do not correspond perfectly with the traits of affability and gregariousness described in earlier published case studies of FG syndrome. In terms of neuropsychological assessment, the boys had relatively less developed language, fine motor, and executive function skills, and visual-spatial abilities were a relative strength. Limitations and suggestions for future research are discussed.
authors.affiliation: Department of Psychology, University of Utah, Salt Lake City 84112-0251, USA. sally.ozonoff@hsc.utah.edu
authors.forename: S
authors.initials: S
authors.lastname: Ozonoff
authors.forename: B J
authors.initials: BJ
authors.lastname: Williams
authors.forename: A M
authors.initials: AM
authors.lastname: Rauch
authors.forename: J O
authors.initials: JO
authors.lastname: Opitz
mesh_terms: D000293:Adolescent; D001289:Attention Deficit Disorder with Hyperactivity / Q000235:genetics / Q000523:psychology; D001321:Autistic Disorder / Q000235:genetics / Q000523:psychology; D001526:Behavioral Symptoms / Q000235:genetics* / Q000523:psychology*; D002648:Child; D003071:Cognition; D008040:Genetic Linkage; D006801:Humans; D008607:Intellectual Disability / Q000235:genetics / Q000523:psychology; D007361:Intelligence Tests; D007804:Language Development; D008297:Male; D009048:Motor Skills; D010552:Personality Assessment; D010641:Phenotype; D019956:Stereotypic Movement Disorder / Q000235:genetics / Q000523:psychology; D013577:Syndrome; D014960:X Chromosome / Q000235:genetics