title: Executive functions in young males with fragile X syndrome in comparison to mental age-matched controls: baseline findings from a longitudinal study.
abstract: The performance of 54 boys with fragile X syndrome (FXS), ages 7 to 13 years, was compared to that of a group of typically developing boys who were matched on mental age (MA) and ethnicity across multiple measures of executive function (EF). Boys with FXS varied in their ability to complete EF measures, with only 25.9% being able to complete a set-shifting task and 94.4% being able to complete a memory for word span task. When compared to the control group, and controlling for MA and maternal education, boys with FXS showed significant deficits in inhibition, working memory, cognitive flexibility/set-shifting, and planning. No group differences were observed in processing speed. Mental age significantly impacted performance on working memory, set-shifting, planning, and processing speed tasks for both groups. In boys with FXS, MA significantly predicted performance on working memory and set-shifting tasks. Our findings suggest that deficits in EF in boys with FXS are not solely attributable to developmental delays but, rather, present as a true array of neurocognitive deficits.
authors.affiliation: Department of Psychiatry, University of North Carolina School of Medicine, Chapel Hill, NC27599-7255, USA. stephan.hooper@cdl.unc.edu
authors.forename: Stephen R
authors.initials: SR
authors.lastname: Hooper
authors.forename: Deborah
authors.initials: D
authors.lastname: Hatton
authors.forename: John
authors.initials: J
authors.lastname: Sideris
authors.forename: Kelly
authors.initials: K
authors.lastname: Sullivan
authors.forename: Julie
authors.initials: J
authors.lastname: Hammer
authors.forename: Jennifer
authors.initials: J
authors.lastname: Schaaf
authors.forename: Penny
authors.initials: P
authors.lastname: Mirrett
authors.forename: Peter A
authors.initials: PA
authors.lastname: Ornstein
authors.forename: Donald P
authors.initials: DP
authors.lastname: Bailey
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D005600:Fragile X Syndrome / Q000503:physiopathology*; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D009483:Neuropsychological Tests; D011237:Predictive Value of Tests; D011340:Problem Solving / Q000502:physiology*