title: The cognitive developmental profile associated with fragile X syndrome: A longitudinal investigation of cognitive strengths and weaknesses through childhood and adolescence.
abstract: Few studies have investigated developmental strengths and weaknesses within the cognitive profile of children and adolescents with fragile X syndrome (FXS), a single-gene cause of inherited intellectual impairment. With a prospective longitudinal design and using normalized raw scores (Z scores) to circumvent floor effects, we measured cognitive functioning of 184 children and adolescents with FXS (ages 6 to 16) using the Wechsler Scale of Intelligence for Children on one to three occasions for each participant. Participants with FXS received lower raw scores relative to the Wechsler Scale of Intelligence for Children normative sample across the developmental period. Verbal comprehension, perceptual organization, and processing speed Z scores were marked by a widening gap from the normative sample, while freedom from distractibility Z scores showed a narrowing gap. Key findings include a relative strength for verbal skills in comparison with visuospatial-constructive skills arising in adolescence and a discrepancy between working memory (weakness) and processing speed (strength) in childhood that diminishes in adolescence. Results suggest that the cognitive profile associated with FXS develops dynamically from childhood to adolescence. Findings are discussed within the context of aberrant brain morphology in childhood and maturation in adolescence. We argue that assessing disorder-specific cognitive developmental profiles will benefit future disorder-specific treatment research.
authors.affiliation: McGill University.
authors.forename: Eve-Marie
authors.initials: EM
authors.lastname: Quintin
authors.affiliation: Stanford University.
authors.forename: Booil
authors.initials: B
authors.lastname: Jo
authors.affiliation: Stanford University.
authors.forename: Scott S
authors.initials: SS
authors.lastname: Hall
authors.affiliation: Stanford University.
authors.forename: Jennifer L
authors.initials: JL
authors.lastname: Bruno
authors.affiliation: Stanford University.
authors.forename: Lindsay C
authors.initials: LC
authors.lastname: Chromik
authors.affiliation: Stanford University.
authors.forename: Mira M
authors.initials: MM
authors.lastname: Raman
authors.affiliation: Stanford University.
authors.forename: Amy A
authors.initials: AA
authors.lastname: Lightbody
authors.affiliation: Stanford University.
authors.forename: Arianna
authors.initials: A
authors.lastname: Martin
authors.affiliation: Stanford University.
authors.forename: Allan L
authors.initials: AL
authors.lastname: Reiss
mesh_terms: D000293:Adolescent; D041923:Adolescent Development / Q000502:physiology*; D002648:Child; D002657:Child Development / Q000502:physiology*; D003071:Cognition / Q000502:physiology*; D003072:Cognition Disorders / Q000523:psychology; D032882:Comprehension / Q000502:physiology; D005260:Female; D005600:Fragile X Syndrome / Q000523:psychology*; D006801:Humans; D007360:Intelligence / Q000502:physiology*; D008297:Male; D008570:Memory, Short-Term / Q000502:physiology; D009483:Neuropsychological Tests; D011446:Prospective Studies; D014888:Wechsler Scales