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The cognitive developmental profile associated with fragile X syndrome: A longitudinal investigation of cognitive strengths and weaknesses through childhood and adolescence

Published online by Cambridge University Press:  09 December 2015

Eve-Marie Quintin ,
Booil Jo ,
Scott S. Hall ,
Jennifer L. Bruno ,
Lindsay C. Chromik ,
Mira M. Raman ,
Amy A. Lightbody ,
Arianna Martin  and
Allan L. Reiss
Eve-Marie Quintin*
Affiliation:
McGill University
Booil Jo
Affiliation:
Stanford University
Scott S. Hall
Affiliation:
Stanford University
Jennifer L. Bruno
Affiliation:
Stanford University
Lindsay C. Chromik
Affiliation:
Stanford University
Mira M. Raman
Affiliation:
Stanford University
Amy A. Lightbody
Affiliation:
Stanford University
Arianna Martin
Affiliation:
Stanford University
Allan L. Reiss
Affiliation:
Stanford University
*
Address correspondence and reprint requests to: Eve-Marie Quintin, Educational and Counseling Psychology Department, McGill University, 3700 McTavish Street, Montreal, QCH3A 1Y2, Canada; E-mail: eve-marie.quintin@mcgill.ca.
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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.

Type
Regular Articles
Information
Development and Psychopathology , Volume 28 , Special Issue 4pt2: Epigenetics: Development, Psychopathology, Resilience, and Preventive Intervention , November 2016 , pp. 1457 - 1469
Copyright
Copyright © Cambridge University Press 2015 

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