Cognitive aspects of Fragile X syndrome

• Type of Material: Article
• Authors: Lillie B. Huddleston, Emory University; Jeannie Visootsak, Emory University; Stephanie Sherman, Emory University
• Language: English
• Date: 2014-07-01
• Publisher: Wiley: 12 months
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2014 John Wiley & Sons, Ltd.
• Final Published Version (URL): http://dx.doi.org/10.1002/wcs.1296
• Title of Journal or Parent Work: Wiley Interdisciplinary Reviews: Cognitive Science
• ISSN: 1939-5078
• Volume: 5
• Issue: 4
• Start Page: 501
• End Page: 508
• Keywords: PROFILES; EXECUTIVE FUNCTIONS; Psychology; PERFORMANCE; FMR1; BOYS; MALES; Social Sciences; CHILDREN; INVOLVEMENT; EXPRESSION; Psychology, Experimental; PREVALENCE
• Research Categories: Psychology, Cognitive; Biology, Genetics

Abstract

Fragile X syndrome (FXS) is the leading inherited cause of intellectual disability. It is primarily caused by the expansion of a CGG trinucleodide repeat located in the 5′ untranslated region of the X-linked FMR1 gene. Individuals with FXS present with variable intellectual quotients (IQs) ranging from the average to the severe intellectual disability level. A range of neurocognitive strengths and challenges are observed in individuals with FXS. This article provides an overview of our current understanding related to cognition and FXS. Cognitive functioning levels, profiles, and IQ trajectories are discussed. Limitations of existing neuropsychological measures are described.