Executive Function in Fragile X Syndrome: A Systematic Review

Executive function (EF) supports goal-directed behavior and includes key aspects such as working memory, inhibitory control, cognitive flexibility, attention, processing speed, and planning. Fragile X syndrome (FXS) is the leading inherited monogenic cause of intellectual disability and is phenotypi...

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Main Authors: Lauren M. Schmitt, Rebecca C. Shaffer, David Hessl, Craig Erickson
Format: Article
Language:English
Published: MDPI AG 2019-01-01
Series:Brain Sciences
Subjects:
Online Access:http://www.mdpi.com/2076-3425/9/1/15
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author Lauren M. Schmitt
Rebecca C. Shaffer
David Hessl
Craig Erickson
author_facet Lauren M. Schmitt
Rebecca C. Shaffer
David Hessl
Craig Erickson
author_sort Lauren M. Schmitt
collection DOAJ
description Executive function (EF) supports goal-directed behavior and includes key aspects such as working memory, inhibitory control, cognitive flexibility, attention, processing speed, and planning. Fragile X syndrome (FXS) is the leading inherited monogenic cause of intellectual disability and is phenotypically characterized by EF deficits beyond what is expected given general cognitive impairments. Yet, a systematic review of behavioral studies using performance-based measures is needed to provide a summary of EF deficits across domains in males and females with FXS, discuss clinical and biological correlates of these EF deficits, identify critical limitations in available research, and offer suggestions for future studies in this area. Ultimately, this review aims to advance our understanding of the underlying pathophysiological mechanisms contributing to EF in FXS and to inform the development of outcome measures of EF and identification of new treatment targets in FXS.
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spelling doaj.art-7e9261c758874153a3bede3fdb83e32c2022-12-22T01:02:55ZengMDPI AGBrain Sciences2076-34252019-01-01911510.3390/brainsci9010015brainsci9010015Executive Function in Fragile X Syndrome: A Systematic ReviewLauren M. Schmitt0Rebecca C. Shaffer1David Hessl2Craig Erickson3Division of Child and Adolescent Psychiatry, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH 45229, USADivision of Developmental and Behavioral Pediatrics, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH 45229, USAMIND Institute, Department of Psychiatry and Behavioral Sciences, University of California, Davis, CA 95616, USADivision of Child and Adolescent Psychiatry, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH 45229, USAExecutive function (EF) supports goal-directed behavior and includes key aspects such as working memory, inhibitory control, cognitive flexibility, attention, processing speed, and planning. Fragile X syndrome (FXS) is the leading inherited monogenic cause of intellectual disability and is phenotypically characterized by EF deficits beyond what is expected given general cognitive impairments. Yet, a systematic review of behavioral studies using performance-based measures is needed to provide a summary of EF deficits across domains in males and females with FXS, discuss clinical and biological correlates of these EF deficits, identify critical limitations in available research, and offer suggestions for future studies in this area. Ultimately, this review aims to advance our understanding of the underlying pathophysiological mechanisms contributing to EF in FXS and to inform the development of outcome measures of EF and identification of new treatment targets in FXS.http://www.mdpi.com/2076-3425/9/1/15fragile X syndromeexecutive functionworking memoryset-shiftingcognitive flexibilityinhibitory controlattentionplanningprocessing speed
spellingShingle Lauren M. Schmitt
Rebecca C. Shaffer
David Hessl
Craig Erickson
Executive Function in Fragile X Syndrome: A Systematic Review
Brain Sciences
fragile X syndrome
executive function
working memory
set-shifting
cognitive flexibility
inhibitory control
attention
planning
processing speed
title Executive Function in Fragile X Syndrome: A Systematic Review
title_full Executive Function in Fragile X Syndrome: A Systematic Review
title_fullStr Executive Function in Fragile X Syndrome: A Systematic Review
title_full_unstemmed Executive Function in Fragile X Syndrome: A Systematic Review
title_short Executive Function in Fragile X Syndrome: A Systematic Review
title_sort executive function in fragile x syndrome a systematic review
topic fragile X syndrome
executive function
working memory
set-shifting
cognitive flexibility
inhibitory control
attention
planning
processing speed
url http://www.mdpi.com/2076-3425/9/1/15
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