Executive Function in Fragile X Syndrome: A Systematic Review - PubMed (original) (raw)

Review

Executive Function in Fragile X Syndrome: A Systematic Review

Lauren M Schmitt et al. Brain Sci. 2019.

Abstract

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.

Keywords: attention; cognitive flexibility; executive function; fragile X syndrome; inhibitory control; planning; processing speed; set-shifting; working memory.

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Conflict of interest statement

R.C.S. receives funding from Fulcrum Therapeutics. D.H receives compensation for consulting to Zynerba, Autifony, and Ovid pharmaceutical companies regarding Fragile X clinical trials. C.E. has received current or past funding from Confluence Pharmaceuticals, Novartis, F. Hoffmann-La Roche Ltd., Seaside Therapeutics, Riovant Sciences, Inc., Fulcrum Therapeutics, Neuren Pharmaceuticals Ltd., Alcobra Pharmaceuticals, Neurotrope, Zynerba Pharmaceuticals, Inc., and Ovid Therapeutics Inc. to consult on trial design or development strategies and/or conduct clinical trials in FXS or other neurodevelopmental disorders. C.E. is additionally the inventor or co-inventor on several patents held by Cincinnati Children’s Hospital Medical Center or Indiana University School of Medicine describing methods of treatment in FXS or other neurodevelopmental disorders. Funding source had no role in the conceptualization of the review, the writing of the manuscript, or in the decision to publish the results.

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