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Wiley Interdiscip Rev Cogn Sci. 2014 Jul;5(4):501-508. doi: 10.1002/wcs.1296. Epub 2014 May 29.

Cognitive aspects of Fragile X syndrome.

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1
Department of Human Genetics, Emory University, Decatur, GA, USA.

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. WIREs Cogn Sci 2014, 5:501-508. doi: 10.1002/wcs.1296 This article is categorized under: Neuroscience > Cognition.

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