Language in young females with fragile X syndrome: Influence on the neurocognitive profile and adaptive behavior

Am J Med Genet A. 2021 May;185(5):1448-1460. doi: 10.1002/ajmg.a.62130. Epub 2021 Feb 18.

Abstract

Fragile X syndrome (FXS) is the leading cause of inherited intellectual disability. The objective of this research is to analyze the relationship between linguistic functions and performance of the following neuropsychological functions: executive, quantitative reasoning, social perception, behavior, social skills, and adaptive behavior. A neuropsychological and behavioral evaluations were carried out with a group of 26 girls with FXS, and 14 girls without FXS as a control group, using standardized tests. The two groups were homogeneous in age and IQ. Significant differences were found between groups in the relationship between some language processes: inhibition, auditory working memory, cognitive flexibility, level of social adaptation, self-direction, conceptual adaptation, academic skills, leadership ability, theory of mind, and arithmetic. In the group of girls with FXS, it was found that different aspects of language influence some of the executive functions evaluated, in addition to some specific aspects of social perception, adaptive behavior, and quantitative reasoning, in different ways. Future research should incorporate the study of the influence of other cognitive variables such as visual perception and executive function on behavioral, social, and adaptive aspects to know the real influence of all the cognitive variables on the behavior of girls with FXS.

Keywords: behavior; cognition; females; fragile-X syndrome; language.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adaptation, Psychological / physiology*
  • Adolescent
  • Child
  • Female
  • Fragile X Syndrome / complications
  • Fragile X Syndrome / epidemiology
  • Fragile X Syndrome / genetics
  • Fragile X Syndrome / physiopathology*
  • Humans
  • Intellectual Disability / complications
  • Intellectual Disability / epidemiology
  • Intellectual Disability / genetics
  • Intellectual Disability / physiopathology*
  • Social Behavior
  • Social Perception / psychology
  • Social Skills
  • Speech-Language Pathology*