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Associations Between Executive Functioning, Behavioral Functioning, and Adaptive Functioning Difficulties in

Rowena Ng1,2, Hans Tomas Bjornsson3,4,5,6, Jill A Fahrner3,4

  • 1Kennedy Krieger Institute, Baltimore, MD, USA.

Archives of Clinical Neuropsychology : the Official Journal of the National Academy of Neuropsychologists
|August 11, 2023
PubMed
Summary
This summary is machine-generated.

Individuals with Wiedemann-Steiner syndrome (WSS) often experience executive functioning (EF) deficits, hyperactivity, and emotional dysregulation. Cognitive flexibility and emotional control are key factors influencing behavior and adaptive functioning in WSS patients.

Keywords:
Behavior regulationCognitive flexibilityEmotion regulationExecutive controlKMT2AWiedemann–Steiner syndrome

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Area of Science:

  • Neurogenetics
  • Neurodevelopmental Disorders
  • Behavioral Science

Background:

  • Wiedemann-Steiner syndrome (WSS) is a rare neurogenetic disorder linked to KMT2A gene variants.
  • Existing research suggests WSS may be associated with increased anxiety and behavioral regulation issues.
  • The comprehensive neurobehavioral phenotype of WSS remains incompletely understood.

Purpose of the Study:

  • To investigate the executive functioning (EF) profile in individuals with WSS.
  • To examine the relationship between executive functioning and behavioral/adaptive functioning in WSS.
  • To identify specific EF challenges that may predict behavioral and adaptive difficulties.

Main Methods:

  • Caregiver-report inventories were used: Behavior Rating Inventory of Executive Function 2nd Edition (BRIEF-2), Adaptive Behavior Assessment 3rd Edition (ABAS-3), and Strengths and Difficulties Questionnaire (SDQ).
  • The study included 24 individuals with WSS (mean age 10.68 years).
  • Statistical analyses included correlational and hierarchical linear regression to assess associations between EF, adaptive behaviors, and emotional regulation.

Main Results:

  • A significant proportion of individuals with WSS exhibited clinical difficulties in executive functioning (63% on BRIEF-2).
  • High rates of hyperactivity (75%) and emotional problems (54%) were reported on the SDQ.
  • Deficits in cognitive flexibility (BRIEF-2 Shift) predicted emotional problems, and difficulties in emotional control were linked to adaptive deficits.

Conclusions:

  • Individuals with WSS are at elevated risk for executive functioning deficits, hyperactivity, and emotional dysregulation.
  • Executive functioning, particularly cognitive flexibility and emotional control, significantly correlates with adaptive and affective behaviors.
  • Targeted behavioral interventions focusing on cognitive flexibility and emotional regulation show promise for improving outcomes in WSS.