Associations Between Executive Functioning, Behavioral Functioning, and Adaptive Functioning Difficulties in Wiedemann–Steiner Syndrome

Author:

Ng Rowena12ORCID,Bjornsson Hans Tomas3456,Fahrner Jill A34,Harris Jacqueline1347

Affiliation:

1. Kennedy Krieger Institute , Baltimore, MD , USA

2. Johns Hopkins University School of Medicine Department of Psychiatry and Behavioral Sciences, , Baltimore, MD , USA

3. Johns Hopkins University School of Medicine Department of Genetic Medicine, , Baltimore, MD , USA

4. Johns Hopkins University School of Medicine Department of Pediatrics, , Baltimore, MD , USA

5. University of Iceland Faculty of Medicine, , Reykjavik , Iceland

6. Landspitali University Hospital , Reykjavik , Iceland

7. Johns Hopkins University School of Medicine Department of Neurology, , Baltimore, MD , USA

Abstract

AbstractObjectivesWiedemann–Steiner syndrome (WSS) is a neurogenetic disorder caused by heterozygous variants in KMT2A. Recent investigations suggest increased anxiety and behavior regulation challenges among those with WSS although the neurobehavioral phenotype remains largely unknown. This study aims to examine the pattern of and associations between executive functioning (EF) and behavior functioning among those with WSS.MethodThis study involved utilizing caregiver-report inventories (Behavior Rating Inventory of Executive Function 2nd Edition, BRIEF-2; Adaptive Behavior Assessment 3rd Edition, ABAS-3; Strengths and Difficulties Questionnaire, SDQ) to assess day-to-day behavior functioning among those with WSS (N = 24; mean age = 10.68 years, SD = 3.19). Frequency of clinical elevations in daily difficulties in EF, adaptive behaviors, and behavior regulation were reported. Correlations and hierarchical linear regressions were used to determine the relationships between EF with behavior and adaptive functioning.ResultsOut of our sample, 63% met clinical levels of executive functioning difficulties on the BRIEF-2, and 75% with Hyperactivity and 54% with Emotional Problems on the SDQ. In addition, 33% were rated >2 SD below the normative mean in overall adaptive functioning on the ABAS-3. Elevated ratings in BRIEF-2 Shift, reflective of challenges with mental flexibility, predicted more Emotional Problems and accounted for 33.5% of its variance. More difficulties in Emotional Control were related to greater adaptive deficits, accounting for 33.3% of its variance.ConclusionsThose with WSS are at risk for EF deficits, hyperactivity, and emotional dysregulation. EF correlates with adaptive and affective behaviors, highlighting the promise of behavioral interventions to target cognitive flexibility, emotional awareness, and reactivity in this population.

Funder

ICTR

Maryland Stem Cell Research Fund

National Institute of Child Health and Human Development

Publisher

Oxford University Press (OUP)

Subject

Psychiatry and Mental health,Clinical Psychology,Neuropsychology and Physiological Psychology,General Medicine

Reference45 articles.

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