Anxiety in Wiedemann-Steiner syndrome

Am J Med Genet A. 2023 Feb;191(2):437-444. doi: 10.1002/ajmg.a.63040. Epub 2022 Nov 14.


This study examined anxiety in Wiedemann-Steiner syndrome (WSS). Eighteen caregivers and participants with WSS completed the parent- and self-report versions of the Screen for Child Anxiety Related Disorder or the adapted version of the Screen for Adult Anxiety Related Disorder. Approximately 33.33% of parents and 65% of participants with WSS rated in the clinical range for overall anxiety. Across anxiety subtypes, parents primarily indicated concerns with Separation Anxiety (72%), which was also endorsed by the majority of participants with WSS (82%). The emergent trend showed Total Anxiety increased with age based on parent-informant ratings. The behavioral phenotype of WSS includes elevated anxiety. Clinical management should include incorporating early behavioral interventions to bolster emotion regulation given the observed risk of anxiety with age.

Keywords: KMT2A; anxiety; developmental psychopathology; genetics/genetic disorders; mental health.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Abnormalities, Multiple* / genetics
  • Anxiety
  • Humans
  • Intellectual Disability* / genetics

Supplementary concepts

  • Wiedemann Grosse Dibbern syndrome
  • Growth Deficiency and Mental Retardation with Facial Dysmorphism