Systematic Review: Emotion Dysregulation in Syndromic Causes of Intellectual and Developmental Disabilities

J Am Acad Child Adolesc Psychiatry. 2023 May;62(5):518-557. doi: 10.1016/j.jaac.2022.06.020. Epub 2022 Aug 22.


Objective: To summarize the current state of the literature regarding emotion dysregulation (ED) in syndromic intellectual disabilities (S-IDs) in 6 of the most common forms of S-IDs-Down syndrome, fragile X syndrome (FXS), tuberous sclerosis complex, Williams syndrome, Prader-Willi syndrome, and Angelman syndrome-and to determine future research directions for identification and treatment of ED.

Method: PubMed bibliographic database was searched from date of inception to May 2021. PRISMA 2020 guidelines were followed with the flowchart, table of included studies, list of excluded studies, and checklist provided. Filters applied included human research and English. Only original research articles were included in the final set, but review articles were used to identify secondary citations of primary studies. All articles were reviewed for appropriateness by 2 authors and summarized. Inclusion criteria were met by 145 articles (Down syndrome = 29, FXS = 55, tuberous sclerosis complex = 11, Williams syndrome = 18, Prader-Willi syndrome = 24, Angelman syndrome = 8).

Results: Each syndrome review was summarized separately and further subdivided into articles related to underlying neurobiology, behaviors associated with ED, assessment, and targeted intervention. FXS had the most thorough research base, followed by Down syndrome and Prader-Willi syndrome, with the other syndromes having more limited available research. Very limited research was available regarding intervention for all disorders except FXS.

Conclusion: Core underlying characteristics of S-IDs appear to place youth at higher risk for ED, but further research is needed to better assess and treat ED in S-IDs. Future studies should have a standard assessment measure of ED, such as the Emotion Dysregulation Inventory, and explore adapting established curricula for ED from the neurotypical and autism spectrum disorder fields.

Keywords: Down syndrome; Williams syndrome; emotion dysregulation; fragile X syndrome; intellectual disability.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Adolescent
  • Angelman Syndrome* / complications
  • Autism Spectrum Disorder* / complications
  • Child
  • Developmental Disabilities
  • Down Syndrome* / complications
  • Emotions
  • Fragile X Syndrome* / complications
  • Fragile X Syndrome* / psychology
  • Humans
  • Intellectual Disability* / etiology
  • Prader-Willi Syndrome* / complications
  • Prader-Willi Syndrome* / psychology
  • Tuberous Sclerosis* / complications
  • Williams Syndrome* / complications
  • Williams Syndrome* / psychology