Journal Article Annotations
2023, 2nd Quarter

Child and adolescent psychiatry/pediatrics

Annotations by Sneha Jadhav, MD
July, 2023

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

PUBLICATION #1 — Child and adolescent psychiatry/pediatrics

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

Rebecca C Shaffer, Debra L Reisinger, Lauren M Schmitt, Martine Lamy, Kelli C Dominick, Elizabeth G Smith, Marika C Coffman, Anna J Esbensen

Abstract: 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.

Annotation

The finding:
For each of 6 conditions— Down syndrome, fragile X syndrome, tuberous sclerosis, Williams syndrome, Angelman syndrome, and Prader-Willi syndrome—this article describes the qualities, prognosis, and treatment of emotional dysregulation syndromes. While ED in some conditions may be better managed by specific pharmacotherapy—eg, guanfacine decreases irritability in Down Syndrome—for other conditions there may be greater benefit from treatment of comorbidities (as with treating attention-deficit hyperactivity disorder among patients with Williams syndrome) or psychotherapy (as with mindfulness for patients with Prader-Willi syndrome).

Strength and weaknesses:
The authors applied a reproducible methodology that emphasized peer-reviewed literature. This improve the validity of treatment recommendations, but gray literature from patients, families, and caregivers may have added a helpful perspective. The breadth of the literature review adds to the clinical value of the review, but there is no quality review of articles included. There is little discussion of health disparities and social determinants impacting these patients’ care, even as patients’ care is so frequently impacted by systemic neurodiscrimination.

Relevance:
Emotional dysregulation among youth and adults with intellectual disabilities are impairing symptoms that complicate daily living, social recovery, and treatment while in medical settings. C-L psychiatrists should understand the common behavioral symptoms associated with these conditions and their evidence-based interventions while providing education to family, caregivers, and medical teams. Not only can these treatments improve patients’ well-being, they also reduce the risk of iatrogenic harm from overprescribing for ED and empower caregivers.