Journal Article Annotations
2023, 2nd Quarter
Annotations by Sneha Jadhav, MD
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.
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.