Psychiatric Intervention in a Pre-adolescent Patient With Wiedemann-Steiner Syndrome: A Case Report

Wiedemann-Steiner syndrome (WSS) is a rare genetic condition presenting with a classic triad of intellectual disability, developmental delay, and characteristic facial features, as well as various associated conditions.Patients are prone to episodes of aggressive behavior, sleep disturbances, and comorbid psychiatric conditions.We present a case of an eight-year-old girl admitted to inpatient psychiatric care following ongoing aggression and violent outbursts.She has a monoallelic mutation in KMT2A and is genetically confirmed to have WSS.The inpatient care team utilized a combination of psychopharmacotherapy and various therapy modalities to address her behavioral concerns.Medications utilized in this case include Thorazine, Concerta, Ritalin, guanfacine, and Zoloft to optimally address symptoms of behavioral dysregulation as well as comorbidities of attention-deficit/hyperactivity disorder and autism spectrum disorder.Therapeutic methods used to address behavioral concerns included group therapy, expressive therapy, and individual therapy sessions.During the inpatient stay, the patient's sleep and weight improved remarkably.Behavioral regulation was augmented, but not fully resolved, suggesting ongoing interventions will be necessary.Psychiatric interventions in patients with WSS can improve their emotional regulation, preventing escalation to aggression.WSS is relatively understudied, especially the psychiatric attributes of this condition.Further evaluation of the behavioral traits of WSS is essential, and our case report adds to the limited literature on this topic.