Authors: Eleanor Eckert, Kelli Dominick, Ernest Pedapati, Logan Wink, Rebecca Shaffer, Howard Andrews, TseHwei Choo, Chen Chen, Walter E. Kaufmann, Nicole Tartaglia, Elizabeth Berry-Kravis, and Craig Erickson
Behavioral dysregulation, or the impairment of behavioral processes, is common in FXS. A regularly cited group of behaviors in individuals with FXS, particularly males, is irritability, agitation, aggression, and self-injurious (IAAS) behaviors. These behaviors can put a strain on both the individual and their caregiver’s quality of life and there is little information about how to manage these behaviors with medication. This publication in the Journal of Autism and Developmental Disorders presented information from a FORWARD dataset involving 415 individuals with IAAS behaviors. The study describes the psychopharmacologic management of IAAS and examines the characteristics of individuals that are treated with drug therapy for IASS.
Findings showed that among the individuals with FXS that were exhibiting IAAS, those receiving drug treatment were more likely to be older males with significant intellectual disability. The individuals receiving drug treatment were also more likely to have comorbid autism, anxiety, hyperarousal, and social impairment. The medications most used in this population are antipsychotic medications, particularly aripiprazole and risperidone. Both aripiprazole and risperidone are FDA-approved for treating irritability associated with ASD. Individuals were also prescribed drugs outside of antipsychotic medications, including selective serotonin reuptake inhibitors (SSRIs), stimulants, non-SSRI antidepressants, alpha-agonists, mood-stabilizers, and anxiolytics. Most individuals (63%) did not experience side effects from their drug treatment.
Why This Is Important
This study contributes to the limited understanding of psychopharmacologic management of IAAS in FXS and will help guide future treatment.
What Are the Next Steps
A deeper analysis of long-term drug treatment of various target symptoms, more specific evaluation of each behavior within the IAAS symptom category, and more extensive analysis of drug tolerability over time.
Drs. Berry-Kravis and Erickson presented on this topic using the FORWARD data in her presentation, “Medications for Fragile X: Anxiety, Irritable Behaviors, Aggression,” during the Fragile X Across the Lifespan event in the 17th NFXF International Fragile X Conference Virtual Series, summer 2020.
more research results
Delineating Repetitive Behavior Profiles Across the Lifespan in Fragile X Syndrome
RESEARCH RESULTS ROUNDUP — What restricted and repetitive behaviors looked like in 154 individuals with FXS, 2–50 years old, based on age, ASD diagnosis, and IQ score.
Pharmacologic Interventions for Irritability, Aggression, Agitation, and Self-Injurious Behavior in Fragile X Syndrome: An Initial Cross-Sectional Analysis
RESEARCH RESULTS ROUNDUP — The psychopharmacologic management of irritability, agitation, aggression, and self-injurious behaviors and drug therapy treatment.
Toilet Training in Fragile X Syndrome
Research Summary // FORWARD data on 633 individuals with FXS filled the gap for much needed information on when children with FXS learn bladder and bowel toileting skills.
Preventive Care Services and Health Behaviors in Children with Fragile X Syndrome
RESEARCH RESULTS ROUNDUP — This research can help identify preventive care services that patients with FXS may need and focus on reaching recommended preventative care objectives.
Validation of the NIH Toolbox Cognitive Battery in Intellectual Disability
RESEARCH RESULTS ROUNDUP — Researchers have updated and validated a series of tests within the NIH Toolbox (NIHTB) to assess cognition in people with intellectual disability.
Controlled Trial of Lovastatin Combined With an Open-Label Treatment of a Parent-Implemented Language Intervention in Youth With Fragile X Syndrome
RESEARCH RESULTS ROUNDUP — Controlled trial explores whether the combined lovastatin/behavioral therapy approach would be more effective than just behavioral therapy alone.