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.

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