Authors: Jessica Klusek, Jinkuk Hong, Audra Sterling, Elizabeth Berry-Kravis, and Marsha Mailick
At the time of this writing, three Fragile X specialty clinics are collecting supplemental information for FORWARD. They assess and collect longitudinal (over time) data on a group of tests to measure cognitive and behavioral function in the FXS population so that we can specifically understand the course of development in FXS. This is one of the two publications that was a result of those efforts.
Summary
Restricted and repetitive behaviors (RRBs) are a group of behaviors that include repetitive movements, inflexible adherence to routines, ritualistic behavior, restricted interests, and unusual sensory interests. RRBs are a known domain of ASD but also affect individuals with other neurodevelopmental disorders such as FXS. This study took at a look at what RRBs looked like in 154 individuals with FXS, 2–50 years old. Researchers examined the effect that age, ASD diagnosis, and IQ score had on RRB severity.
Unique RRB profiles emerging based on age and gender with increased severity of RRBs based on ASD diagnosis. Findings suggest a peak in RRBs between the ages of 7 and 12, the exception being sensory-motor behaviors, which appear to peak between the ages of 2 and 12 years old. An added diagnosis of ASD significantly increased repetitive behaviors in these individuals. IQ score alone did not seem to influence the presence of RRBs.
Why This Is Important
These findings contribute to specialists understanding of RRBs in FXS. Taking an individual’s diagnoses and sex into account may help clinicians assess and understand their restricted repetitive behaviors to form a treatment plan.
What Are the Next Steps
More research is needed to develop a deeper understanding of the effect IQ, gender, and ASD diagnosis have on the RRB profile of individuals with FXS and inform treatment. Looking at these behaviors over time may help professionals understand changes in RRBs in individuals with FXS over time.
FOR MORE DETAILS VISIT:
more research results
Inhibition Deficits Are Modulated by Age and CGG Repeat Length in Carriers of the FMR1 Premutation Allele Who Are Mothers of Children with Fragile X Syndrome
RESEARCH RESULTS ROUNDUP — Older mothers of children with Fragile X syndrome who have mid-range CGG repeats (~80–100) may be at increased risk for difficulties with inhibition.
Language Across the Lifespan in Fragile X Syndrome: Characteristics and Considerations for Assessment
RESEARCH RESULTS ROUNDUP — Describe cognition and language in FXS using the data from a large group, while trying to understand if the standard assessments are feasible (able to be done accurately) and valid (reflect reality) in FXS and then compared the assessment results to caregiver report.
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.