NCT05829668 · Hugo W. Moser Research Institute at Kennedy Krieger, Inc.
Behavioral Assessment and Treatment of Problem Behavior in Children With Cornelia de Lange Syndrome
What this study is about
The goals of this clinical trial are to identify factors associated with the development of problem behavior in Cornelia de Lange syndrome (CdLS) and to develop an effective behavioral assessment and treatment model for problem behavior in children with CdLS. The hypotheses are as follows: 1.
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The goals of this clinical trial are to identify factors associated with the development of problem behavior in Cornelia de Lange syndrome (CdLS) and to develop an effective behavioral assessment and treatment model for problem behavior in children with CdLS. The hypotheses are as follows: 1. Based on pilot data, the investigators hypothesize that individuals with CdLS will exhibit preferences for auditory stimuli relative to other categories (e.g., visual, tactile) of stimuli. 2. Based on pilot data, the investigators hypothesize that individuals with CdLS will exhibit problem behavior to obtain adult attention or to escape task demands relative to tangible and control conditions, as measured by functional analysis results. 3. Function-based behavioral treatments will reduce problem behavior in individuals with CdLS by 80% or greater relative to baseline rates. 4. Individuals with CdLS and problem behavior will exhibit more impaired communication, demonstrate increased emotion dysregulation, and exhibit more severe symptoms of Autism Spectrum Disorder (ASD) relative to those with CdLS and no problem behavior. Participants in the intervention group (families of children with CdLS and problem behaviors) will be asked to complete study measures and attend 2 full days and one half-day of clinic services at Kennedy Krieger Institute so that the study team can provide assessment and treatment of child problem behaviors, and then train parents to apply the intervention. Participants in the control group (families of children with CdLS and no problem behavior) will be asked to complete study measures once every 3 months for a 2-year period to monitor the children. This study will improve the ability to effectively treat problem behavior is CdLS, as well as identify key variables associated with problem behavior in CdLS which may be examined in future studies and clinical practice to foster early intervention and prevention efforts.
Interventions
BEHAVIORAL
ABA-based functional analysis and treatment
Functional analysis to identify environmental variables maintaining child problem behavior and function-based treatment
Primary outcome measures
Change in Behavior Problems Inventory (BPI) scores
Time frame: pre-intervention (prior to admission) to post-intervention (at 2-week follow-up visit)
The investigators will also quantify changes in problem behavior following the intervention relative to baseline with the normalized scores on the Behavior Problems Inventory (BPI). This scale has two sections: scores on Frequency of problem behavior (with a maximum score of 208 and a minimum score of zero) and scores on the Severity of problem behavior (with a maximum score of 156 and a minimum score of zero). Higher scores on these sections indicate worse outcomes, so decreases in the scores from pre-intervention to post-intervention would be indicative of a therapeutic outcome.
Change in Aberrant Behavior Checklist (ABC) scores
Time frame: pre-intervention (prior to admission) to post-intervention (at 2-week follow-up visit)
The investigators will also quantify changes in problem behavior following the intervention relative to baseline with the normalized scores on the Aberrant Behavior Checklist (ABC). The ABC has 5 subscales: Irritability, Social Withdrawal, Stereotypic Behavior, Hyperactivity/Noncompliance, and Inappropriate Speech. Scores on the Irritability subscale (maximum of 45 and a minimum of zero), in particular, would be indicative of problem behavior. Higher scores on this subscale indicate worse outcomes, so decreases in this scale scores from pre-intervention to post-intervention would be indicative of a therapeutic outcome.
Who can participate
This study lists these criteria on ClinicalTrials.gov. A study coordinator reviews eligibility during screening — this page does not determine whether you qualify.
Inclusion criteria
- for treatment group:
- Child with CdLS and problem behavior:
- clinical diagnosis of CdLS confirmed;
- age 3 to 15 years;
- is English-speaking;
- exhibits SIB, aggression, and/or disruptive behavior; and
- is able to participate in several hours of assessment/treatment.
- Parent of child with CdLS is English-speaking and is willing to:
- attend 2 full-day and one half-day clinic visits;
- complete and return study measures; and
- implement a behavior plan. Inclusion Criteria for control group:
- Child with CdLS without problem behavior:
- clinical diagnosis of CdLS confirmed;
- is English-speaking
- age 3 to 15 years; and
- child's age and adaptive functioning level matches that of a child in the treatment group
- Parent of child with CdLS is English-speaking and willing to:
- complete and return study measures.
Exclusion criteria
- for treatment group:
- Child with CdLS:
- does not exhibit SIB, aggression, or disruptive behavior;
- child is younger than 3 years or older than 15 years;
- is not English-speaking;
- has severe SIB that produces significant tissue damage or requires protective equipment;
- has uncontrolled seizures; or
- has medical conditions or requires time-intensive medical care that limits attendance or participation.
- Parent of child with CdLS is not English-speaking or is not willing to complete study procedures. Exclusion Criteria for the control group:
- Child with CdLS:
- exhibits SIB, aggression, or disruptive behavior;
- is younger than 3 years or older than 15 years;
- is not English-speaking;
- age and adaptive functioning level do not match that of a child in the treatment group.
- Parent of child with CdLS is not English-speaking or willing to complete study procedures.
Where
- Baltimore, Maryland
Related conditions & keywords
Frequently asked questions
What is a clinical trial?
A clinical trial is a research study that tests new medical treatments, drugs, devices, or procedures to determine their safety and effectiveness. Trials are carefully designed and monitored to protect participants while advancing medical knowledge.
Is it safe to participate?
Clinical trials follow strict safety guidelines and ethical standards. Trials must be reviewed and approved, and participants are closely monitored by medical professionals throughout the study. You can withdraw at any time if you choose.
Will I be compensated?
Many clinical trials offer compensation for your time, travel expenses, and inconvenience. The specific compensation varies by study and will be discussed during the screening process. All study-related medical care is typically provided at no cost to participants.
Will I receive a placebo instead of treatment?
When effective treatment exists, participants typically receive either the standard treatment plus the study intervention, or the standard treatment plus placebo. You would not be denied effective care. Placebos are primarily used when no proven treatment is available, or in addition to standard care. Your trial consent form will clearly explain what treatments you may receive.
Can I leave a trial if I change my mind?
Absolutely. Participation in clinical trials is completely voluntary. You have the right to withdraw from the study at any time, for any reason, without penalty or loss of benefits to which you are otherwise entitled.
How long does a clinical trial last?
Trial duration varies widely depending on the study design and purpose. Some trials last just a few weeks, while others may continue for months or years. The study coordinator will provide specific timeline information during your screening call.
Data: ClinicalTrials.gov · synced Oct 29, 2025 · Source of record for eligibility and locations