Cognitive and Behavioral Therapy of Anxiety in Williams Syndrome

Study Overview
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Brief Summary
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Patients with Williams-Beuren syndrome are eight times more likely to suffer from anxiety compared to the general population. Few therapeutic solutions are proposed to these patients. The objective of this research is to validate a cognitive and behavioral therapy anxiety protocol for patients with this syndrome.

Detailed Description
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Most studies agree that anxiety disorders are more frequent in people with intellectual disabilities that in the general population. Williams syndrome (WS) is a rare disease, associated with an anxiety rate 8 times higher than in the general population. The therapeutic solutions proposed in this population remain limited, in particular concerning approaches without the use of medication. The objective of the study is to evaluate the effectiveness of a program of Cognitive and Behavioral Therapies (CBT) in Williams Syndrome (WS). For this, an ABA-type protocol will be used. It consists of evaluating the effectiveness of an interventional program longitudinally, using a single case type study. Adults patients with WS and suffering from anxiety will be recruited. They will be enrolled in a program consisting in nine sessions of a psychotherapy program targeting anxiety. There will be a pretherapy visit (day 0), nine sessions of psychotherapy (month 0 to month 5) and a visit 3 month after the end of the therapy (at month 8) : the visit of the end of the research (V10). The expected results will be to validate a psychotherapeutic program for patients with WS suffering of anxiety. This program might be extended to other patients with intellectual disability.

Official Title
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Cognitive and Behavioral Therapy of Anxiety in Williams Syndrome

Conditions
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Williams Syndrome, Anxiety

Intervention / Treatment
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* Behavioral: Cognitive and Behavioral Therapy


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Diagnosis of Williams Syndrome Complaint about anxiety 18 years old and more Score of 7 or more at the CELF-4 (it is a scale assessing the language) informed consent signed Exclusion Criteria: Scoring less than 7 at the CELF-4 Hearing impairment Visual impairment

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Cognitive and Behavioral Therapy<br>There is no group, the study will be based on single case method. The sudy concerns 5 patients with a Williams Syndrome | Behavioral: Cognitive and Behavioral Therapy<br>* 9 sessions of Cognitive and Behavioral Therapy will be realised with the patient by a clinical psychologist. They will last between 1h and 1h30.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Likert anxiety Scale | Repeated evaluation of his anxiety by the patient with the Likert scale. Each day, the patient quantifies his anxiety with the scale with a score from 0 to 9. | From day 0 to month 5. | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Hamilton Anxiety Scale | Hamilton anxiety scale will be realised with the patient by a clinical psychologist. It is an evaluation of the anxiety based on 14 questions and observations of the patient. The questions are rated according to the severity of the symptoms: anxious mood, depressed mood, somatic symptoms...Symptoms are evaluated using 5 degrees of severity, from absence of severity to disabling intensity. The overall score ranges from 0 to 60. | This score will be obtained at the pre therapy visit (day 0), at the visit of the end of the therapy (month 5), and at the visit of the end of the research (month 8) | 
| Inhibition score | the inhibition will be assessed with the Go/no Go test which last 3 minutes. | This score will be obtained at the pre therapy visit (day 0), at the visit of the end of the therapy (month 5), and at the visit of the end of the research (month 8) | 
| salivary cortisol | The sample will be realised by a nurse. The level of cortisol is related to the level of stress. | This sample will be obtained at the pretherapy visit (day 0), and at the visit of the end of the research (month 8). | 
| WHOQOL-BREF score | this scale evaluates the quality of life with 26 questions. For each question, the quality of life is evaluated from very low to very high (5 levels). It will be completed by the person of trust. | This score will be obtained at the pre therapy visit (day 0), at the visit of the end of the therapy (month 5), and at the visit of the end of the research (month 8). | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Williams Syndrome, Anxiety, Cognitive and Behavioral Therapy, Intellectual Disability