Study of Emotion and Cognition Abilities of Children With PWS and Proposition of an Innovative Remediation

Prader-Willi Syndrome Clinical Trial

— PRACOM1  

Official title:

Communication in Prader-Willi Syndrome: Study of Emotional Control Related to Behavioral Disorders, Their Daily Repercussions and Examination of an Innovative Therapy: Transcutaneous Electrical Nerve Stimulation of the Vagus Nerve - PRACOM1

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT04526379
• Other study ID #: RC31/18/0335
• Status: Completed
• Phase: N/A
• Start date: September 7, 2020
• Est. completion date: April 27, 2022

Study information

• Verified date: August 2023
• Source: University Hospital, Toulouse
• Contact: n/a
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial Summary

The present project project is divided in two parts. The primary aim of the part 1 of this study is to evaluate emotional control abilities of children with Prader-Willi syndrome (PWS) aged from 9 to 15 years and to study repercussions of this supposed lack of abilities on cognitive capacities and behavioral troubles. The study also evaluate influence of the emotional symptomatology of patients on quality of parents' life and on the care of parents and scholar/institutional caregivers. In the second part of this study, the study evaluate the feasibility and the tolerance to a non-invasive device supposed to reduce emotional symptoms in this disease.

Description:

Prader-Willi Syndrome (PWS) is a rare genetic disorder involving a variety of clinical, behavioral and cognitive symptoms. 83 to 97% of patients have episodes of temper tantrum, associated with an important emotional lability. These different cognitive and behavioral limitations are barriers to the social integration of patients. The PWS also has repercussions on well-being and quality of life of the family. Thus, the present project focuses on the characteristics of emotional control related to the anger of children with PWS and its behavioral, cognitive and social implications. In addition, at a therapeutic level, our project assumes that transcutaneous electrical nerve stimulation (t-VNS), a non-invasive, safe and inexpensive method, could be effective in reducing the severity of behavioral disorders such as temper tantrums while improving cognitive performance and social communication in the PWS.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 50
• Est. completion date: April 27, 2022
• Est. primary completion date: December 7, 2020
• Accepts healthy volunteers: Accepts Healthy Volunteers
• Gender: All
• Age group: 9 Years to 15 Years

Eligibility

Inclusion Criteria:

• Age from 9 to 15 years old
• Prader-Willi syndrome with identified genotype.
• No psychiatric disorder neither PWS.
• Severe global symptomatology attested by a CGI-S score

Exclusion Criteria:

• Identified psychiatric or behavioral disorders
• Severe visual or hearing impairment.
• Sleep apnea syndrome treated with non-invasive ventilation,
• Epileptic seizures
• Cardiac disorders.

Related Conditions & MeSH terms

• Prader-Willi Syndrome
• Syndrome

Intervention

Behavioral:
• Questionnaires
Evaluation of behavioral disorders of children with PWS by questionnaires

Device:
• t-VNS
Evaluation of the influence of 6 month-stimulation by t-VNS on emotional, cognitive, and behavioral/clinical characteristics of 12 children with PWS on 30 PWS patients included.

Locations

Country	Name	City	State
France	University Hospital	Toulouse

Sponsors (1)

Lead Sponsor	Collaborator
University Hospital, Toulouse

Country where clinical trial is conducted

France, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	assessment of the level of emotional control	composite score of emotional control level of children with PWS composed of scores of emotional lability	day 0
Secondary	Evaluation of behavioral disorders of children with PWS by CBCL (Child Behavior Checklist) scale	CBCL (Child Behavior CheckList) scale: is a questionnaire comprising 1) an adaptive functioning scale (activities, social and school spheres), 2) a syndrome scale (social withdrawal, anxiety / depression, somatic complaints, social problems, thinking problems, attention problems, behavior problems and aggressiveness; total of internalized problems and total of externalized problems), 3) a DSM-oriented scale (Lengua et al., 2001; anxiety, attention problems / hyperactivity, behavior problems, depression, opposition / mistrust, social problems / immaturity, somatization). (duration = 20 ') - there is no minimum and maximum values as this is an evaluation of behavioral disorders	month 6
Secondary	Evaluation of behavioral disorders of children with PWS by Hyperphagia Questionnaire	HQ-CT (Hyperphagia Questionnaire) scale: The HQ-CT (Hyperphagia Questionnaire for Use in Prader-Willi Syndrome Clinical Trials, Fehnel et al., 2015, appendix 10) is a scale derived from the Dykens hyperphagia questionnaire (Dykens et al., 2007). The specificity of the Dykens questionnaire makes it a favored tool in the international literature in the evaluation of the eating behavior of patients with PWS (Crain, 2010). The HQ-CT was created specifically to assess the eating behavior of these patients in clinical trials. It consists of 9 items for which a person taking care of the patient (family or caregiver) indicates the frequency and severity of the behavior described on a 5-point Likert scale (with 1 = absence of the behavior and 5 = very frequent behavior or severe) (? = .91). (duration = 5 ') - so 1 is the minimum value and 5 the maximum value and higher scores mean a worse outcome	month 6
Secondary	Evaluation of cognitive abilities of children with PWS	emotional language task	2 days
Secondary	Test of evaluation of cognitive abilities	multimodal emotional recognition test MERT	2 days
Secondary	Evaluation of cognitive abilities	WISC-V	2 days