Clinical trial NCT04526379: Study of Emotion and Cognition Abilities of Children With PWS and Proposition of an Innovative Remediation
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
Study type: INTERVENTIONAL
Overall status: COMPLETED
Phase: NA
Conditions: Prader-Willi Syndrome
Keywords: Emotion
Cognition
Lead sponsor: University Hospital, Toulouse
Sponsor class: OTHER
First posted: 2020-08-25
Enrollment: 50
Sex: ALL
Minimum age (years): 9
Maximum age (years): 15
Standard ages: CHILD
Accepts healthy volunteers: yes
Allocation: NON_RANDOMIZED
Intervention model: PARALLEL
Intervention model description: The study consists of 2 parts. The first part compares 30 children with PWS compared to 30 control children without PWS. The second part will be based on 12 children with PWS who have undergone part 1.
Primary purpose: OTHER

Brief 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.

Detailed 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.

Eligibility criteria: 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.

Primary outcomes:
measure: assessment of the level of emotional control
timeFrame: day 0
Secondary outcomes:
measure: Evaluation of behavioral disorders of children with PWS by CBCL (Child Behavior Checklist) scale
timeFrame: month 6
measure: Evaluation of behavioral disorders of children with PWS by Hyperphagia Questionnaire
timeFrame: month 6
measure: Evaluation of cognitive abilities of children with PWS
timeFrame: 2 days
measure: Test of evaluation of cognitive abilities
timeFrame: 2 days
measure: Evaluation of cognitive abilities
timeFrame: 2 days
Other outcomes:
Locations:
city: Toulouse
country: France
facility: University Hospital
Study officials:
affiliation: University Hospital, Toulouse
name: Maithé TAUBER, MD
role: PRINCIPAL_INVESTIGATOR
References: