Study of Emotion and Cognition Abilities of Children With PWS and Proposition of an Innovative Remediation (PRACOM1)

Toulouse University Hospital

Status: Completed

Conditions: Prader-Willi Syndrome

Treatments: Behavioral: Questionnaires; Device: t-VNS

Study type: Interventional

Funder types: Other

Identifiers

NCT04526379

RC31/18/0335

Details and patient eligibility

About

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.

Full 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.

Enrollment: 50 patients

Sex: All

Ages: 9 to 15 years old

Volunteers: Accepts Healthy Volunteers

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.

Trial design

Primary purpose: Other

Allocation: Non-Randomized

Interventional model: Parallel Assignment

Masking: None (Open label)

---

50 participants in 2 patient groups

children with PWS

Experimental group

Description:

Evaluation of cognitive abilities of children with PWS by several cognitive tasks and neuropsychological tests.

Treatment:

Behavioral: Questionnaires

Device: t-VNS

non affected children

Other group

Description:

Evaluation of cognitive abilities of children with PWS compared to a non-pathologic population of children by several cognitive tasks and neuropsychological tests

Treatment:

Behavioral: Questionnaires