Skip to main content
OpenTrials
Completed

NCT Number: NCT04526379

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

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.

Completed

Looking for future studies?

Notify Me

Key information

Conditions

Prader-Willi Syndrome Abnormalities, Multiple Chromosome Disorders Congenital Abnormalities Congenital, Hereditary, and Neonatal Diseases and Abnormalities Genetic Diseases, Inborn Imprinting Disorders Intellectual Disability Nervous System Diseases Neurobehavioral Manifestations Neurologic Manifestations Nutrition Disorders Nutritional and Metabolic Diseases Obesity Overnutrition Overweight

Age range

9 year–15 year

Sex eligibility

All sexes

Study type

Interventional

Phase

Not applicable

Primary location

University Hospital

Toulouse, France

About this study

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.

Who can participate

Healthy volunteers accepted: Yes

Only the study team can determine whether someone qualifies for participation.

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.

Treatment and study plan

Questionnaires

Behavioral

Evaluation of behavioral disorders of children with PWS by questionnaires

t-VNS

Device

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.

Primary outcomes

  1. assessment of the level of emotional control

    Time frame: day 0

    composite score of emotional control level of children with PWS composed of scores of emotional lability

Secondary outcomes

  1. Evaluation of behavioral disorders of children with PWS by CBCL (Child Behavior Checklist) scale

    Time frame: month 6

    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

  2. Evaluation of behavioral disorders of children with PWS by Hyperphagia Questionnaire

    Time frame: month 6

    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

  3. Evaluation of cognitive abilities of children with PWS

    Time frame: 2 days

    emotional language task

  4. Test of evaluation of cognitive abilities

    Time frame: 2 days

    multimodal emotional recognition test MERT

  5. Evaluation of cognitive abilities

    Time frame: 2 days

    WISC-V

Sponsors and collaborators

Lead sponsor

University Hospital, Toulouse

Other

Registry information

Official study 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

Acronym: PRACOM1

Important dates

Study start
2020
Primary completion
2020
Study completion
2022
First posted
Aug 25, 2020
Registry last updated
Aug 23, 2023

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

Related clinical trials

Published trials that share one or more normalized conditions with this study.