Prader-Willi Syndrome
Conditions
Keywords
cerebral activity, Olfactory activation
Brief summary
Studying the cerebral activity of children with Prader-Willi Syndrom (PWS) when the study propose to them nasal activations.
Detailed description
The present study propose to implement fMRI and olfactory tests to describe olfactory brain activation and olfactory skills in early OT-exposed and non-exposed children with PWS. This study is part of a wider project in collaboration with the team of S. Steculorum at the Max Planck Institute in Germany who assess the architectural and molecular characterization of the olfactory system development in a mice model of PWS and the role of early OT treatment. Moreover, the study will take advantage of the MRI to document the long-term brain connectivity of early OT-exposed and non-exposed children.
Interventions
the patients have a sniff-test
OTHERfunctional MRI
patients have a an f MRI
Sponsors
Institut National de la Santé Et de la Recherche Médicale, France
University Hospital, Toulouse
Study design
Allocation
NON_RANDOMIZED
Intervention model
PARALLEL
Primary purpose
OTHER
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
5 Years to 7 Years
Healthy volunteers
No
Inclusion criteria
* child with a genetically confirmed diagnosis of PWS and for whom the genetic subtype (deletion or non deletion) has been identified * Child aged between 5 and 7 years * Child with a planned hospitalization at the Toulouse reference center for PWS
Exclusion criteria
* Presence of a contraindication to MRI * Allergy to any of the fragrances used in the olfactory test * Presence of an ENT infection such as rhinitis or sinusitis on the day of inclusion * Reported anosmia * Administrative problems.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| hyperactivation of the right amygdala in young children with PWS not early OT-exposed and aged between 5 and 7 years in response to a sweet food odor compared to a neutral non-food odor. | day 1 | intensity of right amygdala in fMRI |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| hyperactivation of the right amygdala in PWS children stimulated by a sweet food odor vs. a non-food odor compared to non-exposed children. | day 1 | intensity of right amygdala in fMRI |
| composite outcome witn an association between olfactory fMRI results and eating behavior in early OT-exposed and non-exposed children with PWS. | day 1 | scores of the eating beahaviour questionnaires |
| olfactory skills in early OT-exposed and non-exposed children with PWS. | Day 1 | scores of the sniff test between OT-exposed and non-exposed children. |
| resting brain connectivity in early OT-exposed and non-exposed children with PWS. | day 1 | brain connectivity in fMRI between OT-exposed and non-exposed children. |
Countries
France
Contacts
Primary ContactJulie CORTADELLAS
Backup ContactNadege ALGANS
Outcome results
None listed