GH in Adults With PWS, Effect on Hypotonia Evaluated by Functional MRI, Relationship With Strength and Body Composition
Status:
Completed
Trial end date:
2019-07-26
Target enrollment:
Participant gender:
Summary
Prader-Willi syndrome (PWS) is a genetic disorder associated with growth hormone (GH)
deficiency, central hypotonia and hyperphagia that leads to life-threatening obesity.
Treatment with GH in adult patients is not well stablished in guidelines of Health National
System (HNS). The investigators has experience in the study of brain connectivity in these
patients in relation to satiety. To date, there is no evidence about the effect of GH on
central hypotonia (brain areas related with muscle tone maintenance). So, the main objective
is to examine these anatomical areas before and one year after GH treatment.
Methodology: Structural and functional magnetic resonance imaging to 30 PWS patients before
and after GH treatment and we will compare them to a control group.
Expected results: PWS group will show abnormal functional and structural connectivity in
circuitry of muscle tone maintenance that will improve after GH treatment. These favorable
changes and the absence of secondary effects will help to justify the use of this treatment
and its inclusion in practical clinical guidelines of HNS for the management of this syndrome
in the adulthood.