Dexamethasone Suppressible Hypergonadotropism in an Adolescent Patient with Prader-Willi Syndrome

Abstract

We report an adolescent patient with Prader-Willi syndrome accompanyingsuppressible hypergonadotropism. The subject is an 18-year-old female. Shewas obese (body mass index: 35.7) and hypomyotonic with mental retardation.On endocrinological examination, a high serum LH concentration and hyperresponsivenessof luteinizing hormone (LH) to intravenously administered LHReleasingHormone (LH-RH) were observed, while the basal follicle stimulatinghormone level was within the normal range. In addition, serum dehydroxyepiandrosteronesulfate (DHEA-S) was also increased. Following 2 mg dexamethasoneadministration for 7 days, serum LH and DHEA-S were almostnormalized and hyperresponse of LH to LH-RH completely disappeared.
The present study provides evidence that altered responsiveness to adrenalsteroid may be involved in the establishment of hypergonadotropinism in an adolescent patient with Prader-Willi syndrome.