Prader-Willi Syndrome with elevated follicle stimulating hormone levels and diabetes mellitus

A 21-year-old man with Prader-Willi syndrome (PWS) was hospitalized due to hyperglycemia. After diet therapy and transient insulin administration, his blood glucose levels improved. Based on the fact that his urinary C-peptid e levels increased, the diabetes mellitus may have been due to insulin resi stance with obesity. In addition, his testes had become atrophied. Testoste rone levels remained low even after human chorionic gonadotropin (HCG) admi nistration. Luteinizing hormone (LH) levels were also low after LH releasin g hormone (LHRH) administration. The LH response increased slightly after d aily LHRH administration, indicating hypothalamic hypogonadism, Follicle st imulating hormone (FSH) levels were, however, high and increased after LHRH administration. The selective FSH elevation may have been due to the accom panying idiopathic oligospermia.