A 32-year-old man was admitted to our institute in January 1995. A 48, XXYY karyotype had been diagnosed at the age of 6 years, subsequent to delayed speech development. Because of the primary hypergonadotrophic hypogonadism common in this disorder, androgen replacement was first considered in 1988. The rationale was to prevent somatic consequences of hypogonadism such as osteoporosis and muscle wasting. However, this proposal was discarded by a counseling psychiatrist because ". . . androgen replacement would be too risky considering the patient's labile personality structure. . . ." For similar reasons, 3 more clinics refused to initiate androgen replacement therapy. Six months prior to admission to the hospital, the patient developed increasing tiredness, sleeping 10 to 12 hours on weekdays and up to 16 hours on weekends. Therefore, he was finally referred to our institute for reevaluation of androgen replacement therapy.
Heuser I, Hartmann A, Oertel H. Androgen Replacement in a 48, XXYY-Male Patient. Arch Gen Psychiatry. 1999;56(2):194-195. doi: