A spectrum of rare (1 in 10.000, male/femaleratio 5/1) congenital disorders caused by failure of the hypothalamus to release the gonadotropin hormone (GnRH) during embryonic brain differentiation. Afflicted patients evinceabsent or incomplete puberty, and somatic and reproductive problems associatedwith hypogonadism. In addition to hypogonadism, there may be a loss or reduced sense of smell (hence it it sometimes referred to as olfacto-genital dysplasia). For reasons still unexplained, some individuals (about 20%), both males and females, may show an eventual reversal of symptoms. If the condition is not treated, infertility is the likely outcome.
See Congenital abnormalities, Dysplasia, Gonadotropin releasing hormone (GnRH), Hypothalamus, Olfaction, Puberty