Embryologically GnRH neurones develop in the ectodermal olfactory placode before they migrate finally to the hypothalamus. GnRH neurones are absent due to partial or complete agenesis of olfactory bulb (olfactogenital dysplasia). This disorder is characterized by hypogonadotropic hypogonadism, anosmia and color blindness. There may be associated cleft lip and palate. Patients present with primary amenorrhea. Mode of inheritance is due to a variety of genetic mutations in the KAL gene (X linked) or as an autosomal dominant or recessive fashion.
Menstruation can be induced with combined estrogen and progestin therapy. Induction of ovulation is successful with exogenous gonadotropins.
Adenoma: In adenoma either micro or macro, there is usually associated inappropriate lactation, secondary amenorrhea and infertility. There may be headache with disturbed vision.
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