Sexual precocity in association with septo-optic dysplasia and hypothalamic hypopituitarism.
Sexual precocity in association with abnormalities of the central nervous system is well known, but its occurrence with hypothalamic hypopituitarism is most unusual. We report five females with septo-optic dysplasia, blindness, and multiple pituitary tropic hormone deficiencies: all were growth hormone and adrenocorticotropic hormone deficient; two had diabetes insipidus; one had sexual precocity, and one had early pubertal maturation, whereas three were prepubertal and responded to administration of synthetic gonadotropin-releasing hormone. These children retained ability to secrete gonadotropins despite the presence of anterior hypothalamic disease. Experimental data from primates plus our observations on these patients raise questions about the role of the anterior hypothalamus in gonadotropin secretion in man.
D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000172:deficiency; D000328:Adult; D061085:Agenesis of Corpus Callosum; D001766:Blindness / Q000150:complications; D002648:Child; D002675:Child, Preschool; D005260:Female; D013006:Growth Hormone / Q000172:deficiency; D006801:Humans; D007018:Hypopituitarism / Q000150:complications*; D007030:Hypothalamo-Hypophyseal System / Q000503:physiopathology; D007032:Hypothalamus, Anterior / Q000503:physiopathology; D007223:Infant; D009900:Optic Nerve / Q000002:abnormalities*; D010906:Pituitary Hormone-Releasing Hormones; D010907:Pituitary Hormones / Q000378:metabolism; D011247:Pregnancy; D011629:Puberty, Precocious / Q000150:complications*; D012688:Septum Pellucidum / Q000002:abnormalities*; D013577:Syndrome
D010906:Pituitary Hormone-Releasing Hormones; D010907:Pituitary Hormones; D000324:Adrenocorticotropic Hormone; D013006:Growth Hormone