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Abstract
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.