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Sexual precocity in association with septo-optic dysplasia and hypothalamic hypopituitarism
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.
Insights
Sexual precocity is rarely seen with hypothalamic hypopituitarism. This study found that some children with septo-optic dysplasia and pituitary deficiencies could still secrete gonadotropins, challenging current understanding.
Area of Science:
- Pediatric Endocrinology
- Neuroendocrinology
- Genetics and Developmental Biology
Background:
- Sexual precocity is often linked to central nervous system abnormalities.
- Hypothalamic hypopituitarism typically presents with deficiencies in pituitary tropic hormones.
- The co-occurrence of sexual precocity and hypothalamic hypopituitarism is considered highly unusual.