Pathogenesis of precocious puberty in hypothalamic hamartoma.
Astroglial-derived factors, as transforming growth factor (TGF)alpha and TGFbeta, act in the hypothalamus to activate luteinizing hormone-releasing hormone (LHRH) secretion. Hypothalamic hamartomas (HHs) contain normal nervous tissue in a heterotopic location. When symptomatic, they cause precocious puberty and/or characteristic gelastic seizures. Thus far, the pathogenesis of these alterations remains unknown. By examining two HHs associated with sexual precocity, we found that they contained astroglial cells expressing TGFalpha, but no LHRH neurons. In a third patient with HH, only epilepsy was present, but precocious puberty developed shortly after surgery, probably as a consequence of a surgery-induced lesion. These results imply that some HHs induce sexual precocity by activating endogenous LHRH secretion via astroglial-derived factors.
Jung
Heike
H
Clinic of Pediatrics and Genetics, University Hospital Hannover, Germany. Jung_Heike@Lilly.com
Ojeda
Sergio R
SR
D001253:Astrocytes / Q000737:chemistry / Q000473:pathology; D002675:Child, Preschool; D004828:Epilepsies, Partial / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006222:Hamartoma / Q000150:complications* / Q000473:pathology / Q000628:therapy; D006801:Humans; D007027:Hypothalamic Diseases / Q000150:complications* / Q000473:pathology / Q000628:therapy; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D007986:Luteinizing Hormone / Q000097:blood; D011629:Puberty, Precocious / Q000209:etiology*; D015291:Transforming Growth Factors / Q000502:physiology
D015291:Transforming Growth Factors; D007986:Luteinizing Hormone