Exposé de trois cas de troubles pubertaires dus à une lésion neurologique

Summary

Case 1. A girl aged 17 ½, with normal puberal morphology, but with primary amenorrhoea and some psychological infantilism. Appearance of a large cyst due to obstruction of the 4th ventricle by a polar spongioblastoma. Insertion of a Pudenz valve. Spontaneous menstruation. Marriage; one healthy child.

Case 2. 14 years old girl with complete infantilism, absence of menses and emaciation. Polar spongioblastoma occupying the whole of the 3rd ventricle. Raised pressure in the posterior fossa followed by involvement of the amygdala. Died.

Case 3. Girl aged 3 with iso-sexual precocious puberty. The fact that the oestrogen and FSH levels are only slightly increased suggests a diencephalic source of the condition. Hamartoma found in the mamillary region. Treatment by interstitial radiotherapy (radioactive gold—9 mc—Talairach). Definite improvement. Relapse treated with a diencephalo-pituitary inhibitor (Orgamétril).

The authors emphasise that the endocrinologist must look for a diencephalic origin in cases of sex disturbance, when these can not be adequately explained by the results of examinations and analyses of the functions of the gonads and the pituitary.