[Precocious puberty revealing Bourneville tuberous sclerosis].

BACKGROUND

Benign tumors are seen in tuberous sclerosis. They are found in many organs, and the precocious puberty due to hypothalamic hamartoma and tuberous sclerosis has been reported. However, precocious puberty exceptionally reveals the tuberous sclerosis.

CASE REPORT: A 2 month-old boy was admitted because of the fortuitous discovery of polycystic renal disease. Precocious puberty developed at 13 months with enlargement of the penis and testes, appearance of pubic hair, acne and deepening of the voice. Linear growth was recently accelerated and the bone maturation was advanced. Plasma testosterone was elevated (460 ng/100 ml) and LH-RH injection induced rapid rises in plasma LH (2.6 to 28 mUl/ml) and FSH (2 to 8 mUl/ml). Brain imaging techniques (CT scan and NMR) showed a hypothalamic hamartoma and periventricular calcified lesions. Examination with the Wood lamp identified two white leaf macules in the dorsal area. Administration of an analogue of LH-RH effectively reduced the manifestations of precocious puberty.

CONCLUSION

Tuberous sclerosis is exceptionally revealed by precocious puberty. The association of polycystic disease and precocious puberty has never before been reported in tuberous sclerosis.