Chamouilli J M, Razafimahefa B, Pierron H
Service de pédiatrie 1, hôpital Font-Pré, Toulon, France.
Arch Pediatr. 1995 May;2(5):438-41. doi: 10.1016/0929-693x(96)81178-x.
Hypothalamic hamartoma, a rare cause of true sexual precocity, may develop slowly so that its diagnosis may be late.
A 7 1/2-month-old girl was admitted because she suffered from development of physical pubertal changes. Laboratory tests were consistent with premature activation of the hypothalamic-pituitary axis. Initial neuroradiological investigation failed to find any cause. The patient was given medroxyprogesterone that resulted in regression of secondary sexual characteristics but growth rates and skeletal maturity remained accelerated. For that reason, the patient was given triptorexine, LH-RH analogue, 18 months later that resulted in a deceleration of puberty growth rate and skeletal maturity. MRI performed at the age of 9 years showed a small hypothalamic hamartoma and the LH-RH analogue was stopped at the age of 10 years.
MRI is now the best technique for investigating the hypothalamo-hypophyseal area.
下丘脑错构瘤是真性性早熟的罕见病因，其发展可能较为缓慢，以至于诊断可能延迟。
一名7个半月大的女孩因出现青春期身体变化而入院。实验室检查结果与下丘脑 - 垂体轴过早激活相符。最初的神经放射学检查未发现任何病因。该患者接受了甲羟孕酮治疗，结果第二性征消退，但生长速度和骨骼成熟度仍加快。因此，18个月后该患者接受了曲普瑞林（一种促性腺激素释放激素类似物）治疗，这导致青春期生长速度和骨骼成熟度减缓。9岁时进行的磁共振成像（MRI）显示有一个小的下丘脑错构瘤，促性腺激素释放激素类似物在10岁时停用。
MRI现在是检查下丘脑 - 垂体区域的最佳技术。
