Vieira Teresa C, Chinen Renata N, Ribeiro Maria R F, Nogueira Roberto Gomes, Abucham Julio
Division of Neuroendocrinology, Department of Medicine, Escola Paulista de Medicina, Universidade Federal de São Paulo, Brazil.
J Pediatr Endocrinol Metab. 2007 Oct;20(10):1141-4. doi: 10.1515/jpem.2007.20.10.1141.
Central precocious puberty (CPP) is due to premature activation of the hypothalamic-pituitary-gonadal axis. It may be idiopathic or result from congenital or acquired CNS lesions. We describe a 7.4 year-old Brazilian girl with CPP who also presented hypertelorism, limitation of lateral neck rotation and synkinesis of the hands. Sellar and cervical column MRIs revealed pituitary duplication and rudimentary intervertebral disks. We present the clinical and imaging observations of this case, and a thorough literature review of this rare developmental abnormality.
中枢性性早熟(CPP)是由于下丘脑-垂体-性腺轴过早激活所致。它可能是特发性的,也可能由先天性或后天性中枢神经系统病变引起。我们描述了一名7.4岁患有CPP的巴西女孩,她还伴有眼距过宽、颈部侧转受限和手部联带运动。蝶鞍和颈椎MRI显示垂体重复及椎间盘发育不全。我们展示了该病例的临床和影像学观察结果,并对这种罕见的发育异常进行了全面的文献综述。