Tarng D C, Huang T P
Department of Medicine, Veterans General Hospital, Taipei, Taiwan, ROC.
Am J Nephrol. 1995;15(2):161-4. doi: 10.1159/000168823.
A 13-year-old boy's acute onset of diabetes insipidus was the first manifestation of a pineal tumor. One year after the physical symptoms of diabetes insipidus occurred, neurological examinations, tumor markers, and computed tomography brain scan were all normal. Three years later, rapid development of neurological changes was observed with the prevailing symptoms of increased intracranial pressure. The changes were related to a pineal germ cell tumor identified by a high level of beta-human chorionic gonadotropin in plasma and neuroradiological investigations, including computed tomography and magnetic resonance imaging. The clinical course of this case is unusual and we advocate the following: (1) the follow-up of children with diabetes insipidus is important for determining whether or not it is an idiopathic type and (2) elevation of plasma beta-human chorionic gonadotropin and development of neurological signs in patients with so-called idiopathic diabetes insipidus should arouse clinical suspicion of an intracranial germ cell tumor. It should be noted that the brain tumor is significant as the underlying pathology of childhood diabetes insipidus.
一名13岁男孩急性发作尿崩症，这是松果体肿瘤的首发表现。尿崩症出现身体症状一年后，神经学检查、肿瘤标志物及脑部计算机断层扫描均正常。三年后，观察到神经学变化迅速发展，主要症状为颅内压升高。这些变化与通过血浆中高水平β-人绒毛膜促性腺激素及包括计算机断层扫描和磁共振成像在内的神经放射学检查所确定的松果体生殖细胞瘤有关。该病例的临床病程不寻常，我们主张如下：（1）对尿崩症患儿进行随访对于确定其是否为特发性类型很重要；（2）在所谓特发性尿崩症患者中，血浆β-人绒毛膜促性腺激素升高及神经学体征出现应引起对颅内生殖细胞瘤的临床怀疑。应当注意，脑肿瘤作为儿童尿崩症的潜在病理情况意义重大。
