Imura H, Nakao K, Shimatsu A, Ogawa Y, Sando T, Fujisawa I, Yamabe H
Department of Medicine, Kyoto University Faculty of Medicine, Japan.
N Engl J Med. 1993 Sep 2;329(10):683-9. doi: 10.1056/NEJM199309023291002.
Central diabetes insipidus may be familial, secondary to hypothalamic or pituitary disorders, or idiopathic. Idiopathic central diabetes insipidus is characterized by selective hypofunction of the hypothalamic-neurohypophysial system, but its cause is unknown.
We studied 17 patients with idiopathic diabetes insipidus, in whom the duration of the disorder ranged from 2 months to 20 years. Only four patients had been treated with vasopressin before the study began. All the patients underwent endocrinologic studies and magnetic resonance imaging (MRI) with a 1.5-T superconducting unit, and two patients had biopsies of the neurohypophysis or the pituitary stalk.
Nine of the 17 patients had thickening of the pituitary stalk, enlargement of the neurohypophysis, or both and lacked the hyperintense signal of the normal neurohypophysis. In the remaining eight patients, the pituitary stalk and the neurohypophysis were normal, although the hyperintense signal was absent. The abnormalities of thickening and enlargement were seen on MRI only in the patients who had had diabetes insipidus for less than two years, and the abnormalities disappeared during follow-up, suggesting a self-limited process. In addition to vasopressin deficiency, two patients had mild hyperprolactinemia and nine had impaired secretory responses of growth hormone to insulin-induced hypoglycemia. The two biopsies revealed chronic inflammation, with infiltration of lymphocytes (mainly T lymphocytes) and plasma cells.
Diabetes insipidus can be caused by lymphocytic infundibuloneurohypophysitis, which can be detected by MRI. The natural course of the disorder is self-limited.
中枢性尿崩症可能是家族性的，继发于下丘脑或垂体疾病，或为特发性。特发性中枢性尿崩症的特征是下丘脑 - 神经垂体系统选择性功能减退，但其病因不明。
我们研究了17例特发性尿崩症患者，疾病持续时间为2个月至20年。在研究开始前，只有4例患者接受过血管加压素治疗。所有患者均接受了内分泌学检查和使用1.5T超导装置的磁共振成像（MRI）检查，2例患者对神经垂体或垂体柄进行了活检。
17例患者中有9例出现垂体柄增粗、神经垂体增大或两者皆有，且缺乏正常神经垂体的高强度信号。其余8例患者的垂体柄和神经垂体正常，尽管缺乏高强度信号。垂体柄增粗和增大的异常仅在尿崩症病程小于两年的患者中在MRI上可见，且这些异常在随访期间消失，提示这是一个自限性过程。除血管加压素缺乏外，2例患者有轻度高催乳素血症，9例患者生长激素对胰岛素诱导的低血糖的分泌反应受损。两次活检均显示慢性炎症，有淋巴细胞（主要是T淋巴细胞）和浆细胞浸润。
尿崩症可由淋巴细胞性漏斗神经垂体炎引起，可通过MRI检测到。该疾病的自然病程是自限性的。
