Mooradian A D, Morley G K, McGeachie R, Lundgren S, Morley J E
Neurology. 1984 Jan;34(1):79-82. doi: 10.1212/wnl.34.1.79.
We studied a patient with spontaneous periodic hypothermia, agenesis of corpus callosum (Shapiro's syndrome), polydipsia polyuria, and hyponatremia. Endocrine evaluation of hypothalamic-pituitary unit, thyroid, adrenals and gonads was normal. Results of a water deprivation test implied primary polydipsia. Four of 10 reported cases of Shapiro's syndrome had abnormalities in water metabolism. In view of the known effects of arginine vasopressin in thermoregulation, the recurrent hypothermia of this syndrome and the abnormalities of water metabolism may have a common pathogenetic mechanism.
我们研究了一名患有自发性周期性体温过低、胼胝体发育不全(夏皮罗综合征)、烦渴多尿和低钠血症的患者。下丘脑 - 垂体单位、甲状腺、肾上腺和性腺的内分泌评估均正常。禁水试验结果提示原发性烦渴。在已报道的10例夏皮罗综合征病例中,有4例存在水代谢异常。鉴于精氨酸加压素在体温调节中的已知作用,该综合征的复发性体温过低和水代谢异常可能具有共同的发病机制。