Spontaneous periodic hypothermia

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.