Chronic and Sustained Hypernatremia, Absence of Thirst, Diabetes Insipidus, and Adrenocorticotrophin Insufficiency Resulting from Widespread Destruction of the Hypothalamus

Abstract

A case of Schuller-Christian disease with incomplete diabetes insipidus and an apparent primary deficiency in the excretion of adrenocorticotrophic hormone is described. A persistent hypernatremia and hyperosmolality not associated with thirst was also present. Post-mortem analysis revealed widespread hypothalamic destruction. Study revealed that the hypernatremia persisted within a relatively narrow range in the presence of water loading and varying intakes of sodium. Following reversal of the diabetes insipidus during prednisone therapy plasma osmolality and Na values continued at the previously elevated levels suggesting separate defects involving fluid balance (1) an abnormality in the release of antidiuretic hormone resulting in incomplete diabetes insipidus which was corrected during therapy with prednisone; (2) a disorder of osmoregulation associated with persistent and regulated hypernatremia and the absence of thirst.