Adipsic hypematremia in a patient with pseudotumor cerebri and the primary empty sella syndrome

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Abstract
Adipsic hypematremia, a rare disorder, usually secondary to a hypothalamic lesion, is caused by the combination of partial central diabetes insipidus with hypo-or adipsia. We studied a patient who presented with a global hypothalamic dysfunction including adipsic hypematremia. An extensive work-up disclosed the presence of pseudotumor cerebri and an empty sella turcica. Although endocrine abnormalities including true diabetes insipidus have been reported in conjunction with pseudotumor cerebri or an empty sella, no patient described presented such a global hypothalamic dysfunction or adipsic hypematremia. The increased intracranial pressure is postulated to be the responsible mechanism for both the empty sella and the hypothalamic dysfunction.

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