Hypothalamic histiocytosis X with diabetes insipidus and Korsakoff's syndrome--case report.

A 54-year-old female presented with apparent isolated hypothalamic histiocytosis X associated with diabetes insipidus and Korsakoff's syndrome. Computed tomographic and magnetic resonance imaging demonstrated a single hypothalamic mass. A craniotomy for biopsy found granulation tissue of unknown cause. Further investigation discovered genital bleeding before admission. Biopsy of the cervix uteri revealed histiocytosis X. Further studies showed the disease was restricted to the hypothalamus and the endometrium of the cervix uteri. Low-dose irradiation led to partial regression of the hypothalamic mass and improvement of Korsakoff's syndrome. Even when a diagnosis of isolated hypothalamic histiocytosis X is confirmed, the possibility of another histiocytosis X lesion in an unexpected region must be considered.