Central diabetes insipidus. 22 years' experience.

The etiology of diabetes insipidus (DI) was determined in 73 children evaluated from 1962 through 1983. Intracranial tumors produced DI in 34 children, but 27 of these 34 children developed DI only after excision of the tumor. Diabetes insipidus occurred in ten children with intracranial birth defects, eight with severe central nervous system infections, and six with histiocytosis. Six had other causes. No etiology was detected in nine. Division of the cases into two time periods (1962 through 1972 and 1973 through 1983) revealed a decrease in the frequency of idiopathic DI (26.7% to 8.6%), an increase in the frequency of intracranial birth defects (0% to 17.3%), and an increase in the frequency of severe central nervous system infections (0% to 13.8%). Significant changes in therapy for DI have occurred during these 22 years. Use of desmopressin acetate has facilitated treatment of this complex management problem.