Central diabetes insipidus in children and young adults

Background: Central diabetes insipidus is rare in children and young adults , and up to 50 percent of cases are idiopathic. The clinical presentation a nd the long-term course of this disorder are largely undefined. Methods: We studied all 79 patients with central diabetes insipidus who wer e seen at four pediatric endocrinology units between 1970 and 1996. There w ere 37 male and 42 female patients whose median age at diagnosis was 7.0 ye ars (range, 0.1 to 24.8). All patients underwent magnetic resonance imaging (MRI) and periodic studies of anterior pituitary function. The median dura tion of follow-up was 7.6 years (range, 1.6 to 26.2). Results: The causes of the central diabetes insipidus were Langerhans'-cell histiocytosis in 12 patients, an intracranial tumor in 18 patients, a skul l fracture in 2 patients, and autoimmune polyendocrinopathy in 1 patient; 5 patients had familial disease. The cause was considered to be idiopathic i n 41 patients (52 percent). In 74 patients (94 percent) the posterior pitui tary was not hyperintense on the first MRI scan obtained, and 29 patients ( 37 percent) had thickening of the pituitary stalk. Eighteen patients had ch anges in the thickness of the pituitary stalk over time, ranging from norma lization (six patients) or a decrease in thickness (one patient) to further thickening (seven patients) or thickening of a previously normal stalk (fo ur patients). Anterior pituitary hormone deficiencies, primarily growth hor mone deficiency, were documented in 48 patients (61 percent) a median of 0. 6 year (range, 0.1 to 18.0) after the onset of central diabetes insipidus. Conclusions: Most children and young adults with acquired central diabetes insipidus have abnormal findings on MRI scans of the head, which may change over time, and at least half have anterior pituitary hormone deficiencies during follow-up. (N Engl J Med 2000;343:998-1007.) (C) 2000, Massachusetts Medical Society.