Central diabetes insipidus resulting from a nonneoplastic tiny mass lesion localized in the neurohypophyseal system.

With the advent of magnetic resonance imaging (MRI), the neurohypophyseal system can be clearly delineated and its functional integrity can be predicted. The authors describe seven cases of central diabetes insipidus (DI) that occurred spontaneously. MRI revealed that the normal hyperintensity of the pituitary posterior lobe, which has been thought to be the neurosecretory material containing antidiuretic hormone, was absent in all cases. In addition, enlargement of a part of the neurohypophyseal system was recognized in five of seven cases on MRI. Three of the five patients with enlargement of a part of the neurohypophyseal tract underwent biopsy and were demonstrated to have chronic inflammation of the neurohypophyseal system. It was demonstrated that the enlarged parts of the neurohypophyseal system had shrunk either spontaneously or after the biopsy in four of the five cases. All patients are alive and have not experienced progression or remission of the disease. This study indicates that some cases of idiopathic DI result from a tiny mass lesion, usually nonneoplastic, localized in the neurohypophyseal system.