FREE NEJM E-TOC    HOME   |   SUBSCRIBE   |   CURRENT ISSUE   |   PAST ISSUES   |   COLLECTIONS   |   Search Term  Advanced Search

Sign in | Get NEJM's E-Mail Table of Contents — Free | Subscribe

Previous Volume 329:683-689 September 2, 1993 Number 10 Next

	Full Text Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited PubMed Citation

ABSTRACT

Background Central diabetes insipidus may be familial, secondary to hypothalamic or pituitary disorders, or idiopathic. Idiopathic central diabetes insipidus is characterized by selective hypofunction of the hypothalamic-neurohypophysial system, but its cause is unknown.

Methods We studied 17 patients with idiopathic diabetes insipidus, in whom the duration of the disorder ranged from 2 months to 20 years. Only four patients had been treated with vasopressin before the study began. All the patients underwent endocrinologic studies and magnetic resonance imaging (MRI) with a 1.5-T superconducting unit, and two patients had biopsies of the neurohypophysis or the pituitary stalk.

Results Nine of the 17 patients had thickening of the pituitary stalk, enlargement of the neurohypophysis, or both and lacked the hyperintense signal of the normal neurohypophysis. In the remaining eight patients, the pituitary stalk and the neurohypophysis were normal, although the hyperintense signal was absent. The abnormalities of thickening and enlargement were seen on MRI only in the patients who had had diabetes insipidus for less than two years, and the abnormalities disappeared during follow-up, suggesting a self-limited process. In addition to vasopressin deficiency, two patients had mild hyperprolactinemia and nine had impaired secretory responses of growth hormone to insulin-induced hypoglycemia. The two biopsies revealed chronic inflammation, with infiltration of lymphocytes (mainly T lymphocytes) and plasma cells.

Conclusions Diabetes insipidus can be caused by lymphocytic infundibuloneurohypophysitis, which can be detected by MRI. The natural course of the disorder is self-limited.

Source Information

From the Departments of Medicine (H.I., K.N., A.S., Y.O., T.S.) and Radiology (I.F.), Kyoto University Faculty of Medicine, and the Laboratory of Anatomical Pathology, Kyoto University Hospital (H.Y.), both in Kyoto, Japan.

Address reprint requests to Dr. Imura at Kyoto University, Kyoto 606-01, Japan.

Full Text of this Article

This article has been cited by other articles:

• Chamberlain, J. J., Small, D. (2008). Successful Use of a Single Subcutaneous Continuous Glucose Monitor Sensor for 28 Days in a Patient with Type 1 Diabetes. Clin. Diabetes 26: 138-139 [Full Text]  
• Alderson, P, Sutcliffe, K, Curtis, K (2006). Children as partners with adults in their medical care. Arch. Dis. Child. 91: 300-303 [Abstract] [Full Text]  
• Ikema, T, Takasu, N (2005). Serial T1-weighted magnetic resonance imaging changes in a patient with central diabetes insipidus, possibly due to lymphocytic infundibuloneurohypophysitis. Eur J Endocrinol 153: 989-990 [Full Text]  
• Panicker, H. K., Janicic, N., Nguyen, D., Verbalis, J. (2005). Presumed Infundibuloneurohypophysitis: Unusual Presentation in a Postpartum Patient. Am. J. Neuroradiol. 26: 357-359 [Abstract] [Full Text]  
• Asahina, M., Suzuki, A., Tamura, N., Yoshida, T., Hattori, T. (2004). Acquired generalized anhidrosis associated with lymphocytic infundibuloneurohypophysitis. Neurology 63: 1752-1752 [Full Text]  
• Skyler, J. S. (2004). Effects of Glycemic Control on Diabetes Complications and on the Prevention of Diabetes. Clin. Diabetes 22: 162-166 [Abstract] [Full Text]  
• Maghnie, M., Altobelli, M., di Iorgi, N., Genovese, E., Meloni, G., Manca-Bitti, M. L., Cohen, A., Bernasconi, S. (2004). Idiopathic Central Diabetes Insipidus Is Associated with Abnormal Blood Supply to the Posterior Pituitary Gland Caused by Vascular Impairment of the Inferior Hypophyseal Artery System. J. Clin. Endocrinol. Metab. 89: 1891-1896 [Abstract] [Full Text]  
• (2004). Nausea and vomiting in a patient with increasing thirst. Postgrad. Med. J. 80: 185-185 [Full Text]  
• Skyler, J. S (2004). DCCT: the study that forever changed the nature of treatment of type 1 diabetes: Diabetes Control and Complications Trial -- a glycaemic control study in type 1 diabetes. British Journal of Diabetes & Vascular Disease 4: 29-32  
• Pivonello, R., De Bellis, A., Faggiano, A., Di Salle, F., Petretta, M., Di Somma, C., Perrino, S., Altucci, P., Bizzarro, A., Bellastella, A., Lombardi, G., Colao, A. (2003). Central Diabetes Insipidus and Autoimmunity: Relationship between the Occurrence of Antibodies to Arginine Vasopressin-Secreting Cells and Clinical, Immunological, and Radiological Features in a Large Cohort of Patients with Central Diabetes Insipidus of Known and Unknown Etiology. J. Clin. Endocrinol. Metab. 88: 1629-1636 [Abstract] [Full Text]  
• La Greca, A. M., Bearman, K. J. (2002). The Diabetes Social Support Questionnaire-Family Version: Evaluating Adolescents' Diabetes-Specific Support From Family Members. J Pediatr Psychol 27: 665-676 [Abstract] [Full Text]  
• DAFNE Study Group, (2002). Training in flexible, intensive insulin management to enable dietary freedom in people with type 1 diabetes: dose adjustment for normal eating (DAFNE) randomised controlled trial. BMJ 325: 746-746 [Abstract] [Full Text]  
• Tubridy, N, Saunders, D, Thom, M, Asa, S L, Powell, M, Plant, G T, Howard, R (2001). Infundibulohypophysitis in a man presenting with diabetes insipidus and cavernous sinus involvement. J. Neurol. Neurosurg. Psychiatry 71: 798-801 [Abstract] [Full Text]  
• Cheung, C. C., Ezzat, S., Smyth, H. S., Asa, S. L. (2001). The Spectrum and Significance of Primary Hypophysitis. J. Clin. Endocrinol. Metab. 86: 1048-1053 [Abstract] [Full Text]  
• Ken, H. (2001). Translating wish lists to blueprints: A National Service Framework for diabetes. British Journal of Diabetes & Vascular Disease 1: 57-61 [Abstract]  
• Madsen, J. R., Karluk, D. (2000). Case 34-2000- A 71-Year-Old Woman with an Enlarging Pituitary Mass. NEJM 343: 1399-1406 [Full Text]  
• Maghnie, M., Cosi, G., Genovese, E., Manca-Bitti, M. L., Cohen, A., Zecca, S., Tinelli, C., Gallucci, M., Bernasconi, S., Boscherini, B., Severi, F., Arico, M. (2000). Central Diabetes Insipidus in Children and Young Adults. NEJM 343: 998-1007 [Abstract] [Full Text]  
• De Bellis, A., Colao, A., Di Salle, F., Muccitelli, V. I., Iorio, S., Perrino, S., Pivonello, R., Coronella, C., Bizzarro, A., Lombardi, G., Bellastella, A. (1999). A Longitudinal Study of Vasopressin Cell Antibodies, Posterior Pituitary Function, and Magnetic Resonance Imaging Evaluations in Subclinical Autoimmune Central Diabetes Insipidus. J. Clin. Endocrinol. Metab. 84: 3047-3051 [Abstract] [Full Text]  
• Leger, J., Velasquez, A., Garel, C., Hassan, M., Czernichow, P. (1999). Thickened Pituitary Stalk on Magnetic Resonance Imaging in Children with Central Diabetes Insipidus. J. Clin. Endocrinol. Metab. 84: 1954-1960 [Abstract] [Full Text]  
• Greiner, F. G., Takhtani, D. (1999). Neuroradiology Case of the Day. RadioGraphics 19: 826-829 [Full Text]  
• Mahler, R. J., Adler, M. L. (1999). Type 2 Diabetes Mellitus: Update on Diagnosis, Pathophysiology, and Treatment. J. Clin. Endocrinol. Metab. 84: 1165-1171 [Full Text]  
• Li, J.-Y., Lai, P.-H., Lam, H.-C., Lu, L.-Y., Cheng, H.-H., Lee, J.-K., Lo, Y.-K. (1999). Hypertrophic cranial pachymeningitis and lymphocytic hypophysitis in Sjogren's syndrome. Neurology 52: 420-420 [Abstract] [Full Text]  
• Baylis, P. H, Cheetham, T. (1998). Diabetes insipidus. Arch. Dis. Child. 79: 84-89 [Full Text]  
• Crock, P. A. (1998). Cytosolic Autoantigens in Lymphocytic Hypophysitis. J. Clin. Endocrinol. Metab. 83: 609-618 [Abstract] [Full Text]  
• Gagliardi, P. C., Bernasconi, S., Repaske, D. R. (1997). Autosomal Dominant Neurohypophyseal Diabetes Insipidus Associated with a Missense Mutation Encoding Gly23->Val in Neurophysin II. J. Clin. Endocrinol. Metab. 82: 3643-3646 [Abstract] [Full Text]  
• Mootha, S. L., Barkovich, A. J., Grumbach, M. M., Edwards, M. S., Gitelman, S. E., Kaplan, S. L., Conte, F. A. (1997). Idiopathic Hypothalamic Diabetes Insipidus, Pituitary Stalk Thickening, and the Occult Intracranial Germinoma in Children and Adolescents. J. Clin. Endocrinol. Metab. 82: 1362-1367 [Abstract] [Full Text]  
• Hasegawa, G., Hunter, A. J., Charonis, A. S. (1995). Matrix Nonenzymatic Glycosylation Leads to Altered Cellular Phenotype and Intracellular Tyrosine Phosphorylation. J. Biol. Chem. 270: 3278-3283 [Abstract] [Full Text]  
• Samuels, M. A., de la Monte, S. (1994). Case 36-1994- A 49-Year-Old Man with Hypopituitarism, Multifocal Neurologic Defects, and an Intracranial Mass. NEJM 331: 861-868 [Full Text]