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Kim, Kang, Kim, Jeong, Kim, Kim, and Kim: Retrospective Observation of Long-Term Clinical Courses of Idiopathic Central Diabetes Insipidus in Adults
Original Articles
Journal of Korean Endocrine Society

Journal of Korean Endocrine Society 2006; 21(6): 482-489.

Published online: 20 December 2006

DOI: https://doi.org/10.3803/jkes.2006.21.6.482

Retrospective Observation of Long-Term Clinical Courses of Idiopathic Central Diabetes Insipidus in Adults

Hee Joung Kim, Mi Yeon Kang, Kyung Won Kim, Hyun Seung Jeong, Hae Sung Kim, Sang Wan Kim, Seong Yeon Kim

Department of Internal Medicine, Seoul National University College of Medicine, Korea.

Received 22 June 2006       Accepted 30 October 2006

Copyright © 2006 Korean Endocrine Society

Abstract

Background

Idiopathic central diabetes insipidus (CDI) can be diagnosed when it occurs in the absence of a genetic or secondary cause known to be responsible for diabetes insipidus (DI). Some studies have reported that idiopathic CDI in adults shows a more benign clinical course than in children and young patients. However, the clinical characteristics and progress of this disorder have not been fully described. Therefore, we investigated the clinical courses of adult patients over the age of sixteen years with idiopathic central DI.

Methods

We reviewed the medical records of all patients who had documented cases of idiopathic CDI from 1989 to 2005, and studied clinical features, hormone data, and imaging studies at diagnosis and during at least 1-year of follow-up.

Results

There were 9 male (30.0%) and 21 female (70.0%) patients with a mean age of 39.3 years at diagnosis and a mean follow-up duration of 6.9 years. At diagnosis, deficits in anterior pituitary hormones were documented in 6 patients (20%), hyperprolactinemia in 4, and hypogonadism in 2. Two patients had an anterior pituitary hormone deficiency that was newly detected at a mean 3.4 years after the onset of DI. On initial MRI, the posterior pituitary was not hyperintense in 7 of the 30 patients (23.3%), but pituitary stalk thickening was observed in 15 (50.0%). After a mean follow-up of 6.9 years (range: 1 to 18), follow-up pituitary MRI showed improvement or no changes in patients with initial MRI findings of a pituitary abnormality, and no development of new lesions in 7 patients with a normal pituitary finding on initial MRI.

Conclusion

Two of the 30 patients with idiopathic CDI developed an anterior pituitary hormone deficiency during follow-up, but no subject showed any aggravation on follow-up MRI. No patient showed a newly developed pituitary abnormality on follow-up MRI after a negative finding on the initial MRI

Keywords: Anterior pituitary hormone, Clinical course, Idiopathic diabetes insipidus, MRI, Pituitary

Figures and Tables

Fig. 1
Age distribution of patients with idiopathic central diabetes insipidus. Our study showed the peak in the age-group 30-39.
jkes-21-482-g001
Table 1
Clinical courses of central diabetes insipidus in 7 patients with endocrine hormone abnormality
jkes-21-482-i001

PRL, prolactin; GHD, growth hormone deficiency; GnTHD, gonadotropin deficiency

Table 2
Changes of brain MRI and anterior pituitary hormones in patients with pituitary stalk thickening at presentation
jkes-21-482-i002

PRL, prolactin; GHD, growth hormone deficiency; GnTHD, gonadotropin deficiency.

Table 3
Changes of brain MRI and anterior pituitary hormones in patients with normal pituitary stalk thickness at presentation
jkes-21-482-i003

GHD, growth hormone deficiency; GnTHD, gonadotropin deficiency.

Table 4
Clinical courses of central diabetes insipidus in 2 patients who had discontinued DDAVP medication
jkes-21-482-i004

References

1. Kim BW. Idiopathic central diabetes insipidus in adult. J Kor Soc Endocrinol. 2001. 16:185–189.
2. Maghnie M, Cosi G, Genovese E, Manca-Bitti ML, Cohen A, Zecca S, Tinelli C, Gallucci M, Bernasconi S, Boscherini B, Severi F, Arico M. Central diabetes insipidus in children and young adults. N Engl J Med. 2000. 343:998–1007.
3. Mootha SL, Barkovich AJ, Grumbach MM, Edwards MS, Gitelman SE, Kaplan SL, Conte FA. Idiopathic hypothalamic diabetes insipidus, pituitary stalk thickening, and the occult intracranial germinoma in children and adolescents. J Clin Endocrinol Metab. 1997. 82:1362–1367.
4. Maghnie M, Villa A, Arico M, Larizza D, Pezzotta S, Beluffi G, Genovese E, Severi F. Correlation between magnetic resonance imaging of posterior pituitary and neurohypophyseal function in children with diabetes insipidus. J Clin Endocrinol Metab. 1992. 74:795–800.
5. Maghnie M, Bossi G, Klersy C, Cosi G, Genovese E, Arico M. Dynamic endocrine testing and magnetic resonance imaging in the long-term follow-up of childhood langerhans cell histiocytosis. J Clin Endocrinol Metab. 1998. 83:3089–3094.
6. Imura H, Nakao K, Shimatsu A, Ogawa Y, Sando T, Fujisawa I, Yamabe H. Lymphocytic infundibuloneurohypophysitis as a cause of central diabetes insipidus. N Engl J Med. 1993. 329:683–689.
7. Maghnie M, Altobelli M, Di Iorgi N, Genovese E, Meloni G, Manca-Bitti ML, Cohen A, Bernasconi S. 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. 2004. 89:1891–1896.
8. Blumberg DL, Sklar CA, Wisoff J, David R. Abnormalities of water metabolism in children and adolescents following craniotomy for a brain tumor. Childs Nerv Syst. 1994. 10:505–508.
9. Wang LC, Cohen ME, Duffner PK. Etiologies of central diabetes insipidus in children. Pediatr Neurol. 1994. 11:273–277.
10. Pomarede R, Czernichow P, Finidori J, Pfister A, Roger M, Kalifa C, Zucker JM, Pierre-Kahn A, Rappaport R. Endocrine aspects and tumoral markers in intracranial germinoma: an attempt to delineate the diagnostic procedure in 14 patients. J Pediatr. 1982. 101:374–378.
11. Leger J, Velasquez A, Garel C, Hassan M, Czernichow P. Thickened pituitary stalk on magnetic resonance imaging in children with central diabetes insipidus. J Clin Endocrinol Metab. 1999. 84:1954–1960.
12. Jeon HJ, Park SH, Kwon SH, Lee SH, Park KS, Kim SY, Lee HK. Clinical course of idiopathic central diabetes insipidus in adults. J Kor Soc Endocrinol. 2001. 16:190–198.
13. 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. 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. 2003. 88:1629–1636.
14. Charmandari E, Brook CG. 20 years of experience in idiopathic central diabetes insipidus. Lancet. 1999. 353:2212–2213.
15. Scherbaum WA, Bottazzo GF. Autoantibodies to vasopressin cells in idiopathic diabetes insipidus: evidence for an autoimmune variant. Lancet. 1983. 1:897–901.
16. Nijenhuis M, van den Akker EL, Zalm R, Franken AA, Abbes AP, Engel H, de Wied D, Burbach JP. Familial neurohypophysial diabetes insipidus in a large Dutch kindred: effect of the onset of diabetes on growth in children and cell biological defects of the mutant vasopressin prohormone. J Clin Endocrinol Metab. 2001. 86:3410–3420.
17. Maghnie M. Diabetes insipidus. Horm Res. 2003. 59:suppl 1. 42–54.
18. Ghirardello S, Malattia C, Scagnelli P, Maghnie M. Current perspective on the pathogenesis of central diabetes insipidus. J Pediatr Endocrinol Metab. 2005. 18:631–645.
19. Greger NG, Kirkland RT, Clayton GW, Kirkland JL. Central diabetes insipidus. 22 year's experience. Am J Dis Child. 1986. 140:551–554.
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