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Kim, Kim, Park, Yang, Kim, Jung, Nam, Kim, and Yun: A Case of Adipsic Hypernatremia Associated with Anomalous Corpus Callosum in Adult with Mental Retardation
Case Report
Endocrinology and Metabolism

Endocrinology and Metabolism 2012; 27(3): 232-236.

Published online: 19 September 2012

DOI: https://doi.org/10.3803/EnM.2012.27.3.232

A Case of Adipsic Hypernatremia Associated with Anomalous Corpus Callosum in Adult with Mental Retardation

Boo Gyoung Kim, Ka Young Kim, Youn Jeong Park, Keun Suk Yang, Ji Hee Kim, Hee Chan Jung, Hee Chul Nam, Young Ok Kim, Yu Seon Yun

Department of Internal Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea.

Corresponding author: Yu Seon Yun. Department of Internal Medicine, Uijeongbu St. Mary's Hospital, The Catholic University of Korea College of Medicine, 271 Cheonbo-ro, Uijeongbu 480-717, Korea. Tel: +82-31-820-3643, Fax: +82-31-847-2719, junpearl19@catholic.ac.kr

Received 14 February 2012       Accepted 30 April 2012

Copyright © 2012 Korean Endocrine Society

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Adipsic hypernatremia cause chronic hyperosmolality and hypernatremia through a combination of impaired thirst and osmotically stimulated antidiuretic hormone secretion. This syndrome can be grouped together as disorders of osmoreceptor dysfunction due to the various degrees of osmoreceptor destruction related with different types of intracranial lesions around the anterior hypothalamus, consistent with the location of primary osmoreceptor cells. Adipsic hypernatremia, associated with developmental disorder of corpus callosum, is very rare. Most cases are diagnosed at infancy and early childhood; the replacement of desmopressin is necessary. Herein, we report adipsic hypernatremia associated with anomalous corpus callosum in adult with mental retardation; they were treated with only free water without desmopressin.

Keywords: Corpus callosum, Hypernatremia, Thirst

Figures and Tables

Fig. 1
(A) The sella MRI shows abnormal architecture of bilateral frontal horns of lateral ventricle. (B) Notice the non-visualization of the septum pellucidum. (C) A small nodular lesion at the posterior aspect of the anterior pituitary lobe suggesting Rathke's cleft cyst and anomalous corpus callosum.
enm-27-232-g001
Fig. 2
Relation of antidiuretic hormone (ADH) to plasma osmolality in our patient, showing no significant correlation (bivariate correlation analysis, r = 0.262; P = 0.436).
enm-27-232-g002
Table 1
Laboratory values during water restriction
enm-27-232-i001

ADH, antidiuretic hormone; Posm, plasma osmolality; Uosm, urine osmolality.

Table 2
Laboratory values during water load test
enm-27-232-i002

ADH, antidiuretic hormone; Posm, plasma osmolality; Uosm, urine osmolality.

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