Journal List > Endocrinol Metab > v.27(3) > 1085961

Endocrinol Metab. 2012 Sep;27(3):232-236. Korean.
Published online September 19, 2012.  https://doi.org/10.3803/EnM.2012.27.3.232
Copyright © 2012 Korean Endocrine Society
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 and 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, Email: junpearl19@catholic.ac.kr
Received February 14, 2012; Accepted April 30, 2012.

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


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.
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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).
Click for larger image

Tables


Table 1
Laboratory values during water restriction
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Table 2
Laboratory values during water load test
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