Endocrinol Metab.  2012 Sep;27(3):232-236. 10.3803/EnM.2012.27.3.232.

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

• Kim BG
• Kim KY
• Park YJ
• Yang KS
• Kim JH
• Jung HC
• Nam HC
• Kim YO
• Yun YS

• KMID: 2282408
• DOI: http://doi.org/10.3803/EnM.2012.27.3.232

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.

Keyword

Corpus callosum; Hypernatremia; Thirst

MeSH Terms

Adult
Corpus Callosum
Deamino Arginine Vasopressin
Humans
Hypernatremia
Hypothalamus, Anterior
Intellectual Disability
Thirst
Water
Deamino Arginine Vasopressin
Water

Figure

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  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).

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