Intracranial Calcification in Siblings with Nephrogenic Diabetes Insipidus: CT and MRI
| Title: | Intracranial Calcification in Siblings with Nephrogenic Diabetes Insipidus: CT and MRI |
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| Authors: | Tohyama, J.; Inagaki, M.; Koeda, T.; Ohno, K.; Takeshita, K. |
| Publisher: | Neuroradiology |
| Date Published: | 1993 |
| Reference Number: | 145 |
Computed tomography and magnetic resonance imaging (MRI) were used to examine three male siblings with nephrogenic diabetes insipidus (NDI). The two elder brothers had varying degrees of unusual intracranial calcification; the eldest also showed involvement of the cerebral white matter on MRI. The severity of intracranial calcification was related to the time before initiation of treatment and inversely to mental ability. Brain damage and mental retardation in NDI may be caused by a delay in initiating treatment; early detection and treatment are important to prevent brain damage.
This translation by the NDI Foundation is to assist the lay reader. To provide a clear, accessible interpretation of the original article, we eliminated or simplified some technical detail and complicated scientific language. We concentrated our translation on those aspects of the article dealing directly with NDI. The NDI Foundation thanks the researchers for their work toward understanding and more effectively treating this disorder.
© Copyright NDI Foundation 2007 (JC)
The youngest brother showed signs of NDI shortly after his first week of birth and was diagnosed and treated for CDNI at one month of age. He was treated with hydrochlorothiazide and his sodium intake was restricted. At four years of age, he was developing normally and CT and MRI showed no signs of brain tissue calcification.
The authors reasoned the biochemical abnormalities (such as the calcification in the brain) produced by the genetic defect causing CDNI should be the same in all three brothers because they all had the same mother. The difference in the amount of brain tissue calcification among the brothers was attributed to the frequency and severity of CNDI-triggered dehydration episodes experienced by the brothers. The eldest brother experienced the most severe dehydration because he did not begin treatment until he was almost three years old, whereas the second brother began treatment at eight months and the youngest brother at one month. The authors speculated that high levels of sodium in the blood resulting from CDNI-caused dehydration contributed to brain calcification. They also suspect that repeated episodes of dehydration caused by high sodium levels in the blood may slow blood flow in the brains resulting in nerve cell death and hemorrhaging of brain blood vessels.
The study clearly shows that CNDI may cause brain tissue calcification and organic brain damage, and they advise early diagnosis and effective treatment to avoid such a possibility.