| Neurogenic diabetes insipidus | |
|---|---|
| Classification and external resources | |
| ICD-10 | E23.2 |
| ICD-9 | 253.5 |
| MeSH | D020790 |
Neurogenic diabetes insipidus, more commonly known as central diabetes insipidus, is due to a lack of vasopressin production in the brain. Vasopressin acts to increases the volume of blood (intravascularly) and, decreases the volume of urine produced therefore a lack of it causes increased urine production and dehydration.
It is also known as "neurohypophyseal diabetes insipidus".[1][2]
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In at least twenty-five percent of cases (the most commonly occurring classification), neurogenic diabetes insipidus is idiopathic, meaning that the lack of vasopressin production arose from an unknown cause.[3] It is also due to damage of the hypothalamus, pituitary stalk, posterior pituitary, and can arise from head trauma.
The lack of vasopressin production usually results from some sort of damage to the pituitary gland. The damage to the brain could have been caused by a benign tumor (20 percent of cases), trauma (17 percent of cases), neurosurgery (9 percent of cases)[3] or some rather rare causes which include hemochromatosis, sarcoidosis, and histiocytosis.
Vasopressin is released by the posterior pituitary, but unlike most other pituitary hormones, vasopressin is produced in the hypothalamus. Neurogenic diabetes insipidus can be a failure of production at the hypothalamus, or a failure of release at the pituitary.[4]
The most rare form of central DI is familial neurogenic diabetes insipidus. This form of DI is due to an inherited mutation of the arginine vasopressin-neurophysin II (AVP-NPII) gene, inherited in an autosomal dominant manner.[5] At one point, only 45 families worldwide were known to possess this genetic trait.[6] It is now more widely recognized, although the precise number of people affected with this form of DI is unknown at the present time.
The disorder is treated with vasopressin analogs such as Desmopressin.
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