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Neurogenic diabetes insipidus

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Neurogenic diabetes insipidus

Not to be confused with Nephrogenic diabetes insipidus.
Neurogenic diabetes insipidus
Classification and external resources
ICD-10 9 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 increase the volume of blood (intravascularly), and decrease the volume of urine produced. Therefore, a lack of it causes increased urine production and volume depletion.

It is also known as "neurohypophyseal diabetes insipidus".[1][2]

Causes

Idiopathic

In at least 25% 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.

Acquired

The lack of vasopressin production usually results from some sort of damage to the pituitary gland. It may be caused due to damage to the brain caused by:

  • Benign suprasellar tumors (20% of cases)[3]
  • Infections (encephalitis, tuberculosis etc.)
  • Trauma (17% of cases)[3] or neurosurgery (9% of cases)[3]
  • Non-infectious granuloma (sarcoidosis, Hand-Schuller Christian disease etc.)
  • Leukaemia
  • Autoimmune - associated with thyroiditis
  • Other rare causes which include hemochromatosis 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]

Genetic

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.

There is also an X-linked familial form.

Wolfram Syndrome (also called DIDMOAD) is characterised by DI, diabetes mellitus nerve deafness and optic atrophy.

Treatment

The disorder is treated with vasopressin analogs such as Desmopressin.

See also

References

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