Central diabetes insipidus

Not to be confused with Nephrogenic diabetes insipidus.

Central diabetes insipidus
Other namesArginine vasopressin deficiency; AVP-D; pituitary diabetes insipidus; neurohypophyseal diabetes insipidus; cranial diabetes insipidus; neurogenic diabetes insipidus
SpecialtyEndocrinology
SymptomsPolyuria, nocturia, and polydipsia.
ComplicationsDehydration, seizures
Usual onsetAny age
Diagnostic methodUrine tests, blood tests, fluid deprivation test
Differential diagnosisAVP-R (nephrogenic), Diabetes mellitus
TreatmentDrinking sufficient fluids
MedicationDesmopressin
Frequency3 per 100,000 per year

Central diabetes insipidus, recently renamed arginine vasopressin deficiency (AVP-D),[1] is a form of diabetes insipidus that is due to a lack of vasopressin (ADH) 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,[2][3] referring to the posterior pituitary (neurohypophysis), which receives vasopressin from the hypothalamus in the brain, via the hypothalamo-hypophyseal tract in the pituitary stalk. This condition has only polyuria in common with diabetes. Although not mutually exclusive, with most typical cases, the name diabetes insipidus is misleading.[4]

Untreated patients with central diabetes insipidus often experience polyuria, nocturia, and polydipsia due to the initial increase in serum sodium and osmolality. Central diabetes insipidus can be caused by various congenital or acquired lesions, and when the cause is unknown, it is classified as idiopathic.

The water deprivation test (WDT) is a commonly used test for diabetes insipidus, a two-step process involving parenteral desmopressin administration after an initial 8-hour water fast. It differentiates primary polydipsia from diabetes insipidus and central diabetes insipidus from nephrogenic diabetes insipidus. Diabetes insipidus is trated by restoring free water deficit, replacing the missing hormone, and addressing the underlying ailment. Desmopressin, an arginine vasopressin analog, is used to treat central diabetes insipidus.

  1. ^ "Diabetes Insipidus is changing its name: Arginine Vasopressin Deficiency". Pituitary Foundation. 2022-11-17. Retrieved 2024-01-31.
  2. ^ Chitturi S, Harris M, Thomsett MJ, Bowling F, McGown I, Cowley D, et al. (December 2008). "Utility of AVP gene testing in familial neurohypophyseal diabetes insipidus". Clinical Endocrinology. 69 (6): 926–930. doi:10.1111/j.1365-2265.2008.03303.x. PMID 18494865. S2CID 22350358.
  3. ^ Lee YW, Lee KW, Ryu JW, Mok JO, Ki CS, Park HK, et al. (2008). "Mutation of Glu78 of the AVP-NPII gene impairs neurophysin as a carrier protein for arginine vasopressin in a family with neurohypophyseal diabetes insipidus". Annals of Clinical and Laboratory Science. 38 (1): 12–14. PMID 18316776.
  4. ^ "The Pathogenesis of Diabetes Insipidus". Journal of the American Medical Association. 49 (6): 499. 1907. doi:10.1001/jama.1907.02530060049004.