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central diabetes insipidus; diabetes insipidus, neurohypophyseal type (CDI)

A deficiency of antidiuretic hormone (ADH) or vasopressin. Etiology: 1) hypoxic or ischemic encephalopathy 2) head trauma 3) post hypophysectomy/pituitary ablation 4) idiopathic suprasellar or intrasellar tumors or cysts a) craniopharyngioma b) neurofibroma 5) anorexia nervosa 6) hypothalamic/pituitary infiltration a) granulomatous disease 1] sarcoidosis 2] histiocytosis X 3] Wegener's granulomatosis [2] b) lymphocytic hypophysitis c) IgG4 disease [2] 7) CNS infection a) tuberculosis b) meningoencephalitis 8) encroachment by aneurysm 9) Sheehan's syndrome 10) Guillain-Barre syndrome 11) fat embolus 12) empty sella 13) following correction of supraventricular tachycardia [2] 14) familial disorder 15) autoimmune hypophysitis 16) idiopathic Pathology: - complete or partial deficiency of antidiuretic hormone (ADH) or vasopressin secretion by the posterior pituitary (neurohypophysis) - disruption of preprovasopressin processing Genetics: - cases of primary central diabetes insipidus are associated with mutations in the gene for preprovasopressin & arginine vasopressin a) autosomal dominant neurohypophyseal diabetes insipidus b) autosomal recessive neurohypophyseal diabetes insipidus Clinical manifestations: - persistent thirst, polydipsia & polyuria Laboratory: 1) severe urine concentrating deficit 2) urine osmolality < 100 mosm/kg 3) 24 hour urine - volumes > 3 L/day 4) urine concentrating test a) response of urine osmolality to water deprivation b) close monitoring indicated c) urine osmolality normally increases with water deprivation d) urine osmolality does not increase after water deprivation with central DI 5) serum osmolality is elevated 6) desmopressin stimulation test a) when urine does NOT concentrate with water deprivation b) an increased urine osmolality indicates central DI c) a lack of response indicates nephrogenic DI d) patients with primary polydipsia may show response similar to nephrogenic DI due to washout of the medullary concentration gradient 7) serum sodium: - free water diuresis leads to high normal serum sodium if fluid intake adequate but hypernatremia if fluid intake is compromised [6] 8) ADH in plasma is low [2] 9) plasma copeptin distinguishes central diabetes insipidus from polydipsia [2] - antigen & activity low in central diabetes insipidus *also see ARUP consult Differential diagnosis: - psychogenic polydipsia Management: 1) general (see diabetes insipidus) 2) desamino-D-arginine vasopressin dDAVP 5-10 ug QD or BID intranasally a) use cautiously in patients with coronary artery disease b) avoid water retention & hyponatremia c) administer QHS to prevent nocturia d) adjust dose to permit daily breakthrough polyuria thus avoiding iatrogenic SIADH 3) chlorpropamide potentiates effects of dDAVP - avoid hypoglycemia

Related

syndrome of inappropriate antidiuretic hormone; SIADH; nephrogenic syndrome of inappropriate antidiuresis; NSIAD urine osmolality vasopressin (antidiuretic hormone, ADH)

General

diabetes insipidus (DI) genetic disease of the central nervous system hypothalamic disease

Database Correlations

OMIM correlations MORBIDMAP 192340

References

  1. Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 50-51
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2012, 2015, 2018, 2022.
  3. Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 239
  4. Loh JA, Verbalis JG. Disorders of water and salt metabolism associated with pituitary disease. Endocrinol Metab Clin North Am. 2008 Mar;37(1):213-34 PMID: 18226738
  5. ARUP Consult:Central Diabetes Insipidus (Posterior Pituitary) Testing Algorithm https://arupconsult.com/algorithm/central-diabetes-insipidus-posterior-pituitary-testing-algorithm
  6. NEJM Knowledge+ Endocrinology