central diabetes insipidus; diabetes insipidus, neurohypophyseal type (CDI)
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Introduction
A deficiency of antidiuretic hormone (ADH) or vasopressin.
Etiology
- hypoxic or ischemic encephalopathy
- head trauma
- post hypophysectomy/pituitary ablation
- idiopathic suprasellar or intrasellar tumors or cysts
- anorexia nervosa
- hypothalamic/pituitary infiltration
- CNS infection
- encroachment by aneurysm
- Sheehan's syndrome
- Guillain-Barre syndrome
- fat embolus
- empty sella
- following correction of supraventricular tachycardia[2]
- familial disorder
- autoimmune hypophysitis
- 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
- autosomal dominant neurohypophyseal diabetes insipidus
- autosomal recessive neurohypophyseal diabetes insipidus
Clinical manifestations
- persistent thirst, polydipsia & polyuria
Laboratory
- severe urine concentrating deficit
- urine osmolality < 100 mosm/kg
- 24 hour urine - volumes > 3 L/day
- urine concentrating test
- response of urine osmolality to water deprivation if diagnosis unclear
- water deprivation tests are stopped when the serum osmolality is > 295 mOsm/kg H2O or the serum sodium is > 145 mEq/L[2][7]
- close monitoring indicated
- urine osmolality normally increases with water deprivation
- urine osmolality does not increase after water deprivation with central DI
- response of urine osmolality to water deprivation if diagnosis unclear
- serum osmolality is elevated
- desmopressin stimulation test
- when urine does NOT concentrate with water deprivation
- an increased urine osmolality indicates central DI
- a lack of response indicates nephrogenic DI
- patients with primary polydipsia may show response similar to nephrogenic DI due to washout of the medullary concentration gradient
- serum sodium:
- free water diuresis leads to high normal serum sodium if fluid intake adequate but hypernatremia if fluid intake is compromised [6]
- ADH in plasma is low[2]
- plasma copeptin distinguishes central diabetes insipidus from polydipsia[2]
- antigen & activity low in central diabetes insipidus
*also see ARUP consult
Differential diagnosis
Management
- general (see diabetes insipidus)
- desamino-D-arginine vasopressin dDAVP 5-10 ug QD or BID intranasally
- use cautiously in patients with coronary artery disease
- avoid water retention & hyponatremia
- administer QHS to prevent nocturia
- adjust dose to permit daily breakthrough polyuria thus avoiding iatrogenic SIADH
- chlorpropamide potentiates effects of dDAVP
- avoid hypoglycemia
More general terms
Additional terms
- syndrome of inappropriate antidiuretic hormone; SIADH; nephrogenic syndrome of inappropriate antidiuresis; NSIAD
- urine osmolality
- vasopressin (antidiuretic hormone, ADH)
References
- ↑ Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 50-51
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 Medical Knowledge Self Assessment Program (MKSAP) 11, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2012, 2015, 2018, 2022.
Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025 - ↑ Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 239
- ↑ 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: https://pubmed.ncbi.nlm.nih.gov/18226738
- ↑ ARUP Consult:Central Diabetes Insipidus (Posterior Pituitary) Testing Algorithm https://arupconsult.com/algorithm/central-diabetes-insipidus-posterior-pituitary-testing-algorithm
- ↑ 6.0 6.1 NEJM Knowledge+ Endocrinology
- ↑ 7.0 7.1 Tomkins M, Lawless S, Martin-Grace J, et al. Diagnosis and management of central diabetes insipidus in adults. J Clin Endocrinol Metab. 2022;107:2701-2715. PMID: https://pubmed.ncbi.nlm.nih.gov/35771962
Patient information
central diabetes insipidus patient information