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Acquired central diabetes insipidus

Disease definition

A subtype of central diabetes insipidus (CDI) characterized by polyuria and polydipsia, due to an idiopathic or secondary decrease in vasopressin (AVP) production.

ORPHA:95626

Classification level: Subtype of disorder
  • Synonym(s):
    • Acquired CDI
    • Acquired neurogenic diabetes insipidus
  • Prevalence: Unknown
  • Inheritance: -
  • Age of onset: All ages
  • ICD-10: E23.2
  • ICD-11: 5A61.5
  • OMIM: -
  • UMLS: C5680256
  • MeSH: -
  • GARD: -
  • MedDRA: -

Summary

Epidemiology

The prevalence is unknown.

Clinical description

It occurs equally in both sexes and in all age groups but most often has an onset around the ages of 10-20. Symptoms include polyuria, polydipsia, usually associated with weight loss, and nocturia.

Etiology

Acquired CDI is secondary to a condition damaging the central nervous system or it is idiopathic (unknown etiology). Causes that lead to damage of the hypothalamus-neurohypophyseal brain region include head trauma (resulting from an accident or surgery), congenital abnormalities (pituitary stalk interruption syndrome (PSIS, see this term), infections (meningitis, encephalitis), autoimmune disorders (primary hypophisitis, see this term) and inflammatory (sarcoidosis and Wegener's granulomatosis, see these terms) or neoplastic (germinoma, meningioma, Langerhans cell histiocytosis, craniopharyngioma (see these terms), Rathke cleft cysts and metastasis). Up to 50% of CDI cases are idiopathic and the cause is unknown but autoimmunity is suspected to be involved in a number of them.

Expert reviewer(s):  Dr Stefano GHIRARDELLO - Last update: July 2012

  A summary on this disease is available in Français (2012) Deutsch (2012) Español (2012) Italiano (2012) Nederlands (2012) Português (2012) Polski (2012, pdf)

Detailed information

Guidelines

ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.