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For further information, see CMDT Part 28-02: Central Diabetes Insipidus

KEY FEATURES

Essentials of Diagnosis

  • Antidiuretic hormone (ADH) deficiency causes central diabetes insipidus (DI) with polyuria (2–20 L/d) and polydipsia

  • Hypernatremia occurs if fluid intake is inadequate

General Considerations

  • DI is an uncommon disease caused by a deficiency of or resistance to ADH, also known as vasopressin, from the posterior pituitary

  • Primary central DI

    • No lesion on MRI of pituitary and hypothalamus

    • Accounts for about one-third of cases of DI

    • May be familial, idiopathic, or due to autoimmunity

    • Reversible central diabetes insipidus can occur

      • With administration of ketamine, temozolomide, or the anti PD-L1 monoclonal antibody avelumab

      • In the myelodysplastic preleukemic phase of acute myelogenous leukemia

  • Secondary central DI

    • Most commonly due to damage to hypothalamus or pituitary stalk by

      • Tumor

      • Hypophysitis

      • Infarction

      • Hemorrhage

      • Anoxic encephalopathy

      • Traumatic brain injury

      • Surgery involving the pituitary or hypothalamus

    • Adipsic central diabetes insipidus

      • Damage to the anterior hypothalamic thirst center

      • Commonly occurs after neurosurgical clipping of an anterior communicating artery aneurysm

    • Other causes

      • Infection (eg, encephalitis, tuberculosis, syphilis)

      • Craniopharyngioma

      • Germinoma

      • Granulomas (sarcoidosis or Langerhans cell granulomatosis)

  • Metastases to the pituitary or infundibulum cause DI more often than do pituitary adenomas (33% versus 1%)

  • Nephrogenic DI

    • Caused by unresponsiveness of the kidney tubules to the normal secretion of vasopressin

    • Congenital nephrogenic DI is

      • Familial, X-linked

      • Due to defective expression of renal vasopressin V2 receptors or vasopressin-sensitive water channels

    • Acquired form is less severe and is seen in

      • Pyelonephritis

      • Renal amyloidosis

      • Plasma cell myeloma

      • Hypokalemia

      • Sjögren syndrome

      • Sickle cell anemia

      • Chronic hypercalcemia

      • Recovery from acute tubular necrosis

    • Nephrogenic DI may also be induced by drugs

      • Corticosteroids

      • Diuretics

      • Demeclocycline

      • Lithium

      • Foscarnet

      • Methicillin

  • Vasopressinase-induced DI

    • Associated with oligohydramnios, preeclampsia, or liver dysfunction, and in the puerperium

    • Maternal circulating vasopressin is destroyed by placental vasopressinase; synthetic desmopressin is unaffected

Demographics

  • In familial autosomal dominant central DI, symptoms begin at about age 2 years

CLINICAL FINDINGS

Symptoms and Signs

  • Intense thirst, especially for ice water

  • Polyuria

  • 2 L to 20 L of fluid ingested daily, with corresponding urine volumes

  • Most patients maintain fluid balance

  • However, dehydration and hypernatremia occur if patients do not have free access to water or if the anterior hypothalamic thirst center is damaged

  • Partial DI presents with less intense symptoms and should be suspected in unremitting enuresis

  • Wolfram syndrome

    • DI can occur in this rare, autosomal-recessive disorder

    • Also known by the acronym DIDMOAD (diabetes insipidus, type 1 diabetes mellitus, optic atrophy, and deafness)

    • Manifestations usually present in childhood but may not occur until adulthood, along with depression and cognitive problems

Differential Diagnosis

  • Polyuria caused by

    • Psychogenic polydipsia

    • Diabetes mellitus

    • Cushing syndrome

    • Hypercalcemia

    • Hypokalemia

    • Nocturnal polyuria of Parkinson disease

DIAGNOSIS

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