II. Definition

  1. Antidiuretic Hormone (ADH) deficiency

III. Pathophysiology

  1. Polyuria
    1. Decreased ADH release
  2. Permanent Polyuria
    1. Central lesion above median eminence
  3. Transient Polyuria
    1. Central lesion below median eminence
    2. ADH passes via Hypothalamus to portal capillaries
    3. Results in ADH release below the median eminence

IV. Etiology

  1. Idiopathic (30%)
    1. Autoimmune disease (common)
      1. Lymphocyte inflammation
        1. Pituitary stalk (thickened stalk on MRI)
        2. Posterior pituitary
      2. Anterior Pituitary deficiency
        1. Growth Hormone
        2. ACTH deficient
    2. Familial Diabetes Insipidus (very rare)
      1. Point mutation in ADH precursor gene
        1. Precursor accumulates
        2. Toxicity to ADH synthesizing cells
      2. Enhancement within Hypothalamus on MRI
  2. Severe Traumatic Brain Injury
    1. Hypothalamus or pituitary injury
  3. Neurosurgery (Transsphenoidal)
    1. Results from Hypothalamus or Pituitary Trauma
    2. Most common cause of polyuria post neurosurgery
    3. Differential Diagnosis
      1. Excess fluids
      2. Mannitol
      3. Corticosteroids
    4. Approach
      1. Check Urine Osmolality
      2. Observe response to water restriction
  4. Malignancy
    1. Examples: Lung Cancer, Leukemia, Lymphoma
    2. Polyuria may be presenting symptom
  5. Langerhans Histiocytosis (Histiocytosis X)
    1. Infiltrative disease
    2. Sarcoidosis causes similar infiltration
  6. Post SVT resolution
  7. Anorexia Nervosa
  8. Pregnancy exacerbates any of above forms

V. Diagnosis

  1. Fluid Deprivation Test
    1. No response to water deprivation
    2. Response to exogenous ADH administration
  2. Hare-Hickey Test
    1. Decreased ADH to Serum Osmolality ratio

VI. Radiology: MRI Head

  1. Central DI: Diminished signal at posterior pituitary

VII. Management

  1. General Measures that potentiate ADH
    1. Low Sodium diet
    2. NSAIDs
  2. dDAVP (Desmopressin)
    1. Synthetic ADH replacement hormone
    2. dDAVP 10 to 20 ug bid intranasally
  3. Chlorpropamide 125-250 mg PO qd-bid
    1. Antidiuretic effect - may lower Urine output by 50%
    2. Risk of Hypoglycemia at higher doses
  4. Carbamazepine 100-300 mg bid
    1. Enhances ADH response
    2. May lower urine output by 50%
  5. Hydrochlorothiazide with low salt intake
    1. Decreases polyuria
    2. Dose: 25 mg qd to bid

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Ontology: Central Diabetes Insipidus (C0687720)

Definition (CHV) a lack of antidiuretic hormone that causes excessive production of very dilute urine
Definition (CHV) a lack of antidiuretic hormone that causes excessive production of very dilute urine
Definition (CHV) a lack of antidiuretic hormone that causes excessive production of very dilute urine
Definition (CHV) a lack of antidiuretic hormone that causes excessive production of very dilute urine
Definition (CHV) a lack of antidiuretic hormone that causes excessive production of very dilute urine
Definition (CHV) a lack of antidiuretic hormone that causes excessive production of very dilute urine
Definition (CHV) a lack of antidiuretic hormone that causes excessive production of very dilute urine
Definition (NCI) Diabetes insipidus caused by decreased secretion of antidiuretic hormone from the pituitary gland.
Definition (MSH) A genetic or acquired polyuric disorder caused by a deficiency of VASOPRESSINS secreted by the NEUROHYPOPHYSIS. Clinical signs include the excretion of large volumes of dilute URINE; HYPERNATREMIA; THIRST; and polydipsia. Etiologies include HEAD TRAUMA; surgeries and diseases involving the HYPOTHALAMUS and the PITUITARY GLAND. This disorder may also be caused by mutations of genes such as ARVP encoding vasopressin and its corresponding neurophysin (NEUROPHYSINS).
Concepts Disease or Syndrome (T047)
MSH D020790
SnomedCT 15771004, 190484000, 64410003, 267393007, 154699008, 45369008
Dutch hypofysaire diabetes insipidus, centrale diabetes insipidus, antidiuretisch hormoon-deficiëntie, diabetes; insipidus, hypofysair, hypofyse; diabetes insipidus, Diabetes insipidus, neurogene, Neurogene diabetes insipidus
French Diabète insipide pituitaire, Déficit d'hormone anti-diurétique, Diabète insipide neurogénique, Diabète insipide central, Diabète insipide hypophysaire, Diabète insipide neuro-hypophysaire
German hypophysaerer Diabetes insipidus, zentraler Diabetes insipidus, Antidiuretisches-Hormon-Mangel, Diabetes insipidus, neurogener
Italian Diabete insipido ipofisario, Diabete insipido centrale, Diabete insipido neuroipofisale, Deficit di ormone antidiuretico, Diabete insipido neurogeno
Portuguese Diabetes insípida hipofisária, Diabetes insípida central, Deficiência de hormona antidiurética, Diabetes Insípida Neurogênica, Diabetes Insípido Neurogênico
Spanish Diabetes insípida neurohipofisaria, Diabetes insípida central, Deficiencia de hormona antidiurética, diabetes insipidus secundaria a deficiencia de vasopresina, síndrome de deficiencia de vasopresina (trastorno), síndrome de deficiencia de vasopresina, diabetes insípida craneal, diabetes insípida neurogénica, diabetes insípida neurohipofisaria (trastorno), diabetes insípida neurohipofisaria, diabetes insípida pituitaria, diabetes insípida primaria central, Diabetes Insípida Neurogénica
Japanese 下垂体性尿崩症, カスイタイセイニョウホウショウ, チュウスウセイニョウホウショウ, 中枢性尿崩症, 尿崩症-神経原性, 神経原性尿崩症, コウリニョウホルモンケツボウショウ, 抗利尿ホルモン欠乏症, 尿崩症-神経性, 神経性尿崩症
Swedish Diabetes insipidus, neurogen
English DIABETES INSIPIDUS, NEUROHYPOPHYSEAL TYPE, DIABETES INSIPIDUS, PRIMARY CENTRAL, DIABETES INSIPIDUS, CRANIAL TYPE, antidiuretic hormone defective syndrome, vasopressin defective diabetes insipidus, pituitary diabetes insipidus, Central Diabetes Insipidus, Diabetes Insipidus, Central, Diabetes Insipidus, Neurogenic, Diabetes Insipidus, Neurohypophyseal, Diabetes Insipidus, Pituitary, DIABETES INSIPIDUS, NEUROHYPOPHYSEAL, Diabetes Insipidus, Neurogenic [Disease/Finding], cranial diabetes insipidus, diabete insipidus, diabetes insipidus, neurogenic diabetes insipidus, central diabetes insipidus, vasopressin deficiency, CDI, Diabetes Insipidus, Neurohypophyseal Type, Diabetes Insipidus, Primary Central, Diabetes Insipidus, Cranial Type, Antidiuretic hormone deficiency, Vasopressin Deficiency, Pituitary Diabetes Insipidus, Diabetes Insipidus Cranial Type, Vasopressin Defective Diabetes Insipidus, Neurohypophyseal Diabetes Insipidus, Diabetes Insipidus Primary Central, Diabetes Insipidus Secondary To Vasopressin Deficiency, Central diabetes insipidus (disorder), Vasopressin deficiency syndrome (disorder), Neurohypophyseal diabetes insipidus (diagnosis), Cranial diabetes insipidus, Neurogenic diabetes insipidus, Pituitary diabetes insipidus, Primary central diabetes insipidus, Vasopressin deficiency syndrome, Central diabetes insipidus, Diabetes insipidus - pituitary, Vasopressin deficiency, Diabetes insipidus secondary to vasopressin deficiency, Neurohypophyseal diabetes insipidus (disorder), Neurohypophyseal diabetes insipidus, diabetes; insipidus, pituitary, pituitary; diabetes insipidus, Neurohypophyseal diabetes insipidus, NOS, Central diabetes insipidus [Ambiguous], Neurogenic Diabetes Insipidus
Czech diabetes insipidus neurogenní, Hypofyzární diabetes insipidus, Centrální diabetes insipidus, Deficit antidiuretického hormonu
Finnish Neurogeeninen diabetes insipidus
Russian DIABET NESAKHARNYI NEVROGENNYI, DIABET NESAKHARNYI NEIROGENNYI, ДИАБЕТ НЕСАХАРНЫЙ НЕВРОГЕННЫЙ, ДИАБЕТ НЕСАХАРНЫЙ НЕЙРОГЕННЫЙ
Polish Moczówka prosta neurogenna
Hungarian Centralis diabetes insipidus, Hypophysaer diabetes insipidus, Antidiuretikus hormon hiánya
Norwegian Diabetes insipidus, neurogen, Neurogen diabetes insipidus