Central diabetes insipidus

Central diabetes insipidus
Synonyms	Neurogenic diabetes insipidus, Neurohypophyseal diabetes insipidus
Pronounce	N/A
Specialty	N/A
Symptoms	Polyuria, polydipsia, nocturia
Complications	Dehydration, electrolyte imbalance
Onset	Any age
Duration	Long-term
Types	N/A
Causes	Head trauma, pituitary surgery, genetic mutations, autoimmune disorders
Risks	Family history, brain tumors, infections
Diagnosis	Water deprivation test, urine osmolality, serum osmolality
Differential diagnosis	Nephrogenic diabetes insipidus, psychogenic polydipsia, diabetes mellitus
Prevention	N/A
Treatment	Desmopressin, thiazide diuretics, low-salt diet
Medication	N/A
Prognosis	Good with treatment
Frequency	Rare
Deaths	N/A

Other Names: Diabetes insipidus neurogenic; Neurohypophyseal diabetes insipidus; Pituitary diabetes insipidus; Diabetes insipidus neurohypophyseal; Diabetes insipidus cranial type; Neurogenic diabetes insipidus Central diabetes insipidus (DI) is a form of DI that occurs when the body has lower than normal levels of antidiuretic hormone (vasopressin), which is characterized by frequent urination. Central DI results from damage to the pituitary gland, which disrupts the normal storage and release of antidiuretic hormone (ADH). When this hormone reaches the kidneys, it directs them to make less urine.

Cause

• Diabetes insipidus (DI) is an uncommon condition in which the kidneys are unable to prevent the excretion of water. DI is a different disease than diabetes, though both share common symptoms of excessive urination and thirst.
• Central diabetes insipidus is a form of DI that occurs when the body has a lower than normal amount of antidiuretic hormone (ADH). ADH is also called vasopressin. ADH is produced in a part of the brain called the hypothalamus. ADH is then stored and released from the pituitary gland. This is a small gland at the base of the brain.
• ADH controls the amount of water excreted in urine. Without ADH, the kidneys do not work properly to keep enough water in the body. The result is a rapid loss of water from the body in the form of dilute urine. This results in the need to drink large amounts of water due to extreme thirst and to make up for excessive water loss in the urine (10 to 15 liters a day).
• The reduced level of ADH may be caused by damage to the hypothalamus or pituitary gland. This damage may be due to surgery, infection, inflammation, tumor, or injury to the brain.
• In rare cases, central diabetes insipidus is caused by a genetic problem.

Signs and symptoms

The major symptoms of central diabetes insipidus (DI) include urinating too much (polyuria), getting up at night to urinate (nocturia), and drinking too much liquids (polydipsia). For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 80%-99% of people have these symptoms

• Anorexia
• Dehydration
• Diabetes insipidus
• Failure to thrive(Faltering weight)
• Nocturia
• Polydipsia(Extreme thirst)
• Weight loss

30%-79% of people have these symptoms

• Anxiety(Excessive, persistent worry and fear)
• Depressivity(Depression)
• Excessive daytime somnolence(Excessive daytime sleepiness)
• Fever
• Headache(Headaches)
• Lethargy

5%-29% of people have these symptoms

• Diarrhea(Watery stool)
• Hyponatremia(Low blood sodium levels)
• Nausea and vomiting
• Seizure

Diagnosis

The health care provider will ask about your medical history and symptoms. Tests that may be ordered include:

• Blood sodium and osmolarity
• Desmopressin (DDAVP) challenge
• MRI of the head
• Urinalysis
• Urine concentration
• Urine output

Treatment

The cause of the underlying condition will be treated.

• Vasopressin (desmopressin, DDAVP) is given either as a nasal spray, tablets, or injections. This controls urine output and fluid balance and prevents dehydration.
• In mild cases, drinking more water may be all that is needed. If the body's thirst control is not working (for example, if the hypothalamus is damaged), a prescription for a certain amount of water intake may also be needed to ensure proper hydration.

Prognosis

Outcome depends on the cause. If treated, central diabetes insipidus usually does not cause severe problems or result in early death. Possible Complications Not drinking enough fluids can lead to dehydration and electrolyte imbalance. When taking vasopressin and your body's thirst control is not normal, drinking more fluids than your body needs can cause dangerous electrolyte imbalance.

NIH genetic and rare disease info

• NIH info on Central diabetes insipidus

Central diabetes insipidus is a rare disease.

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