Central Diabetes Insipidus (CDI)

What is central diabetes insipidus (CDI)?

Central diabetes insipidus (CDI) is a rare condition in which your body doesn’t have enough antidiuretic hormone (ADH, or vasopressin), which causes you to pee large volumes of urine and become very thirsty. Your hypothalamus produces ADH, but your pituitary gland stores and releases it. You can get central diabetes insipidus if your pituitary gland or hypothalamus is damaged.

People with diabetes insipidus pee large volumes of urine several times a day and drink large amounts of water because they feel constantly thirsty. If you have CDI and don’t drink enough fluid to replace water loss through urine, you can become dehydrated, which is dangerous to your health.

Some people may have a severe form of central diabetes insipidus (complete CDI) with little or no ADH activity. Others may have a mild form of it (partial CDI) with some ADH activity.

Central diabetes insipidus is the most common type of diabetes insipidus. Other types include:

• Nephrogenic diabetes insipidus.
• Dipsogenic diabetes insipidus.
• Gestational diabetes insipidus.

Central diabetes insipidus is not to be confused with diabetes mellitus, which is a condition in which your pancreas doesn’t make any or enough insulin or your body doesn’t use the insulin it makes properly. Your body needs insulin to transform the food you eat into energy. They share the name “diabetes” because they both cause increased thirst and frequent urination. “Diabetes” comes from the Greek word “diabainein,” which means “go through” — much like the liquids that quickly go through your body in these conditions.

What is antidiuretic hormone (ADH, or vasopressin)?

Antidiuretic hormone (ADH, or vasopressin) is a hormone that your hypothalamus makes and your pituitary gland stores and releases.

Hormones are chemicals that coordinate different functions in your body by carrying messages through your blood to your organs, muscles and other tissues. These signals tell your body what to do and when to do it.

Your hypothalamus is the part of your brain that controls your autonomic nervous system and the activity of your pituitary gland. It makes ADH and then sends it to your pituitary gland for storage and release.

Your pituitary gland is a small gland consisting of two lobes located at the base of your brain below your hypothalamus. It’s a part of your endocrine system and is in charge of making, storing and releasing many different important hormones. The posterior lobe of your pituitary gland stores and releases ADH.

ADH helps regulate the water balance in your body by controlling the amount of water your kidneys reabsorb while they filter wastes out of your blood. Your body normally produces and releases more ADH when you’re dehydrated or losing blood pressure. The increase in ADH tells your kidneys to hold onto more water instead of releasing it in your pee.

In central diabetes insipidus, your hypothalamus isn’t making enough ADH and/or your pituitary gland isn’t releasing enough ADH. This causes frequent and excessive water loss through your urine.

What’s the difference between central diabetes insipidus and nephrogenic diabetes insipidus?

Both central and nephrogenic diabetes insipidus cause the same signs and symptoms: peeing large amounts of urine and drinking a lot of fluids. The difference is in what causes the two conditions and how they’re treated.

Nephrogenic diabetes insipidus happens when your pituitary gland releases enough antidiuretic hormone (ADH, or vasopressin), but your kidneys don’t respond to it properly and can’t retain water. It can have several causes, including:

• Certain medications, such as lithium and tetracycline.
• Low levels of potassium in your blood (hypokalemia).
• High levels of calcium in your blood (hypercalcemia).
• A blocked urinary tract.

Central diabetes insipidus happens when your hypothalamus and/or pituitary gland aren’t working properly and aren’t releasing enough ADH. It’s usually caused by damage to your hypothalamus or pituitary gland, which can come from several conditions, such as a head injury, surgery, inflammation or tumors.

Who does central diabetes insipidus (CDI) affect?

Central diabetes insipidus can affect anyone at any age. The onset of CDI is more common between the ages of 10 and 20 years.

How common is central diabetes insipidus (CDI)?

Central diabetes insipidus is a rare condition. It affects approximately 1 out of every 25,000 people.

What are the symptoms of central diabetes insipidus (CDI)?

The main symptoms of central diabetes insipidus (CDI) include:

• Needing to pee often (frequent urination), including through the night.
• Passing large amounts of light-colored or clear urine each time you pee.
• Feeling very thirsty and drinking liquids very often.

While most people produce one to three quarts of urine a day, people with CDI can produce up to 20 quarts of urine a day.

Infants with central diabetes insipidus may also have the following symptoms:

• Irritability.
• Lethargy.
• Vomiting.
• Constipation.
• Fever.

If central diabetes insipidus isn’t treated or if a person with CDI stops drinking fluids, it can quickly lead to dehydration.

Symptoms of dehydration include:

• Feeling dizzy or lightheaded.
• Feeling tired.
• Having a dry mouth, lips and eyes.
• Difficulty performing simple mental tasks
• Nausea.
• Fainting.

If you’re experiencing extreme thirst and frequent urination and/or dehydration, it’s important to go to the hospital as soon as possible. While CDI is rare, it’s not the only condition that causes these symptoms. Extreme thirst and frequent urination are significant signs that something in your body isn’t working properly and needs medical treatment.

What causes central diabetes insipidus (CDI)?

Central diabetes insipidus results from a partial or complete deficiency of antidiuretic hormone (ADH, or vasopressin). This deficiency usually results from damage to your hypothalamus, pituitary gland or the tissue that connects them (pituitary stalk). Specific causes include:

• Damage to your hypothalamus or your pituitary gland from surgery.
• Damage to your hypothalamus or pituitary gland from a head injury, particularly basal skull fractures.
• Inflammation (granulomas) from sarcoidosis or tuberculosis.
• Tumors such as a craniopharyngioma or a germinoma.
• Langerhans cell histiocytosis (a rare disorder that can damage tissue or cause lesions to form in one or more places in your body).
• An inherited gene mutation on chromosome 20 (this is an extremely rare cause — fewer than 100 cases have been reported in medical literature).

In approximately one-third of CDI cases, healthcare providers can't determine the cause. This is known as idiopathic central diabetes insipidus. Researchers believe that some cases of idiopathic CDI may be caused by autoimmune factors. Autoimmune conditions happen when your body’s immune system begins to attack healthy tissue for unknown reasons. In this case, your immune system would produce antibodies or lymphocytes that attack cells that release ADH.

CDI can also occur as part of a larger syndrome or condition, including Wolfram syndrome (an inherited condition) and septo-optic dysplasia (a rare disorder of early brain development).

How is central diabetes insipidus (CDI) diagnosed?

Since other conditions, including diabetes mellitus, cause frequent thirst and urination, part of the process of diagnosing central diabetes insipidus involves ruling out other conditions. Because of this, your healthcare provider may order several tests if you’re experiencing these symptoms.

A diagnosis of central diabetes insipidus also involves determining the cause of it.

What tests will be done to diagnose central diabetes insipidus (CDI)?

A water deprivation test is the simplest and most reliable method for diagnosing central diabetes insipidus (CDI). If your provider orders this test, they will make sure you’re under constant supervision during the process since it can cause dehydration.

A water deprivation test involves not drinking any liquid for several hours to see how your body responds. If you have central diabetes insipidus, you'll continue to pee large amounts of watery (dilute), light-colored urine when normally you'd only pee a small amount of concentrated, dark yellow urine.

Your provider may also order the following tests to help diagnose CDI or rule out other conditions:

• Blood test to check antidiuretic hormone (ADH, or vasopressin) levels.
• Blood test to check glucose levels to rule out diabetes mellitus.
• Urinalysis to check osmolality (the concentration of your urine) and/or to check for ketones, which could indicate diabetes mellitus.
• A diagnostic injection of synthetic ADH to determine your kidneys’ response. People with nephrogenic diabetes insipidus don’t respond to synthetic ADH because their kidneys are resistant to the effects of ADH. On the other hand, people with CDI respond to synthetic ADH.
• Imaging tests, such as an MRI, to see what is causing damage to your pituitary gland or hypothalamus, if anything.

How is central diabetes insipidus (CDI) treated?

Treatment for CDI depends on the severity of antidiuretic hormone (ADH, or vasopressin) deficiency.

If you have complete CDI, desmopressin is the first-line treatment. It’s a medication that works like antidiuretic hormone (ADH, or vasopressin). You can take desmopressin as an injection (shot), a pill or in a nasal spray.

If you have partial CDI and still have some natural ADH activity, your healthcare provider may prescribe other medications, such as hydrochlorothiazide (HCTZ).

Providers may treat infants with CDI by diluting formula or breast milk with water and hydrochlorothiazide.

What are the side effects of desmopressin?

Desmopressin is generally very safe to use and has few side effects. Possible side effects can include:

• Headache.
• Abdominal pain.
• Nausea.
• A blocked or runny nose.
• Nosebleeds.

If you take too much desmopressin or drink too much fluid while taking it, it can cause your body to retain too much water. This can cause the following symptoms:

• Headaches.
• Dizziness.
• Feeling bloated.
• A low level of salt (sodium) in your blood (hyponatremia).

Symptoms of hyponatremia include:

• A severe or prolonged headache.
• Confusion.
• Nausea and vomiting.

If you’re experiencing symptoms of hyponatremia, call 911 or go to your nearest emergency room as soon as possible.

What can I do to manage central diabetes insipidus (CDI)?

If you have CDI, aside from medical treatment, it’s important to drink water regularly to make sure you don’t get dehydrated.

It’s also important to see your provider regularly to make sure your treatment is working and that you have the right dosage of medication.

What are the risk factors for developing central diabetes insipidus (CDI)?

Unfortunately, most cases of central diabetes insipidus (CDI) can’t be prevented. Risk factors for developing CDI include:

• Brain surgery.
• Family history of diabetes insipidus and/or Wolfram syndrome.
• Head injury.
• Infection of the brain.

What is the outlook (prognosis) for central diabetes insipidus (CDI)?

The outlook (prognosis) for central diabetes insipidus is generally good, and it usually doesn’t cause serious problems as long as it’s treated properly and you drink enough water.

The risk of complications and death is higher for infants, seniors and those who have mental health conditions, because they may have trouble recognizing their thirst or they can’t do anything about it.

What are the complications of central diabetes insipidus (CDI)?

The main complication of central diabetes insipidus is dehydration, which happens when your body loses too much fluid and electrolytes to work properly. If you have CDI, you can usually make up for the significant volume of fluids you pass in your urine by drinking more liquids. But if you don’t, you could quickly become dehydrated.

Dehydration is dangerous and can be life-threatening. If you’re experiencing symptoms of dehydration, such as feeling dizzy, nauseous and sluggish, get to the nearest hospital as soon as possible.

In infants, if CDI goes untreated, repeated episodes of dehydration can potentially result in the following complications:

• Seizures.
• Brain damage.
• Developmental delays.
• Stunted growth.

In children and adults, untreated CDI can also lead to the following complications:

• Irregular heartbeats (arrhythmia).
• Fever.
• Dry skin and mucous membranes (such as your mouth and nasal passages).
• Seizures.
• Coma.

Adults with CDI may develop orthostatic hypotension, a condition in which you experience a dramatic decrease in blood pressure upon standing or sitting. It causes dizziness or momentary loss of consciousness.

When should I see my healthcare provider?

If you have central diabetes insipidus, it’s important to see your healthcare provider regularly so that they can run tests to monitor your condition and make sure your treatment is working. If you’re experiencing any concerning symptoms, be sure to contact your provider.

If you’re experiencing symptoms of severe dehydration, such as altered consciousness and nausea, get to the nearest emergency room as soon as possible.

What questions should I ask my healthcare provider?

If you’ve been diagnosed with central diabetes insipidus (CDI), it may be helpful to ask your healthcare provider the following questions:

• What caused my CDI?
• Is my CDI chronic or temporary?
• What are my treatment options?
• What are the benefits and risks of different treatment options?
• How much water should I be drinking in a day?
• Is there anything else I can do to manage my condition?
• Are my family members at risk for developing CDI?

A note from Cleveland Clinic

Central diabetes insipidus (CDI) is a rare but serious condition. The good news is that it’s treatable and manageable. If you have CDI, it’s important to see your healthcare provider regularly to make sure your treatment is working. If you have any questions about your condition, don’t be afraid to ask your provider. They’re there to help you manage your condition.