Hypothalamic Diabetes Insipidus

Hypothalamic diabetes insipidus, or HDI, is a form of central diabetes insipidus. HDI is a rare condition that originates in the hypothalamus and causes extremely high levels of thirst. Individuals consume a greater quantity of fluids to relieve that thirst, which results in high levels of urination.

In extreme instances, it is not unusual for a person with HDI to pass over 20 liters of urine in a single day.

What Causes Hypothalamic Diabetes Insipidus?

HDI occurs when the kidneys are unable to perform water excretion. It is different from diabetes mellitus, though the symptoms of excessive thirst and urination are similar. The reason why the kidneys are unable to perform their necessary functions is that the hypothalamus does not produce enough ADH.

ADH, which is an antidiuretic hormone, is also called “vasopressin.” The hypothalamus is directly responsible for triggering the production of this hormone. Once produced, ADH is stored within the pituitary gland. It is released whenever there is a need to retain water. For people suffering from HDI, however, the release is not experienced or not enough of the hormone is being produced.

Without ADH, there is a rapid loss of fluids from the body because the kidneys are not being told to retain them. This causes the body to purge fluids, which results in the symptoms of always being thirsty.

Symptoms to Expect with Hypothalamic Diabetes Insipidus

Outside of the excessive thirst and frequent urination, HDI is known to cause changes to a person’s alertness, perception, and thinking. In many people, dehydration occurs frequently because incoming fluids are not absorbed enough to replace the outgoing fluids. Confusion, dizziness, and fatigue are experienced quite often when suffering from HDI.

If a person with HDI is unable to get enough to drink for an extended period, they may experience symptoms such as dry skin, a severe headache, and a dry mouth.

HDI that is left untreated can eventually lead to severe health concerns that may be life threatening. Although uncommon, there is the possibility of experiencing the following with this condition.

  • Hypovolemic Shock. This occurs because of a low blood volume within the circulatory system. Low fluid levels can cause a drop in blood pressure, which then drops the amount of oxygen that circulates through the body.
  • Seizures. Sodium and potassium are two important electrolytes that are replenished with fluid uptake. They help to carry electrical impulses to each cell and can become out of balance when dehydration occurs. Should these impulses not reach their intended destination, a seizure can be the result. Involuntary muscle twitches are common with prolonged dehydration as well.
  • Kidney Health. With the high volume of fluids passing through the kidneys, there is the possibility of an infection occurring. Kidney stones can still form despite the high fluid levels as well. Prolonged issues with HDI may even lead to organ failure.
  • Cramping. It is not unusual for people with HDI to experience cramping in their calves and quad muscles, as if they had been working out for a long time. Cramping in the side can also occur.

How to Treat Hypothalamic Diabetes Insipidus Successfully

Treating HDI requires a multi-faceted approach. The symptoms of the condition must be addressed at the same time the lack of ADH is addressed. That may mean restoring an electrolyte balance, introducing more fluids into the body, and various testing to determine the health status of the kidneys.

For many people with HDI, the condition can be successfully treated with a replacement hormone called “desmopressin.” It may also be referred to as DDAVP. It may be given via injection for the first treatments to restore balance to the body. Long-term treatment plans usually offer desmopressin through tablets or a nasal spray.

Within 2-3 weeks, the hormone can replace what is not being distributed by the pituitary gland and reduce the amount of fluids that are being passed through the body.

In mild cases of hypothalamic diabetes insipidus, no desmopressin may be necessary. Some medical providers may ask individuals to drink more water to restore fluid levels within the body.

Many cases of HDI may not be prevented. It may occur through an injury to the hypothalamus or occur because of a genetic condition. Certain infections and tumors may be increase the risk of experiencing the symptoms of HDI, as may certain surgeries.

The prognosis of an HDI diagnosis is generally good. The outcome depends upon the severity of the condition. For most people, it will not cause severe health issues or increase the risk of early death.

As with any medical concern, be sure to speak with your local doctor or medical provider if you believe that HDI may be causing your bothersome symptoms.