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What Is Idiopathic Intracranial Hypertension?

Idiopathic intracranial hypertension (IIH) essentially means that there is high blood pressure in the brain. In the past, this was also referred to as pseudotumor cerebri. Why the pressure inside the brain increases is not well understood.

Idiopathic intracranial hypertension can occur in both adults and children but is most common in obese or overweight women of childbearing age.

The symptoms of the disorder are often vague and can be confused with a brain tumor, stroke or infection. The diagnosis of idiopathic intracranial hypertension is confirmed when no other cause is found.

If high blood pressure in the brain is missed, or treatment is delayed, the optic nerve could be damaged, potentially resulting in permanent vision loss.

It can also lead to paralysis if the pressure inside the brain is very high.

Today, the majority of people who have this disorder are treated with blood pressure medications or drainage of the fluid.

 

What Are Symptoms Of Idiopathic Intracranial Hypertension?

The symptoms and signs of the disorder may include:

  • A slow progressive headache that may be moderate to severe in intensity. It normally starts right behind the eyes and worsens with eye movements.
  • Moderate to severe episodes of nausea and vomiting.
  • Sensation of dizziness or feeling faint.
  • Hearing a ringing sound or feeling a pulsatile sensation in the ears.
  • Blurred or double vision
  • Transient loss of vision.
  • Temporary weakness in the arms or legs.
  • Vague pains in the upper body and neck area
  • General feeling of fatigue.

 

What Causes Idiopathic Intracranial Hypertension?

The exact cause of this disorder is a mystery. However, recent research suggests that it may be due to excess production of the fluid that surrounds the brain. This fluid, known as cerebrospinal fluid, cushions the brain against injury.

When the production of cerebrospinal fluid is increased or its excretion is decreased, it can lead to increased pressure in the brain. The increased pressure then presses on sensitive nerves which control the eyes, body movements, hearing, taste, smell and so on.

What Are Risk Factors For Idiopathic Intracranial Hypertension?

Some of the risk factors include:

  • Being overweight. Obese females of childbearing age are at the highest risk for this disorder
  • Use of certain medications like excess vitamin A, growth hormone and tetracycline.
  • Having other health problems. Anemia, lupus, sleep apnea underactive parathyroid gland, kidney failure and polycystic ovarian syndrome have also been linked to this disorder.

Asides from obesity, the majority of patients who develop idiopathic intracranial hypertension have no other risk factors.

 

How Is Diagnosis Of Idiopathic Intracranial Hypertension Made?

Idiopathic means that the cause is unknown and therefore diagnosis of IIH is difficult to make. The symptoms are often vague and mistaken for a brain tumor or even a stroke.

In an attempt to rule out other conditions,

  • The fluid in the brain will be sampled by performing a lumbar puncture to rule out an infection.

If idiopathic intracranial hypertension is suspected the healthcare provider will also perform an eye exam and look for swelling of the optic nerve (called papilledema). The visual field will also be tested to check for blind spots.

 

How Is Idiopathic Intracranial Hypertension Treated?

Once the diagnosis of idiopathic intracranial hypertension is made, the goal of treatment is to lower the pressure inside the brain. The treatment depends on whether the patient is an outpatient or an inpatient (usually those with acute symptoms).

Inpatients are usually admitted to the ICU where they can be closely monitored. The aim is to quickly reduce the pressure in the brain and lower the risk of permanent injury to the eye. The pressure is often lowered with the use of medications, draining the fluid, or inserting a shunt.

Outpatients who have IIH with mild or no symptoms are treated on a less urgent basis. Medications to lower the pressure in the brain include water pills which work quite rapidly. Most patients see improvement within days.

If the patient is also obese, a weight loss program is recommended. This is usually done in consultation with a dietitian. As weight is lost, the symptoms generally start to decrease.

The initial approach to weight loss is with the use of diet and exercise.

In general there is no specific diet for idiopathic intracranial hypertension. It is important to remember that there is no specific food that can lower pressure inside the brain. However, since most patients who acquire idiopathic intracranial hypertension are obese, then a low calorie diet is recommended. The foods to avoid include synthetic and processed foods. Avoid saturated fats and heavy consumption of oily foods.

Foods that you should include in your diet include fruits, vegetables, whole grains, nuts and low fat dairy products. It is important not to eat more than 2,000 calories per day. Extremely low calorie diets are not recommended because most are not sustainable for more than a few weeks.

Plenty of water should be drunk and the amount of caffeinated beverages and alcohol consumed should be limited.

If this approach fails, then the healthcare provider may start the individual on weight loss medications or recommend weight loss surgery. Weight loss surgery is the fastest way to treat idiopathic intracranial hypertension but the surgery is also fraught with complications and is expensive. Continuing exercises and maintaining a healthy diet are vital after surgery to prevent weight regain.

All patients with IIH are followed up for 1-3 years to ensure that the symptoms are not recurring. At each follow up exam, a complete neurological exam is done, the eyes are checked and in some cases an MRI scan of the brain is obtained.

 

Idiopathic Intracranial Hypertension And Pregnancy

Some pregnant women develop IIH during the second or third trimester but it can occur at any time. The disorder is often confused with eclampsia or meningitis and can lead to a spontaneous abortion if not treated.

As IIH is often associated with a headache and vision changes, an astute healthcare provider can make the diagnosis. Once diagnosed, treatment is required.

Medical therapy for idiopathic intracranial hypertension during pregnancy involves performing repeated lumbar punctures to drain the fluid from the brain, which quickly lowers the pressure inside the brain.

If the female is in the 2nd or 3rd trimester she may be given water pills or steroids. In extremely rare cases, surgery is required to drain the fluid in women who fail to respond to medications and spinal fluid drainage. However, surgery does require anesthesia and this can be detrimental to the infant.

Once the condition is controlled, a cesarean section is not always required. Subsequent pregnancies do not increase the risk of intracranial hypertension.

 

What Is Prognosis For Idiopathic Intracranial Hypertension?

If the diagnosis of idiopathic intracranial hypertension is made quickly and there is no vision damage, the prognosis is excellent. If the diagnosis is made late, then the prognosis depends on the amount of visual damage and other neurological deficits.

If the patient has already developed weakness in the extremities, they will need physical and occupational therapy to regain muscle strength and endurance.

If vision has been lost, there is no guarantee it will come back.

All patients who have had a diagnosis of idiopathic intracranial hypertension should regularly see a healthcare provider to ensure that they are not developing recurrent symptoms.

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