Idiopathic Intracranial Hypertension

What is idiopathic intracranial hypertension?

Idiopathic intracranial hypertension (IIH), formerly known as pseudotumor cerebri, is increased pressure around your brain. It occurs when cerebrospinal fluid (CSF), the liquid that cushions your spinal cord and brain, builds up in your skull. Pressure builds up in your brain and on your optic nerve, the nerve at the back of your eye that helps you see.

The words within the condition name mean:

• Idiopathic: No known cause
• Intracranial: In the skull
• Hypertension: High pressure

A former name for IIH is pseudotumor cerebri. Pseudotumor cerebri means “false brain tumor.” Providers used to call it this because the symptoms can be similar to a brain tumor.

Let a healthcare provider know if you notice symptoms like changes to your vision in addition to a headache or ringing in your ears. Treatment options are available for IIH.

How common is idiopathic intracranial hypertension?

IIH is rare. It affects an estimated .2 to 2 people (between ages 25 and 36) out of 100,000 in a general population study.

Is IIH a serious condition?

IIH isn’t life-threatening. However, symptoms can cause permanent vision changes that can affect your quality of life. A healthcare provider can quickly treat this condition to prevent complications.

What are the symptoms of idiopathic intracranial hypertension?

Symptoms of IIH include:

• Double vision
• Fatigue
• Headaches (sudden and severe)
• Loss of peripheral (side) vision
• Nausea and vomiting
• Shoulder and neck pain
• Temporary blindness or blind spots in vision
• Tinnitus (ringing in your ears)

Several conditions can cause these symptoms, so check with a provider to find out if these symptoms are related to IIH or something else.

What causes idiopathic intracranial hypertension?

Healthcare providers don’t know the exact cause of IIH. “Idiopathic” means that the cause is unknown.

Researchers theorize that there may be a blockage in the path cerebrospinal fluid uses to travel through your brain (CSF pathway) or a narrowing of large veins (venous sinuses) in your brain. This may cause fluid or blood to back up as it tries to exit your brain, which increases pressure.

Is idiopathic intracranial hypertension hereditary?

Studies are ongoing to learn more about whether IIH is hereditary (runs in your biological family). Some people who have the condition report family members having it, too.

What are the risk factors for idiopathic intracranial hypertension?

IIH can affect anyone. You may be more at risk of developing it if you:

• Have a body mass index (BMI) above 30
• Are assigned female at birth
• Are between ages 20 and 45

What are the complications of idiopathic intracranial hypertension?

IIH may lead to vision loss if untreated. This is irreversible. A healthcare provider can offer treatment options to prevent this complication, so let them know as soon as possible if you notice changes to your vision.

How is idiopathic intracranial hypertension diagnosed?

To diagnose IIH, a healthcare provider will take your medical history and perform a physical exam. They’ll learn more about what symptoms you experience and order several diagnostic tests to rule out conditions with similar symptoms. Tests may include:

• Eye exam with a visual field test to check for blind spots in your vision
• Brain CT scan or MRI
• Spinal tap (lumbar puncture)

How is idiopathic intracranial hypertension treated?

The goals of IIH treatment are to decrease pressure on your brain and prevent vision loss. Depending on the severity, your healthcare provider may recommend:

• Taking medications
• Undergoing surgery
• Weight management

Your provider will determine what type of treatment is best by reviewing diagnostic test results. They’re looking for areas of your brain where there may be problems with cerebrospinal fluid movement or blood flow. They’ll also assess the severity of your condition. Severe cases may require surgery to prevent permanent vision loss.

What medications treat idiopathic intracranial hypertension?

Some medicines can help you manage IIH symptoms. Your provider may prescribe:

• Acetazolamide or topiramate to help your body produce less CSF
• A diuretic (water pill) to decrease fluid retention
• Pain relievers for headaches

Let your healthcare provider know if you’re pregnant, plan on becoming pregnant or take birth control pills. They may offer alternatives for acetazolamide and topiramate due to potential side effects.

IIH surgery

In severe cases, you may need surgery for IIH. Surgical options could include:

• Shunt: A shunt is a long, thin tube placed in your brain to drain excess CSF.
• Stent: A stent is a small, mesh tube placed in your venous sinus (a large vein in your brain). The stent makes the venous sinus wider.
• Optic nerve sheath fenestration: This is an eye surgery where your provider makes small incisions around your optic nerve to allow better CSF drainage.

Weight management for IIH

To prevent IIH from happening again, your provider may suggest making lifestyle changes to improve your overall health. This could include participating in a weight management program if you have a BMI over 30.

Researchers suggest that excess body fat in your chest and abdomen creates pressure that makes it harder for blood from your brain to reach that area. As a result, fluid can collect in your brain and increase your risk of IIH.

Can idiopathic intracranial hypertension be prevented?

You can’t prevent IIH because the cause isn’t well understood. You can help reduce your risk of developing the condition by maintaining a healthy weight that’s appropriate for you. A healthcare provider can help you manage any risk factors for IIH, too.

What’s the outlook for idiopathic intracranial hypertension?

Timely treatment at the first sign of vision changes or symptoms can help reduce your risk of complications like permanent vision loss. Treatment is often successful at relieving symptoms. Your healthcare provider can give you the best information on what you can expect in your situation.

Your provider and ophthalmologist (eye doctor) will want to schedule regular check-ups to monitor your health after you receive an IIH diagnosis.

What is the life expectancy for someone with IIH?

IIH doesn’t directly affect your life expectancy. Most people have a normal life expectancy.

Does IIH ever go away?

There isn’t a cure for IIH, so you may have to manage this condition for the rest of your life.

This condition may return in the future. It’s important to follow your treatment plan, especially if your provider recommends making changes to the foods you eat or your activity level to help reduce your risk.

When should I see a healthcare provider?

Let a provider know if you notice symptoms of IIH (like a headache or ringing in your ears) with changes to your vision. This could include:

• Blurry vision
• Double vision
• Blind spots

Even if these happen for a moment and your vision returns to normal soon after, discuss your symptoms with a provider.

What questions should I ask my healthcare provider?

You may also want to ask your provider:

• Can I do anything at home to manage symptoms?
• Are there side effects of the medications you prescribed?
• What symptoms should I look out for?
• How do I prevent IIH from happening again?