Idiopathic intracranial hypertension (IIH) — formerly known as pseudotumor cerebri, which means "false brain tumor" — is a condition that occurs when a buildup of cerebrospinal fluid causes increased pressure inside the skull. Its symptoms include headache, vision and hearing problems, or double vision — similar to those caused by a large brain tumor.
UPMC experts provide advanced care for people with IIH, including prompt diagnosis and treatment that reduces your risk of complications.
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What Is Idiopathic Intracranial Hypertension (IIH)?
Idiopathic intracranial hypertension (IIH), formerly known as pseudotumor cerebri, is a condition that occurs when a buildup of cerebrospinal fluid (CSF) causes increased pressure inside the skull. Symptoms of IIH include headache, vision and hearing problems, nausea, and vomiting — similar to those caused by a large brain tumor.
How common is idiopathic intracranial hypertension (IIH)?
IIH is a rare condition that affects up to two out of 100,000 people in the general population.
What causes idiopathic intracranial hypertension?
Researchers aren’t sure what causes IIH. However, it may be caused by a problem in your brain that prevents CSF from circulating normally.
Idiopathic intracranial hypertension risk factors
IIH can affect anyone, but you may be at increased risk if you are:
- Between 20 and 45 years of age.
- Female.
- Obese.
- Taking hormones.
- Taking certain medications, including cycline antibiotics or retinoic acid derivatives.
Complications of idiopathic intracranial hypertension
If left untreated, IIH can cause permanent vision loss in both eyes due to damage to the optic nerves.
How serious is idiopathic intracranial hypertension?
IIH isn’t life-threatening, but it is a sight-threatening medical emergency. You should seek treatment immediately.
How can I reduce my risks of idiopathic intracranial hypertension?
You can’t control all of your risk factors for IIH. However, maintaining a healthy weight and body fat percentage may lower your risk of developing IIH or prevent it from happening again.
What Are the Signs and Symptoms of idiopathic Intracranial Hypertension?
Symptoms of IIH mimic the symptoms of a large brain tumor and may include:
- Headaches that worsen when lying down.
- Double vision.
- Loss of peripheral (side) vision in both eyes.
- New blind spots in your vision.
- Pulsating heartbeat.
- Temporary vision loss, triggered by changes in head position.
When should I see a doctor about my idiopathic intracranial hypertension symptoms?
Idiopathic intracranial hypertension is a medical emergency. If left untreated, it can lead to permanent vision loss in both eyes. Symptoms of IIH can be similar to other serious health conditions, such as a stroke or brain tumor, making an accurate diagnosis essential to reducing your risk of complications.
If you have symptoms of IIH, dial 911 or ask someone to drive you to the nearest hospital Emergency Department.
How Do You Diagnose Idiopathic Intracranial Hypertension?
To diagnose IIH, your doctor will ask about your symptoms. They'll do a thorough physical exam and eye exam with dilating drops and order diagnostic tests.
What to expect during your visit
If your doctor suspects IIH, they will:
- Ask about your symptoms.
- Perform a physical exam.
- Dilate your eyes to see your optic nerves
- Perform a neurologic exam.
- Order diagnostic tests.
- Review your health history and medications.
Tests to diagnose idiopathic intracranial hypertension (IIH)
Your doctor may order diagnostic tests, including:
- Brain magnetic resonance imaging (MRI) — A test that uses a magnetic field and radio waves to create images of your brain.
- Eye exam — A test to check the health of your eyes.
- MRV or CTV - Tests similar to an MRI or CT, but using technology that creates images of the veins that draw CSF from the brain to look for a blockage or clot.
- Lumbar puncture (spinal tap) — A test that involves measuring the spinal fluid pressure.
- Visual field test — A test to check for blind spots in your vision.
- OCT and fundus photography — Tests that create images of the back of your eye, including the retina and optic nerves.
Idiopathic intracranial hypertension prognosis
Although IIH isn’t usually life-threatening, getting an accurate diagnosis and fast treatment is key to reducing the risk of permanent vision loss caused by the condition. The majority of patients have good visual outcomes with treatment. You may need to make lifestyle changes and receive lifelong follow-up care to reduce your risk of recurrence.
How Do You Treat Idiopathic Intracranial Hypertension?
If you are diagnosed with IIH, the goal of treatment is to relieve pressure inside your skull and on your optic nerves and reduce your risk of vision loss.
Treatment options may include:
Medication for idiopathic intracranial hypertension
Your doctor may prescribe medications to:
- Decrease pressure on your optic nerves.
- Decrease fluid retention in your body.
- Reduce the amount of CSF your body produces.
- Relieve headache pain.
In cases where the symptoms are severe and don't respond to weight loss or medications, surgical treatment may be required to relieve the pressure and to preserve vision.
Surgery to treat idiopathic intracranial hypertension
If your IIH symptoms are severe or do not respond to medication, you doctor may recommend a surgical procedure, such as:
- Optic nerve sheath fenestration — An eye surgery that involves making incisions around your optic nerve to reduce pressure, promote CSF drainage, and lower the risk of permanent vision loss.
- Stent — Involves using a small tube to prop open a vein in your brain called the venous sinus. It reduces pressure in your brain by allowing blood and CSF to drain more effectively.
- Ventriculoperitoneal shunt — Involves placing a tube in your brain to continuously drain excess CSF and reduce pressure. Shunts are placed using state-of-the-art image guidance.
Lifestyle changes
Getting ongoing follow-up care and making healthy lifestyle choices that support weight management may reduce your risk of recurrence. These treatments allow most patients to have a normal life and normal vision.
Your doctor may recommend lifestyle changes, including:
- Avoiding excessive alcohol use.
- Eating a healthy diet.
- Exercising regularly.
- Maintaining a healthy weight and body fat percentage.
- Managing your blood pressure, diabetes, and other chronic conditions.
- Quitting smoking.
- Stopping certain medications or hormone treatments.
Why Choose UPMC for Idiopathic Intracranial Hypertension Care?
When you choose UPMC for pseudotumor cerebri care, you will receive:
- Access to world-class expertise — Our world-renowned experts care for people with pseudotumor cerebri and other neuro-ophthalmologic disorders.
- A full range of treatment options — We offer treatment and follow-up care to reduce the risk of complications.
- Multidisciplinary care — We partner with ophthalmologists, neurosurgeons, neurologists, vision rehabilitation specialists, and other medical experts to provide complete care that optimizes your quality of life.
