Pseudotumor Cerebri

Overview

What is pseudotumor cerebri?

Pseudotumor cerebri is a condition in your skull. For unknown reasons, too much cerebrospinal fluid (CSF) builds up in the skull and creates pressure.

The phrase “pseudotumor cerebri” translates to “false brain tumor” because the symptoms are similar. However, it doesn’t mean you have a tumor. It’s also called idiopathic intracranial hypertension, benign intracranial hypertension or intracranial venous hypertension.

If the condition isn’t treated, it can lead to visual impairment.

Who might get idiopathic intracranial hypertension?

Benign intracranial hypertension can develop in people of both sexes and all ages. For reasons that scientists don’t entirely understand, it’s much more common in women who are overweight of reproductive age.

The condition occurs in about 1 person in 100,000 of the general U.S. population. It’s 20 times more common in women who are 20 to 44 years old and weigh 20% more than their ideal body weight.

Symptoms and Causes

What causes pseudotumor cerebri?

Cerebrospinal fluid (CSF) surrounds and cushions your brain and spinal cord. It also delivers nutrients to your brain and removes waste from it. Idiopathic intracranial hypertension (pseudotumor cerebri) occurs when your body produces too much CSF or doesn’t absorb enough of it back into the system. As CSF accumulates, pressure increases within your skull.

Scientists don’t understand what causes CSF to build up in the skull or why pseudotumor cerebri often affects people who are overweight. But, researchers have noted some other factors that might make people more likely to develop the condition:

  • Having smaller veins that drain blood away from their brain (the venous sinuses).
  • Stopping corticosteroids or growth hormones, particularly in children.
  • Taking tetracycline antibiotics, large amounts of vitamin A, oral contraceptives or lithium.

What are the symptoms of pseudotumor cerebri?

The symptoms of pseudotumor cerebri may go unnoticed at first, but they usually worsen over time without treatment. These can include:

Diagnosis and Tests

How is pseudotumor cerebri diagnosed?

If a healthcare professional suspects you have pseudotumor cerebri, a diagnosis might include:

  • Physical exam: During a physical examination, your healthcare provider asks you about your symptoms and medical history. They examine your body and measure your vital signs.
  • Eye exam: An ophthalmologist (eye doctor) may use a tool to look inside of your eye. This pain-free test magnifies the interior of your eye to obtain a better picture. Alternatively, they may use a test called perimetry. This test uses a visual stimulus (like a flash of light) and records your eye’s responses.
  • Imaging tests: Computed tomography (CT) or magnetic resonance imaging (MRI) takes pictures inside of your head to rule out other problems
  • Spinal tap: A spinal tap (lumbar puncture) involves inserting a needle into your lower back to sample the CSF. It can tell how much pressure is building up around your brain and spinal cord. This test can also analyze the fluid to rule out other causes (like infections). Some of the fluid can also be removed to help relieve the pressure.
  • Venography: After a special contrast material is injected into your veins, CT, MRI or X-ray machines then take pictures of your veins. This test can show whether your venous sinuses are narrow or blocked, which could be another reason for the increased pressure.

Management and Treatment

How is pseudotumor cerebri treated?

The goals of treatment for pseudotumor cerebri are to:

  • Preserve vision.
  • Reduce pressure inside of your skull.
  • Relieve symptoms (such as headaches).

If left untreated, vision loss can be permanent. You should have frequent follow-up eye exams to monitor for any changes in your vision.

Your healthcare provider may recommend the following strategies to help reduce the pressure and relieve symptoms:

  • Acetazolamide, a medication that reduces the amount of CSF your body produces.
  • Diuretics (water pills) to lessen fluid in your brain.
  • Pain relievers such as ibuprofen or acetaminophen.
  • Repeat spinal taps to remove excess CSF and relieve the pressure.
  • Weight loss of at least 5% to 10% of your total body weight, if appropriate.

If those treatments aren’t adequate, you may need surgery to reduce pressure in your skull:

  • Optic nerve sheath fenestration: This surgery cuts slits into the covering of your optic nerve behind your eyeball. The slits allow CSF to escape to relieve pressure.
  • Shunt implantation: This procedure places a device in the spaces of your brain or spinal cord to drain extra CSF into another part of your body, often your abdomen, where your body can absorb it.
  • Stent implantation: If a narrowing is found in one of your veins, this procedure places a tube (stent) to widen it.

Prevention

How can I reduce my risk of pseudotumor cerebri?

The best way to reduce the risk of pseudotumor cerebri is to maintain a healthy weight. The condition is 20 times more likely in people who are overweight.

If you’re taking any of the following medications that have been associated with this condition, your healthcare provider can help determine whether they’re necessary:

Outlook / Prognosis

What is the outlook for people with pseudotumor cerebri?

With treatment, weight loss and regular eye exams, many people don’t develop permanent vision loss.

Does pseudotumor cerebri return after treatment?

Idiopathic intracranial hypertension (pseudotumor cerebri) recurs (returns) in about 10% to 20% of people. Even if treatment is successful, you should visit your healthcare provider and ophthalmologist for regular checkups.

Living With

How do I take care of myself and manage the symptoms of pseudotumor cerebri?

One of the best things you can do to manage the condition is to lose extra body weight with diet and exercise. Sometimes, even a small incremental loss in weight can eliminate symptoms. You also might consider limiting salt, which can cause your body to retain fluid.

If you have pseudotumor cerebri, it’s essential to have regular checkups with your ophthalmologist. Eye exams can detect problems even before you notice vision loss.

A note from Cleveland Clinic

Pseudotumor cerebri occurs when too much cerebrospinal fluid (CSF) accumulates in your skull and creates pressure. Anyone can develop the condition, but it’s much more common in women who are overweight. If left untreated, this can cause vision loss. But weight loss and certain treatments can reduce the pressure in your skull and relieve symptoms.

Last reviewed by a Cleveland Clinic medical professional on 03/30/2022.

References

  • Intracranial Hypertension Research Foundation. What Is IH? (https://ihrfoundation.org/what-is-ih) Accessed 3/30/2022.
  • Merck Manual. Idiopathic Intracranial Hypertension. (https://www.merckmanuals.com/professional/neurologic-disorders/headache/idiopathic-intracranial-hypertension) Accessed 3/30/2022.
  • Mondragon J, Klovenski V. Pseudotumor cerebri. (https://www.ncbi.nlm.nih.gov/books/NBK536924/) StatPearls. Treasure Island, FL: StatPearls Publishing; 2020. Updated July 10, 2020. Accessed 3/30/2022.
  • National Institute of Neurological Disorders and Stroke. Pseudotumor Cerebri Information Page. (https://www.ninds.nih.gov/Disorders/All-Disorders/Pseudotumor-Cerebri-Information-Page) Accessed 3/30/2022.

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