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Pseudotumor cerebriBy Mayo Clinic staff
Original Article: http://www.mayoclinic.com/health/pseudotumor-cerebri/DS00851
Pseudotumor cerebri (SOO-doh-too-mur SER-uh-bry) occurs when the pressure inside your skull (intracranial pressure) increases for no obvious reason. Symptoms mimic those of a brain tumor, but no tumor is present. Pseudotumor cerebri can occur in children and adults, but it's most common in obese women of childbearing age.
When no underlying cause for the increased intracranial pressure can be discovered, pseudotumor cerebri may also be called idiopathic intracranial hypertension.
The increased intracranial pressure associated with pseudotumor cerebri can cause swelling of the optic nerve and result in vision loss. Medications often can reduce this pressure, but in some cases, surgery is necessary.
Pseudotumor cerebri signs and symptoms may include:
- Moderate to severe headaches that may originate behind your eyes, wake you from sleep and worsen with eye movement
- Ringing in the ears that pulses in time with your heartbeat
- Nausea, vomiting or dizziness
- Blurred or dimmed vision
- Brief episodes of blindness, lasting only a few seconds and affecting one or both eyes (visual obscurations)
- Difficulty seeing to the side
- Double vision (diplopia)
- Seeing light flashes (photopsia)
- Neck, shoulder or back pain
The exact cause of pseudotumor cerebri in most individuals is unknown, but it may be linked to an excess amount of cerebrospinal fluid within the bony confines of your skull.
Your brain and spinal cord are surrounded by cerebrospinal fluid, which acts like a cushion to protect these vital tissues from injury. This fluid is produced in the brain and eventually is absorbed into the bloodstream. The increased intracranial pressure of pseudotumor cerebri may be a result of a problem in this absorption process.
In general, your intracranial pressure increases when the contents of your skull exceed its capacity. For example, a brain tumor typically increases your intracranial pressure because there's no room for it. The same thing happens if your brain swells or if you have too much cerebrospinal fluid.
Recent evidence indicates that the majority of people with pseudotumor cerebri have a narrowing (stenosis) in two large sinuses in the brain (transverse sinuses), but it's not clear whether the narrowing is a cause or effect of the condition.
The following factors have been associated with pseudotumor cerebri:
Pseudotumor cerebri occurs in about 1 person per 100,000 in the general public. Obese women under the age of 44 are nearly 20 times more likely to develop the disorder.
Substances that have been linked to pseudotumor cerebri include:
- Growth hormone
- Oral contraceptives
- Discontinuation of steroids
- Excess vitamin A
The following conditions and diseases have been linked to pseudotumor cerebri:
- Addison's disease
- Head injury
- Kidney disease
- Lyme disease
- Polycystic ovary syndrome
- Sleep apnea
- Underactive parathyroid glands
As many as 10 percent of the people with pseudotumor cerebri experience progressively worsening vision and may eventually become blind. Even if your symptoms have resolved, they can recur — months or even years later.
Preparing for your appointment
While you might first discuss your symptoms with your family doctor, he or she may refer you to a neurologist or an eye specialist for further evaluation.
To get the most from your appointment, it's a good idea to prepare. Here's some information to help you.
What you can do
- Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
- Write down key personal information, including any major stresses or recent life changes.
- Make a list of all medications, vitamins or supplements you're taking.
- Take a family member or friend along, if possible. Sometimes it can be difficult to soak up all the information you get during an appointment. Someone who accompanies you may remember something that you missed or forgot.
- Write down questions to ask your doctor.
Preparing a list of questions to ask your doctor will help you make the most of your time together. For pseudotumor cerebri, some questions to ask your doctor include:
- What is likely causing my symptoms or condition?
- Other than the most likely cause, what are other possible causes for my symptoms or condition?
- What kinds of tests do I need?
- Is my condition likely temporary or chronic?
- What is the best course of action?
- What are the alternatives to the primary approach you're suggesting?
- Would losing weight help my condition?
- I have these other health conditions. How can I best manage them together?
- Are there any restrictions I need to follow?
- Should I see a specialist?
- Is there a generic alternative to the medicine you're prescribing?
- Are there any brochures or other printed material that I can take home with me? What websites do you recommend?
Don't hesitate to ask any other questions that arise during your appointment.
What to expect from your doctor
Your doctor is likely to ask you a number of questions, including:
- When did you begin experiencing symptoms?
- Have your symptoms been continuous or occasional?
- How severe are your symptoms?
- What, if anything, seems to improve your symptoms?
- What, if anything, appears to worsen your symptoms?
Tests and diagnosis
If pseudotumor cerebri is suspected, a doctor specializing in eye disorders will look for a distinctive type of swelling — called papilledema — in the back of your eye. You will also undergo a visual fields test to see if there are any blind spots in your vision besides your so-called normal blind spot in each eye where the optic nerve enters the retina.
CT or MRI scans can rule out other problems that can cause similar symptoms, such as brain tumors and blood clots.
Spinal tap (lumbar puncture)
A lumbar puncture — which involves inserting a needle between two vertebrae in your lower back — can determine how high the pressure is inside your skull.
Treatments and drugs
Pseudotumor cerebri treatment typically begins with medications to control the symptoms. Weight loss is recommended for obese individuals. If your vision worsens, surgery to reduce the pressure around your optic nerve or to decrease the intracranial pressure may be necessary. Once you've had pseudotumor cerebri, you should have your vision checked regularly.
- Glaucoma drugs. One of the first drugs usually tried is acetazolamide (Diamox), a glaucoma drug that reduces the production of cerebrospinal fluid by at least 50 percent. Possible side effects include stomach upset, fatigue, tingling of fingers, toes and mouth, and kidney stones.
- Diuretics. If acetazolamide alone isn't effective, it's sometimes combined with furosemide (Lasix), a potent diuretic that reduces fluid retention by increasing urine output.
- Migraine medications. Medications typically prescribed to relieve migraines can sometimes ease the severe headaches that often accompany pseudotumor cerebri.
- Optic nerve sheath fenestration. This procedure cuts a window into the membrane that surrounds the optic nerve. This allows excess cerebrospinal fluid to escape. Vision stabilizes or improves in more than 85 percent of cases. Most people who have this procedure done on one eye notice a benefit for both eyes. However, this surgery isn't always successful and may even increase vision problems.
- Spinal fluid shunt. Another type of surgery inserts a long, thin tube — called a shunt — into your brain or lower spine to help drain away excess cerebrospinal fluid. The tubing is burrowed under your skin to your abdomen, where the shunt discharges the excess fluid. Symptoms improve for more than 80 percent of the people who undergo this procedure. But shunts can become clogged and often require additional surgeries to keep them working properly. Complications can include low-pressure headaches and infections.
Lifestyle and home remedies
Obesity dramatically increases a young woman's risk of pseudotumor cerebri. In fact, a weight gain of as little as 5 percent of your body weight can increase the risk — even in women who aren't obese. Losing extra pounds and maintaining a healthy weight may help reduce your chances of developing this potentially sight-stealing disorder.
- Lee AG, et al. Idiopathic intracranial hypertension (pseudotumor cerebri): Clinical features and diagnosis. http://www.uptodate.com/home/index.html. Accessed Sept. 29, 2010.
- Lee AG, et al. Idiopathic intracranial hypertension (pseudotumor cerebri): Prognosis and treatment. http://www.uptodate.com/home/index.html. Accessed Sept. 29, 2010.
- Lee AG, et al. Idiopathic intracranial hypertension (pseudotumor cerebri): Epidemiology and pathogenesis. http://www.uptodate.com/home/index.html. Accessed Sept. 29, 2010.
- NINDS pseudotumor cerebri information page. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/pseudotumorcerebri/pseudotumorcerebri.htm. Accessed Sept. 30, 2010.
- Dhungana S, et al. Review article: Idiopathic intracranial hypertension. Acta Neurologica Scandinavica. 2010;23:71
- Uretsky S. Surgical interventions for idiopathic intracranial hypertension. Current Opinion in Ophthalmology. 2009;20:451.
- Neurological diagnostic tests and procedures. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/misc/diagnostic_tests.htm. Accessed Sept. 30, 2010.
- Papilledema. The Merck Manuals: The Merck Manual for Healthcare Professionals. http://www.merck.com/mmpe/sec09/ch107/ch107e.html. Accessed Oct. 1, 2010.
- Sinclair AJ, et al. Low energy diet and intracranial pressure in women with idiopathic intracranial hypertension: Prospective cohort study. British Medical Journal. 2010;340:2701.