What Is Pseudotumor Cerebri?
Pseudotumor cerebri occurs when the pressure of the cerebrospinal fluid (CSF) inside of your skull increases with no apparent cause . This cerebrospinal fluid covers the brain and spinal cord, protecting and cushioning them from injury. It is important to understand that pseudotumor literally means "false tumor," as it is a condition that only mimics the presence of a tumor due to increased pressure.
Symptoms commonly include headaches that may be severe, intense, or throbbing, and noises in the ears such as whooshing or ringing (tinnitus). You may also experience nausea or vomiting, neck, shoulder, or back pain, vision problems, and rarely, facial paralysis.
Treatment varies, but will likely include a combination of medication, procedures, and lifestyle adjustments.
You should visit your primary care physician within the next 2 days to confirm a diagnosis and discuss treatment options for managing symptoms.
Pseudotumor Cerebri Symptoms
Symptoms of pseudotumor cerebri are described below, including those that affect most people as well symptoms that are specific to vision.
Main symptoms of pseudotumor cerebri include the following.
- Headaches: Headaches occur in nearly all (90 to 94 percent) people with pseudotumor cerebri . These headaches are often moderate to severe in intensity, throbbing, pressure-like, and non-stop. You may feel the headaches the most behind your eyes and moving your eyes may exacerbate your pain.
- Noise in the ears: If you're like 58 percent of people with pseudotumor cerebri, you may experience tinnitus that sounds like a whooshing, ringing, or buzzing sound that may be constant, intermittent (comes-and-goes), or pulsating . This typically only occurs in one ear (unilateral).
- Nausea or vomiting: Nausea and/or vomiting is often the body's way of signaling an underlying problem. In the case of pseudotumor cerebri, your body is reacting to the increased pressure in your skull.
- Neck, shoulder or back pain: Since the spinal cord is also covered in cerebrospinal fluid, the processes going on in the brain causing increased pressure can also travel and radiate to the neck, shoulder or back causing pain and irritation.
- Facial paralysis: In rare cases, the nerve that controls the muscles of your face may be affected in a similar way to how it affects the ocular nerve (see above), which can cause paralysis (the inability to move the facial muscles).
Vision issues are a common symptom, occurring in 68 to 85 percent of people with this condition because the nerve to the eye (the optic nerve) is in direct communication with the brain and completely bathed in cerebrospinal fluid . Thus, when the pressure of the cerebrospinal fluid increases, it can compress the optic nerve and cause a loss of blood flow and oxygen. This results in vision issues (see below). This injury to the optic nerve can manifest in the following ways:
- Double vision (diplopia): This occurs in 38 percent of people with this condition. Diplopia is when you see two images of a single object at once. You may see the double image horizontally, vertically or diagonally in your vision.
- Difficulty seeing to the side (tunnel vision): Pseudotumor cerebri can affect your peripheral vision — the ability to see to the side while staring straight ahead.
- Blurred or dimmed vision: In addition to double vision and peripheral vision issues, your vision may seem hazy or out-of-focus as well.
- Seeing light flashes: You may also experience flashes of light in addition to other vision changes.
- Visual obscurations: These are defined as brief episodes of blindness (in one or both eyes) that is transient (lasts for a few seconds).
Pseudotumor Cerebri Causes
It is important to understand that pseudotumor cerebri is a diagnosis of exclusion — meaning that it is usually a diagnosis your physicians will consider for increased intracranial pressure (ICP) after all examinations and tests are completed and other diagnoses are ruled out .
The word "idiopathic" is defined as "relating to or denoting any disease or condition that arises spontaneously or for which the cause is unknown." As discussed prior, "pseudotumor" means "false tumor" because the increase in pressure mirrors that which occurs when someone has a tumor except, of course, you do not have a tumor.
While the cause of pseudotumor cerebri is not perfectly understood, the working hypothesis is that it is the result of a problem in the body's ability to properly absorb cerebrospinal fluid into the bloodstream. This leaves you with an excess amount of cerebrospinal fluid in the brain . This can predispose you to the symptoms and complications associated with this condition.
Who is most likely to be affected
Pseudotumor cerebri is rare, with only nine cases per 1,000,000 people in a given year.
- Women: The disease occurs more frequently in women, who are eight times more likely than men to develop pseudotumor cerebri .
- Overweight individuals: The disease is characteristically most common in overweight to obese women of childbearing age (more than 90 percent).
- Those who use certain medications: The disease is also associated with medications for severe acne that contain vitamin A and antibiotics such as Bactrim and tetracyclines .
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Treatment Options and Prevention for Pseudotumor Cerebri
The goal of pseudotumor cerebri treatment is the improvement of symptoms such as headaches, restoration of the baseline visual exam and prevention of future vision problems, especially blindness.
Fortunately, pseudotumor cerebri is treatable and there are many options for treatment that you can discuss with your physician. These treatments can be separated into medication-related treatments and surgical treatments .
This is the first-line option for treating pseudotumor cerebri. Medications that treat pseudotumor cerebri all have different mechanisms, but all ultimately work to decrease increased intracranial pressure. Examples include:
- Acetazolamide: The first-line treatment for pseudotumor cerebri, acetazolamide works by reducing the production of cerebrospinal fluid.
- Diuretics: Diuretics (also known as "water pills") work to reduce fluid retention by increasing urination. Sometimes diuretics and glaucoma medications are used together for maximum effect.
- Migraine Medications: Migraine medications are used to relieve the headache symptoms associated with increased intracranial pressure.
Surgery is often reserved for rapidly progressing cases of pseudotumor cerebri that severely affect vision. Surgery is needed to quickly reduce the pressure around the optic nerve and can also allow fluid to escape, reducing the pressure in the skull. Options include:
- Lumbar puncture: This is a common treatment for pseudotumor cerebri, also known as a spinal tap, which is a procedure to enter the space in the cerebrospinal fluid below the spinal cord. From there, the physician can siphon cerebrospinal fluid in a safe manner, reducing the pressure in the brain.
- Optic nerve sheath fenestration: The most common treatment for the intracranial pressure that endangers functional vision is called optic nerve sheath fenestration. A surgeon cuts into the membrane that surrounds the optic nerve, allowing cerebrospinal fluid to leak out.
- Fluid shunt: Another example of a surgical procedure that can help with symptoms and prevent vision loss is a fluid shunt. These are tubes inserted into the brain that travel under the skin to the abdomen (peritoneum) and drain any excess fluid.
In addition to the treatment options above, future symptomatic episodes can be prevented by implementing simple lifestyle changes.
- Weight loss: Obesity is a risk factor for this condition, and losing weight may both improve and prevent symptoms. There are promising results of vision restoration from a combination of a weight loss regimen and acetazolamide .
- Medication changes: Since certain medications are associated with pseudotumor cerebri risk, your physician may prescribe alternative options in order to prevent symptoms.
- Limiting salty food: Salty foods can result in fluid retention and exacerbate symptoms such as headaches. Try to limit salt intake by not adding salt to already prepared foods or cooking with excessive amounts of salt.
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When to Seek Further Consultation for Pseudotumor Cerebri
Headaches and other symptoms associated with pseudotumor cerebri are often severe enough that most people will seek medical attention relatively quickly.
If you notice vision problems with any sort of headache
You should go to the emergency room or make an appointment with your primary care physician.
If you are already seeking treatment
Your physician will recommend regular appointments to monitor your symptoms since your symptoms can happen again even after treatment. You will most likely have to follow-up with an ophthalmologist (eye doctor) in order to ensure that your vision is intact and not worsening. Most people need to follow-up for months to even years after their first event.
Questions Your Doctor May Ask to Determine Pseudotumor Cerebri
To diagnose this condition, your doctor would likely ask about the following symptoms and risk factors.
- How long has your current headache been going on?
- How severe is your headache?
- Have you been feeling more tired than usual, lethargic or fatigued despite sleeping a normal amount?
- Any fever today or during the last week?
- Have you experienced any nausea?
If you've answered yes to one or more of these questions
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- Calhoun AH. Case studies in headache: Intercranial hypertension. National Headache Foundation. National Headache Foundation Link
- Pseudotumor cerebri. Mayo Clinic. Published November 17, 2017. Mayo Clinic Link
- Idiopathic intracranial hypertension. National Eye Institute. Published April 2014. NEI Link
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