Pseudotumor Cerebri

Symptoms:

Pseudotumor cerebri (also known as idiopathic intracranial hypertension) is often associated with frequent or even daily headaches, tinnitus (hearing a 'whooshing' sound at the same pace as your pulse), and visual changes. The visual changes may include double vision (diplopia), decreased peripheral vision (an inability to see objects well unless you are looking directly at them), and, sometimes, sudden episodes of complete visual loss, in one or both eyes, lasting a few seconds at a time. If untreated, it can lead to permanent vision loss, which is the most feared result of pseudotumor cerebri.

How is it diagnosed?

A physician will need to order brain imaging (CT or MRI) in order to be sure there isn't a mass lesion in the brain or hydrocephalus (a backup of fluid within the brain). He or she may also wish to order imaging of the large veins in the brain, since a clot in one of these veins can produce similar symptoms. A physician may be able to detect swelling around the optic nerve (papilledema) by looking into the eye with a fundoscope or sending a patient to an ophthalmologist for this exam. Ultimately, the only reliable method of diagnosis is performance of a lumbar puncture. During this procedure, a needle is passed between the bones of the low back (spinous processes) into the cerebrospinal fluid that surrounds the spinal nerves. The pressure of the fluid is then measured.


Who gets pseudotumor?

Pseudotumor cerebri tends to affect women who are overweight and young (15-45 years of age). Other risk factors include the use of certain medications (birth control pills, Vitamin A supplements, or certain antibiotics). The list of things that may or may not be related to the development of this disorder is very large (and unconfirmed), but can be found here.

What is the underlying problem?

Pseudotumor cerebri is NOT a tumor. It gets its name from the similarity of the symptoms to those patients who actually have brain tumors. In pseudotumor cerebri, the pressure within the brain is high (intracranial hypertension), but for unknown reasons (idiopathic). There is speculation that perhaps the brains of those with pseudotumor cerebri produce too much cerebrospinal fluid, or don't absorb it correctly from within the brain, or that veins of the brain are abnormal and lead to a 'back up' of fluid into the brain. None of these theories has yet been proven.

The high pressure within the brain causes headaches and all the other associated symptoms. Because the nerves that control eye movements pass through the brain and skull, they can be stretched, leading to double-vision. The pressure within the brain also puts pressure on the optic nerve, causing it to swell and leading to vision loss (either temporary or permanent).

What are the treatments?

The same lumbar puncture that is used to diagnose pseudotumor cerebri, is also an effective treatment. Because spinal fluid is taken out during this procedure, this has the effect of reducing elevated brain pressure and will usually immediately improve all symptoms. For some patients, the symptoms will stay away for an extended period of time after a lumbar puncture. For others, the symptoms will recur within a few days. Occasionally, a person is 'cured' by the lumbar puncture - the symptoms never again occurring.

Most patients require medical treatment. First line therapy is Diamox (acetazolamide). This medication works by reducing the production of cerebrospinal fluid in the brain, thus reducing brain pressure. Other options include Topamax (topiramate) or digoxin Some physicians place patients on a short course of steroids, which may also be helpful.

For patients whose symptoms are not well-controlled using medications, repeated lumbar punctures can be performed. If this is required enough times, patients are usually offered a surgical procedure (shunting) in which a tube is placed either in the fluid filled spaces of the brain (ventricles) or in the fluid around the spinal nerves of the low back (lumbar). The other end of the tube can be inserted into the heart or abdomen (peritoneal cavity) to drain out the fluid.

In patients who are at high risk of permanent vision loss, optic nerve fenestration may also be offered. This involves releasing the pressure around the optic nerve by cutting a slit into it and allowing continuous drainage of the cerebrospinal fluid.

For more information on this disorder, please visit:

The Intracranial Hypertension Research Foundation


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