Headaches come in dozens of varieties, and, fortunately, most resolve themselves in a few hours with or without an over-the-counter pain remedy. But a recurring headache that causes blurred vision – or even temporary blindness – should not be ignored.
While it may not be a brain tumor, it could be a condition called pseudotumor cerebri, meaning “false brain tumor,” also known as intracranial hypertension. It sounds less scary than an actual brain tumor, but if left untreated, this disorder could lead to permanent vision impairment or even blindness.
What Is Pseudotumor Cerebri?
Cerebrospinal fluid surrounds the brain and spinal cord, providing nutrients, removing waste and acting as a cushion to protect against injury. The body constantly reabsorbs and replenishes this fluid. While the exact cause of pseudotumor cerebri is unknown, researchers believe it occurs when the body produces too much cerebrospinal fluid or the reabsorption process fails to keep up. The fluid buildup increases pressure in the skull, causing swelling of the optic nerve.
Pseudotumor cerebri is rare, affecting fewer than one person per 100,000 in the general population. However, 90 percent of all adult cases occur in women, and the incidence is highest among women who are both overweight and pregnant.
Symptoms of Pseudotumor Cerebri
The main symptom of pseudotumor cerebri is a persistent headache. Pain may be throbbing and may be associated with the position of the head – occurring, for example, upon lying down at night or getting up in the morning. Headache pain may induce nausea or vomiting.
- Temporary dimming or complete loss of vision – typically lasting just seconds
- Persistent ringing heard in one or both ears
- Horizontal double vision
- Radiating pain in the arms or legs
- Visual field defects, including an increase in “floaters,” those small, dark shapes that float across your vision
- Loss of color vision
Because some of these symptoms also occur with regular or migraine headaches, getting in to see an ophthalmologist or neuro-ophthalmologist right away is important to get the correct diagnosis.
How It’s Diagnosed
Your doctor will give you a routine eye exam, which may reveal papilledema (swelling at the head of the optic nerve.) Because papilledema is caused by high intracranial compression, it is considered a hallmark of pseudotumor cerebri. During this exam, your doctor will determine the severity of the swelling and check your vision acuity. Both are used to determine the degree of optic nerve malfunction and the urgency of next steps, which may include imaging and blood work.
Some physicians will begin treatment at this point, but the gold standard for diagnosis is a spinal tap (lumbar puncture), when it is medically safe to do so. This involves inserting a needle into the lower part of the back and removing spinal fluid, which is pulled into a device that measures pressure. A sample will also be sent to the lab to check for other abnormalities, including infection.
Treatment for Pseudotumor Cerebri
The same spinal tap used to diagnose pseudotumor cerebri is also a treatment because removing excess spinal fluid temporarily decreases intracranial pressure. But without further treatment, your headaches are likely to return because your body is continuously making new spinal fluid.
Only one drug is approved by the U.S. Food and Drug Administration to reduce the production of spinal fluid: acetazolamide. Your doctor will use baseline bloodwork to determine the best dosage for you, then monitor you carefully until stability is achieved. At first, you may go in for weekly checkups, then every two weeks, then monthly. Even when stable, you may need to be checked every three months.
In addition to medication, your doctor may recommend certain lifestyle changes, including:
- Losing 5 percent to 10 percent of body weight
- Reducing salt in your diet
- Avoiding excess vitamin A, a fat soluable vitamin that can build up in your liver, increasing intracranial pressure and causing blurred vision
These changes can augment the effect of medicine.
Most patients see improved visual acuity quickly after treatment. However, for up to 38 percent of patients, the condition may reoccur, which is why close monitoring is required by your doctor, who may need to adjust or repeat treatment methods.
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