Woman experiencing headache and eye pain symptoms of Pseudotumor cerebri

Pseudotumor cerebri (PTC) is a health condition that occurs when the pressure in your skull begins to increase for no specific reason. This leads to unexplainable bouts of headaches and vision problems. Pseudotumor cerebri in layman terms means, “false brain tumor”.  The way you pronounce it is “SOO-doh-too-mur SER-uh-bry”.

The symptoms of this condition mimic that of a brain tumor. It is also known as the following:

  • idiopathic intracranial hypertension,
  • benign intracranial hypertension,
  • pseudotumor cerebri syndrome,
  • PTC syndrome,
  • primary intracranial hypertension, and
  • secondary intracranial hypertension.

This wonderful video provides an excellent description of what PTC is and how it makes you feel. After watching this, you will know how to explain the condition to your friends, family and coworkers in a simple way without sounding like a medical professional.

PTC can occur in adults and children alike, but is more common in women of childbearing age who are overweight or obese.  It is also most commonly diagnosed between the ages of 20 and 50 years old (see chart).

The increased intracranial pressure associated with this condition can lead to the inflammation of the optic nerve and result in problems with vision. It can be hard to tell the difference between a pseudotumor and a real tumor. Therefore, you must visit a physician to have your symptoms checked out.

Left untreated, these symptoms can get worse and lead to total vision loss. Getting treated on time can help save your sight. In most cases, medication can help alleviate this eye pressure, but in some cases surgery may be necessary.

Symptoms

Pseudotumor cerebri symptoms include:

  • Moderate to severe headaches that tend to originate behind your eyes and get worse with eye movement
  • Nausea, dizziness, and vomiting
  • Tinnitus, which is nothing but ringing in the ears that match the rhythm of your heartbeat
  • Blurred vision
  • Double vision
  • Short episodes of total blindness that lasts only for a few seconds and affects one or both eyes
  • Trouble seeing to the side
  • Experiencing light flashes
  • Neck stiffness
  • Forgetfulness
  • Unexplained shoulder, neck, and back pain
  • Depression

Causes

While what exactly causes this condition is still unknown, doctors opine that it may be associated with a buildup of cerebro-spinal fluid within the skull that causes this increase in pressure. Your spinal cord as well as your brain is surrounded by cerebro-spinal fluid that renders a cushioning effect to protect these vital organs from injury. This fluid also supplies the brain and spinal cord with the nutrients it needs and removes impurities while protecting them.

Normally, after circulating, the cerebro-spinal fluid is reabsorbed into the body via blood vessels. It is believed that a disruption or problem in this absorption process could lead to an increase in the intracranial pressure within the confines of the skull. This can also happen when way too much fluid is produced by the brain.

According to studies, intracranial pressure tends to increase when the contents within the skull begins to exceed its capacity. For instance, a brain tumor can cause elevated intracranial pressure as there may be not enough room for the tumor. This applies to cerebro-spinal fluid too. When your brain swells up or there is way too much cerebro-spinal fluid, the intracranial pressure is bound to spike up.

Several studies indicate that those suffering from pseudotumor cerebri may have a narrowing also known as stenonsis, in the two large sinuses of the brain. Doctors and researchers are trying to determine if this could cause the condition or affect it adversely.

Risk factors

There are two major factors that have been associated with pseudotumor cerebri. These are:

Obesity

Obesity, a common lifestyle disorder may be associated with pseudotumor cerebri. Studies indicate that 4 out of every 21 in 100,000 obese women develop this condition. Women under 44, who are obese, stand a higher risk of developing this disorder sooner or later. It can be safely said that excess body weight is the most significant and preventable risk factor. However, thin people too can develop this disorder.

Medications

A number of medications that are ingested on a daily basis to treat other ailments, can cause this condition. Studies suggest that the substances listed below may be linked to pseudotumor cerebri:

  • Tetracycline
  • Growth hormones or steroids
  • Excess vitamin A
  • Lithium

It is believed that certain other medications too may be associated with the disorder, but further studies and research is needed to establish it.

Note: Pseudotumor birth control products including Mirena and depo provera, have also recently been linked to the development of this condition.

Gender

Doctors also opine that women are at a higher risk of contracting the condition than men. In fact, they are nine times more likely to contract the condition. The role of hormones is not fully understood yet, but studies are being conducted to evaluate the link.

Blood vessel irregularities

People with congenital conditions such as narrowing of the vein, which drains CSF and blood from the brain, are susceptible to developing this condition, due to increased pressure in the brain.

Sleep apnea

This is an increasingly common sleep disorder that is plaguing several hundreds, across the globe. Not only does this condition affect the quality of life to a great extent, but may also be associated with pseudotumor cerebri.

The following conditions and diseases have been linked to pseudotumor cerebri:

  • Anemia
  • Addison’s disease
  • Kidney disease
  • Sleep apnea
  • Lupus
  • Polycystic ovary syndrome
  • Underactive parathyroid glands
  • Uremia
  • Blood-clotting disorders
  • Behcet’s syndrome

Complications

Some people suffering from pseudotumor cerebri may experience progressively worsening vision and could eventually become blind. In some cases, even if your symptoms are resolved after medication, they can recur months or even several years later.

Diagnosis & Talking to Your Doctor

Woman talking to doctor about Pseudotumor cerebri diagnosis.

Woman talking to doctor about Pseudotumor cerebri diagnosis.

Diagnosis of this condition involves ruling out other health issues that include an actual brain tumor. Your physician will first ask about the symptoms that you are experiencing and take notes on your medical history.

Your doctor will get the biggest clue that you may be suffering from Pseudotumor Cerebri, when he looks at the optic nerve, using a tool called an ophthalmoscope. If the nerve is swollen, he may conduct a physical examination and write out a few tests that can help identify second his suspicion while also ruling out other causes of increased pressure inside the skull.

  • Testing of the eyes
  • Brain Imaging such as CT or MRI scans
  • Lumbar puncture

Testing of the eyes

Since elevated intracranial pressure causes vision problems, a careful eye examination and testing of your visual fields is performed to determine the risk of total vision loss. You may also be referred to an eye doctor who will check whether you have any blind spots in your vision. A thorough eye examination will be performed and may reveal a swelling of the optic nerve at the back of your eyes. This abnormality is known as papilledema.

Imaging of the skull

A CT scan may be ordered to rule out the condition. In some cases this scan may reveal fluid spaces that are smaller than normal, in the brain ventricles.

Your doctor may also ask you to get an MRI scan to diagnose pseudotumor cerebri. The scan could either be normal or show small ventricles. In some cases, the scan may also reveal a flattened pituitary gland. Both of these, can cause elevated pressure in your skull. The scan may also identify the presence of draining veins that may have narrowed or other indirect signs of high spinal fluid pressure.

Testing for intracranial pressure

Imaging tests are generally performed to rule out tumors or other abnormalities. Once the results confirm that you do not have a tumor in the brain, your doctor will assess the pressure of the cerebro-spinal fluid to verify the diagnosis he has arrived at. A spinal tap is performed to arrive at a final diagnosis.

A lumbar puncture or spinal tap is performed to confirm elevated pressure. A sample of fluid from around the spine will be withdrawn for testing to eliminate infectious and inflammatory causes that can lead to raised pressure.

The fluid drainage can give immediate relief to several patients. However, this relief from headache and other symptoms is temporary. Also, this response with signs of vision problems, or elevated pressure is not conclusive evidence that you may be suffering from pseudotumor cerebri.

Treatment

Pseudotumor cerebri surgery treatment

Surgical treatment may be needed for severe cases of Pseudotumor cerebri.

Getting treated as soon as possible can help keep your vision from failing entirely.

One of the easiest way to alleviate symptoms is to shed extra pounds. Since this can be hard to achieve on your own, you may seek the assistance of a dietician, a personal trainer, or a weight loss clinic. You must also limit your salt and fluid intake to lower the production of spinal fluid in your body.

Apart from dietary changes, you may also resort to medication or surgery to treat pseudotumor cerebri. This will help reduce the pressure in your skull.

Medication used to treat pseudotumor cerebri include:

  • Acetazolamide – a glaucoma drug that can lower the amount of cerebro-spinal fluid that is produced by the body.
  • Furosemide – a drug that helps expel fluid from the body by making you urinate more.
  • Certain steroids that can lower pressure in the brain.
  • Migraine medicines to combat with recurrent headaches.

If the symptoms you experience are severe, and simply do not get better with medication, you may need surgery to bring down the pressure in your brain and/or behind your eyes. A number of procedures are performed to treat this condition.

  • Transverse sinus stenting. A new minimally invasive procedure where a team of interventional neuroradiologists and neurosurgeons will use intravascular ultrasound imaging to delicately thread an expandable metal stent, through an opening in the groin, all the way to the main blood vessels in the neck and shoulders draining fluid from the brain. The goal of this surgical procedure is to position the stent across the narrowed portion of the vein, called the transverse sinus, where it expands, allowing blood to drain more freely and relieving fluid pressure in the brain.
  • Shunting. During this procedure, a long and thin tube called a shunt is lowered into your brain or spine by the surgeon to drain out extra fluid.
  • Optic nerve sheath fenestration. In this procedure, your surgeon will make a cut in the tissue around the optic nerve. This cut will allow excess fluid to drain.

What to expect post treatment

When you open the door for one of these procedures, the pressure in your brain will begin to drop. You will begin to feel better within a few months. While most people enjoy total recovery, a few may have to deal with permanent vision loss.

Since this condition can reoccur, you will need to visit your doctor regularly for follow-up visits. You must inform the doctor in case you notice any new symptoms on your visits. You may also have to undergo regular eye checkups.

Also, shed a few pounds if you are overweight to ensure the condition doesn’t come back. If you are obese, diet and exercise alone mat not help. In such cases, your doctor may suggest weight loss surgery too.

When diagnosed early this condition can be easily treated. So do not ignore any of the tell-tale signs or symptoms listed above. Seek the counsel of a physician who can diagnose the problem and suggest the right treatment for you.

Additional Resources

If you are looking for a Pseudotumor support group, you have a couple of options.

  1. Pseudotumor Cerebri Awareness and Support Community – this is a facebook group with approximately 8,000 members.
  2. The Pseudotumor Cerebri Support Group at DailyStrength.com also has a message board containing about 7,000 posts and 500 members.

Frequently Asked Questions

Is pseudotumor cerebri a disability?

  • Unfortunately, pseudotumor cerebri is not listed as a disability by the Social Security Administration, however you may be qualified to claim Supplemental Security Income (SSI) or the Social Security Disability Insurance (SSDI) benefits depending on the severity of your symptoms.  We would suggest that you speak with a disability lawyer.