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Diagnosis / Idiopathic Intracranial Hypertension

What is Idiopathic Intracranial Hypertension?

In idiopathic intracranial hypertension (IIH) there is raised pressure within the skull (intracranial pressure), which puts pressure on the brain. Idiopathic means that the cause of this raised pressure is unknown. The main symptoms are headache and loss of sight. Treatment is aimed at preventing permanent visual loss.

Risk Factors for Idiopathic Intracranial Hypertension

IIH is rare. It affects 1 or 2 people in every 100,000. IIH mostly affects women of childbearing age who are overweight or obese. However, men and children can sometimes be affected as well as people who are not overweight.

More than 9 out of 10 people with IIH are obese women, body mass index (BMI) greater than 30, in the reproductive age range. However in someone who is not overweight, there are some ‘risk factors’ that are thought to be associated with the development of IIH. There are many such rare ‘associations’ but some of these include:

  • Taking (or after stopping) certain medicines such as steroids, some antibiotics and oral contraceptive pills.
  • Other diseases such as systemic lupus erythematosus, sarcoidosis and kidney disease.
  • Pregnancy

What are the Symptoms of Idiopathic Intracranial Hypertension?

The most common symptoms of intracranial hypertension are headaches and visual loss, including blind spots, poor peripheral (side) vision, double vision, and short temporary episodes of blindness. Many patients will experience permanent vision loss. Other common symptoms include pulsatile tinnitus (ringing in the ears) and neck and shoulder pain.

Intracranial hypertension can be either acute or chronic. In chronic IIH, the increased CSF pressure can cause swelling and damage to the optic nerve – a condition called papilledema.

Because the symptoms of IIH can resemble those of a brain tumor, it is sometimes known by the older name pseudotumor cerebri, which means “false brain tumor”.

How is Idiopathic Intracranial Hypertension Diagnosed and Evaluated?

The main goal when diagnosing IIH is to rule out other causes of raised pressure within the skull. These may include problems such as water on the brain (hydrocephalus) or a brain tumor. Your doctor will usually refer you for imaging studies of your brain, such as an MRI or CT.

You may also have a more detailed eye examination. This will allow an eye specialist to examine the back of your eyes fully. You may have visual field testing to see if there are any signs of loss of vision in part or one or both of your eyes.

You may also have tests on your CSF by doing a lumbar puncture. This will show raised CSF pressure if you have IIH. A lumbar puncture – sometimes called a spinal tap – is a procedure where a sample of CSF is taken for testing.

IVC’s Treatment for Idiopathic Intracranial Hypertension

If you are diagnosed with idiopathic intracranial hypertension, it is important that your vision be closely monitored to look for any changes or early signs of vision loss. Any signs of deterioration in your vision can mean that your treatment needs to be adjusted.

At IVC, we offer minimally invasive treatment options, venous sinus pressure measurements and stenting. In some people with IIH, the normal absorption of cerebrospinal fluid into the veins of the brain is impaired due to a narrowing of the venous sinuses, passageways that convey blood and CSF from the brain to the jugular veins and eventually to the heart. This narrowing of these passageways overtime can increase pressure inside the skull and cause IIH.

If weight loss and medication have not controlled your IIH, your doctor may recommend a contrast enhanced MR venogram or contrast enhanced CT venogram to see if there is a narrowing in your venous sinuses. If so, the next step is a referral to an interventional neuro-radiologist to see if you might benefit from having a stent placed in the vein to open up the narrowing.

* This information about Idiopathic Intracranial Hypertension was reviewed by Dr. Jason R. Bauer. If you have any questions, please don’t hesitate to contact us using the form below.

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