Increased Intracranial Pressure

Shawn Aylward
Increased Intracranial Pressure - Patient Education
What is Idiopathic Intracranial Hypertension?
Idiopathic intracranial hypertension (also called primary intracranial hypertension or pseudotumor cerebri) was first described in 1897 and is the result of increased pressure of the cerebral spinal fluid (CSF) inside the skull. CSF is a clear fluid that aids in cushioning and protecting the brain and spinal cord. Throughout the day, CSF is produced and reabsorbed at a constant rate.

What are the symptoms of intracranial hypertension?
Symptoms can include any of the following:
1. Daily, pulsating/throbbing headaches located above the eyes or the back of the head and neck, often worse when lying down or first thing in the morning.
2. Worsening of the headache with movement that increase abdominal pressure such as coughing, Valsalva (bearing down), or bending over.
3. A whooshing or ringing sound heard in one or both ears.
4. Double vision due to pressure on the nerves controlling eye movement.
5. Brief changes in vision, such as temporary blurring, dimming or complete blacking out of vision.
6. Visual field defects, usually in the peripheral vision (vision out to the side).
7. Nausea and vomiting, dizziness.

What causes idiopathic intracranial hypertension?
The exact cause is unknown, current theories include difference in production versus absorption of the CSF. This causes the pressure inside the skull to increase.

What are the risk factors for idiopathic intracranial hypertension?
So far, the only factors found to increase one’s risk of developing idiopathic intracranial hypertension are obesity, female gender, and having reached adolescence. A steady increase in weight over 6-12 months can result in this condition. Some medications and medical conditions have been linked, and then is referred to as secondary intracranial hypertension. These can include medications for acne, steroids, growth hormone, antibiotics, thyroid replacement, and some chemotherapy drugs to name a few. Medical conditions include kidney disease, head injuries, lupus, acute sinus infections, ear infections, Lyme disease, blood clotting disorders, and malnutrition.

Can children develop idiopathic intracranial hypertension?
Yes, though there are two different groups of children. The first group includes children who have not reached puberty and is seen equally in males and females. The second group is teenagers who have reached puberty and fall into the risks listed above.

How is idiopathic intracranial hypertension diagnosed?
If symptoms of intracranial hypertension are seen, an ophthalmologist (eye doctor) usually examines for swelling of a nerve in the back of the eye and for signs of vision loss. Some diseases can appear the same as pseudotumor cerebri; tests such as a CT or MRI scan is done to help rule these out. If the imaging does not show another cause, a lumbar puncture (spinal tap) is performed. The pressure inside the skull is measured and the CSF is analyzed.
The diagnosis of intracranial hypertension is given if there are symptoms listed above and elevated pressure inside the skull.

How is idiopathic intracranial hypertension treated?
The ophthalmologist monitors your vision and the nerve swelling closely. The common method of treatment is medications to lower the pressure and decrease the nerve swelling. There are three groups of medications used individually or together.
Less common is surgery to help lower the pressure. This can include placing small cuts in the covering of the nerve (optic nerve fenestration), or a tube (shunt) to slowly remove some of the CSF (in the spinal canal, or inside the skull).

Are there treatments besides medicine and surgery that can help?
The most important thing anyone can do to improve this condition is to lose weight if obese. Ask your doctor about local resources and clinics designed to assist you in your weight loss.

What is the outcome of idiopathic intracranial hypertension?
If treated, the outcome is good. If untreated, permanent vision loss can occur. Unfortunately, up to 1 in 10 of people with intracranial hypertension experience some vision loss. The increased pressure can return months or even years later, though this risk is reduced by maintaining a healthy weight. It is important to have regular eye examinations done to monitor for vision loss even after the intracranial hypertension resolves.

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