Idiopathic Intracranial Hypertension
Idiopathic Intracranial Hypertension Overview
Idiopathic intracranial hypertension is a disorder in which the fluid that surrounds and protects the brain and spinal cord (cerebrospinal fluid, or CSF) accumulates abnormally for an unknown reason, causing increased pressure within the skull.
Because the symptoms of idiopathic intracranial hypertension can mimic those of a brain tumor, the disorder is also known as pseudotumor cerebri, which means “false brain tumor.” The increased pressure can cause swelling of the optic nerve and subsequent vision loss.
In This Article
Idiopathic Intracranial Hypertension Symptoms
Symptoms of idiopathic intracranial hypertension may include:
- Pulsating sounds within the head (pulsatile tinnitus)
- Blurred, dimmed, or doubled vision
- Seeing light flashes (photopsia)
- Brief episodes of blindness, affecting one or both eyes (visual obscurations)
- Neck, shoulder, or back pain
Symptoms of idiopathic intracranial hypertension can resemble those of a brain tumor or another medical condition. Contact a medical professional if you are experiencing symptoms.
Idiopathic Intracranial Hypertension Treatments
The first line of treatment for idiopathic intracranial hypertension is weight loss. This is often curative, but medications may be used to lower pressure during the weight loss process. One of these medications is called acetazolamide (Diamox), which lowers production of spinal fluid in the brain. Preventive and acute headache treatment will also be utilized if necessary.
In rare cases, surgery may be needed. Surgical interventions, such as the placement of a shunt, are reserved for people who have rapid vision loss or whose pressure/headaches are not helped by medications and weight loss.
Close monitoring of vision and optic nerve swelling is vital in IIH, as vision loss can be permanent and complete blindness can occur. If there are rapid changes in vision or the appearance of the optic nerves, treatment needs to be changed quickly. Sometimes the patient doesn’t notice vision changes that are picked up on the formal testing, so it is important to see the neuro-ophthalmologist or ophthalmologist for this testing as recommended.
How common is idiopathic intracranial hypertension?
Idiopathic intracranial hypertension is rare. It is estimated to affect one in every 100,000 people in the general population.
Who gets idiopathic intracranial hypertension?
Idiopathic intracranial hypertension affects males and females of all ages, but it is more common in women between the ages of 20 and 50. It is also more prevalent among people who are overweight and can develop in the setting of weight gain.
How is idiopathic intracranial hypertension diagnosed?
In addition to performing a physical examination, your doctor may request the following tests to diagnose idiopathic intracranial hypertension:
- Eye examination
- Imaging tests, such as an MRI of the brain with contrast and MRV to evaluate the veins of the head
- Lumbar puncture (spinal tap)
- Xu DS, Hlubek RJ, Mulholland CB, Knievel KL, Smith KA, Nakaji P. Use of Intracranial Pressure Monitoring Frequently Refutes Diagnosis of Idiopathic Intracranial Hypertension. World Neurosurg. 2017 Aug;104:167-170. doi: 10.1016/j.wneu.2017.04.080. Epub 2017 Apr 21. PMID: 28435117.
- Levitt MR, Hlubek RJ, Moon K, Kalani MY, Nakaji P, Smith KA, Little AS, Knievel K, Chan JW, McDougall CG, Albuquerque FC. Incidence and predictors of dural venous sinus pressure gradient in idiopathic intracranial hypertension and non-idiopathic intracranial hypertension headache patients: results from 164 cerebral venograms. J Neurosurg. 2017 Feb;126(2):347-353. doi: 10.3171/2015.12.JNS152033. Epub 2016 Mar 11. PMID: 26967777.