Pseudotumor cerebri is largely a diagnosis of exclusion – if a patient has high intracranial pressure and testing does not reveal any cause, the diagnosis will be idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri.
Since the first symptoms of pseudotumor cerebri are often headache and vision changes, a doctor may first perform vision tests to look for signs of swelling or a tumor. Brain imaging tests, including magnetic resonance imaging (MRI) or computed tomography (CT) scans, may be performed, also in search of tumor or other cause of the headaches.
Intracranial pressure can be tested with a spinal tap, also called a lumbar puncture. If the spinal tap reveals increased intracranial pressure, and tests have not determined any cause for that pressure, the patient will be diagnosed with idiopathic intracranial hypertension, or pseudotumor cerebri.
Treatment Options
If the patient is overweight, the initial recommendation will be weight loss. Achieving a healthy weight may help alleviate headache and other symptoms.
Medical Therapy
There are medications that can help reduce intracranial pressure, including diuretics (which reduce fluids in body tissue) and migraine drugs. Medicines used to treat glaucoma can also be effective, since they reduce pressure by suppressing production of cerebrospinal fluid (CSF).
Traditional Surgery
Traditional surgical treatments include placement of a shunt in the brain to drain excess CSF and relieve the pressure, or a surgery around the optic nerve (“fenestration”) to relieve pressure around the optic nerve and protect the vision.
The illustration at left shows bilateral venous sinus narrowing (red circles), which compromises blood flow from the brain to the neck, contributing to intracranial hypertension. In the illustration at right, the narrowing has been treated with placement of a stent. As a result the blood flow from the brain to the neck is now restored (blue arrows), relieving the increased intracranial pressure and the symptoms of pseudotumor cerebri.
New Minimally Invasive Treatment
An innovative procedure performed at Weill Cornell Medicine has shown promising results for patients with venous sinus stenosis. Venous sinus stenting is a minimally invasive procedure treatment appropriate for patients who have venous sinus stenosis on MRI or CT and involves the placement of a stent (mesh in the shape of a tube) in one of the narrowed veins in the brain, via small incisions in the groin area. Relieving the stenosis (narrowing) may relieve the increased pressure and alleviate symptoms. (Find out more about this clinical trial.)
Reviewed by: Srikanth Boddu, MD, MSc
Last reviewed/updated: September 2023
Illustrations by Thom Graves, CMI
