Pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH), describes a condition of high pressure inside the skull.
Until recently, much about this condition, including the causes, was difficult to pinpoint and address, as most brain scans did not demonstrate a concrete problem to fix. Now, doctors have demonstrated that many pseudotumor cerebri cases occur due to a narrowing of veins that drain blood from the brain, causing the blood to back up and pressure to build.
The condition affects young women (in their 30s and 50s) more than men and is often associated with obesity. Patients with CSF rhinorrhea, CSF otorrhea (leaking spinal fluid from the nose or ear) or nasal meningoceles/encephaloceles may also have pseudotumor cerebri.
Patients suffering from this disorder can experience:
- Loss of vision
- Hearing loss
- Tinnitus (ringing in the ears)
Headaches are often severe and occur in over 90 percent of patients with pseudotumor cerebri.
Patients often need multiple tests to rule out other causes of their symptoms, including meningitis, obstructive hydrocephalus, tumors and blockage of the veins that drain blood away from the brain.
Diagnosis begins with a careful history and physical exam. Patients may need to see multiple health care providers, including neurologists, neurosurgeons, otolaryngologists (ear, nose and throat specialists) and ophthalmologists.
Tests for the condition may include:
- Eye exam to test for vision loss and papilledema, which occurs when a portion of back of the eye becomes swollen
- Hearing test to look for any other cause of tinnitus
MRI of the brain to look at the size of the ventricles and look for other signs of pseudotumor like empty sella (a condition where the pituitary gland shrinks or is flattened)
- Lumbar puncture (spinal tap) to measure the brain and spinal fluid pressure and check for the presence of bacteria or viruses and rule out meningitis
Treating Fluid Buildup
Until recently, pseudotumor cerebri was notoriously difficult to treat adequately. Doctors such as ophthalmologists and neurologists typically addressed the issue of fluid buildup and pressure with medications to reduce fluid production and relieve headaches. Sometimes, neurosurgeons would implant a shunt device to drain the fluid, or ophthalmologists would make small cuts behind the eye to try to prevent further vision loss.
A New Procedure for Pseudotumor Cerebri at UVA
Doctors have since found that many of these cases result from a narrowing of veins in the brain. UVA is one of the few places in the country that can identify and treat this situation, using a balloon and stent to open the veins and relieve the pressure, reducing symptoms. Neurosurgeons at UVA, who were among the first to identify the problem and perform the procedure, have recently been the first to demonstrate that this cutting-edge stenting procedure actually returns patients’ pressure back to normal.
If the condition has caused any brain fluid leaks (CSF rhinorrhea or otorrhea), surgery may be needed to repair those conditions.
Medications can be used to help decrease the production of the fluid that surrounds the brain (cerebrospinal fluid or CSF). Medications include:
- Acetazolamide, a carbonic anhydrase inhibitor
- Diuretics (water pills), such as furosemide
- Steroids, such as prednisone (needed in some cases where vision loss has occurred quickly)
Follow-Up and Care
With all treatments, frequent follow-up with your doctor is needed until symptoms improve. To help lessen symptoms and control blood pressure, we also recommend weight loss and a low-sodium diet.
The causes of IIH are not completely known, but the condition is associated with obesity and high blood pressure.