Pseudotumor Cerebri

Definition

Pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH), is a condition in which the pressure inside the skull where the brain rests is too high.

The condition affects women more than men and is often associated with obesity. It tends to occur in patients in their 40s and 50s. Patients with CSF rhinorrhea, CSF otorrhea, or nasal meningoceles/encephaloceles may also have pseudotumor cerebri.

Symptoms

Patients suffering from this disorder can experience:

• Headaches
• Loss of vision
• Dizziness
• Hearing loss
• Tinnitus (ringing in the ears)

Headaches are often severe and occur in over 90% of patients with pseudotumor cerebri.

Diagnosis

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

Treatment

Treatment is designed to reduce major symptoms, including headaches and vision loss. Frequent follow-up with your doctors is needed until symptoms improve. Weight loss and a low-sodium diet to help control blood pressure is also recommended. 

Medications

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)

Surgery

Surgery may be needed if symptoms can't be treated with medication.

Surgery usually includes the placement of shunts, which drain off the extra fluid into the abdominal cavity.

Other surgeries like optic nerve fenestration or venous stenting may also be used, depending on the severity of the disease.

If the condition has caused any brain fluid leaks (CSF rhinorrhea or otorrhea), surgery may be needed to repair those conditions.

Prevention

The causes of IIH are not completely known, but the condition is associated with obesity and high blood pressure.