What is Idiopathic Intracranial Hypertension?

Idiopathic Intracranial Hypertension (IIH), otherwise known as Pseudotumor Cerebri (PTC) or Benign Intracranial Hypertension (BIH), is a neurological condition that is typically characterized by an increase of pressure in the skull. Basically, the pressure of the fluid that surrounds the brain (CSF) is too high. Elevated pressure can often cause severe headaches and visual disturbances.

Intracranial Hypertension can either be acute or chronic. In chronic intracranial hypertension, the increased CSF pressure can cause complications such as swelling and damage to the optic nerve, which is known as Papilledema, and can sometimes lead to vision loss.

Idiopathic Intracranial Hypertension ribbon

How is Idiopathic Intracranial Hypertension diagnosed?

After a comprehensive exam, typically including a brain scan to rule out tumors, and a lumbar puncture (spinal tap) to establish an opening pressure, will be performed in order to rule out possible other causes. In IIH, the exam is typically normal with the exception of findings related to increased CSF pressure, and visual loss.


How is it treated?

Once IIH is confirmed, comprehensive eye exams and visual field tests are performed in order to monitor any changes in vision.

Recommendations for sustainable weight loss via healthy eating including salt restriction, along with exercise is critical. If lifestyle changes are not successful, weight loss surgery may be recommended for those with a BMI greater than 40.

For many people, weight loss can be difficult. Often times subtle changes to their weight will help relieve symptoms. A drug that decreases CSF production, Acetazolamide (Diamox), is sometimes used as an alternate therapy and can also aid in weight loss.

In cases where rapid progression is present and the patient does not respond to treatments, surgery may be needed to help relieve the pressure on the optic nerve. Shunting, which involves surgically inserting a tube to drain the CSF, can be used to lower pressure. Another procedure called optic nerve sheath fenestration, where pressure on the optic nerve is relieved, is another a technique not widely utilized.


What is the prognosis?

Typically IIH improves with treatment. For some, it progressively gets worse when left untreated. Usually the condition resolves and then reoccurs. Most patients do not need surgical treatment.

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