Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a condition when the pressure inside the skull increases without an apparent cause (some common, identifiable causes of increased intracranial pressure might include a brain tumor or a vascular problem in the brain). This increased intracranial pressure leads to a pressure build up around the brain and the spinal cord which can cause permanent neurologic deficits.

Idiopathic intracranial hypertension can occur in anyone, however the risk is up to 20 times higher in overweight women of childbearing age. A subset of patients is children; although in children, there is no predilection for gender or weight. By definition, IIH occurs for no apparent reason, however there are some medications that are associated with increased intracranial pressure. These medications include oral contraceptive pills, tetracycline, nalidixic acid, cyclosporine and vitamin A.

The most common symptom of IIH is headache. Headaches are diffuse and can be associated with vomiting. Vision changes can also be seen with IIH. These vision changes can include short episodes of temporary vision loss, especially with changes in position, reduced peripheral vision, and/or double vision. Patients can also experience pulsatile tinnitus (ringing or whooshing in the ears).

IIH is diagnosed first with a complete eye exam, which includes dilation of the pupils. Your ophthalmologist will examine the optic nerves closely, as increased intracranial pressure can lead to optic nerve swelling, or papilledema. Additionally, your ophthalmologist will likely get tests, such as a picture of the optic nerve and a visual field test. In order to confirm the diagnosis, patients will need to have a CT or MRI of the brain, as well as a lumbar puncture (spinal tap). Patients will need to follow closely with their ophthalmologist to monitor improvement or progression.

The most common symptom of IIH is headache. Headaches are diffuse and can be associated with vomiting. Vision changes can also be seen with IIH. These vision changes can include short episodes of temporary vision loss, especially with changes in position, reduced peripheral vision, and/or double vision. Patients can also experience pulsatile tinnitus (ringing or whooshing in the ears).

Because IIH is commonly associated with obesity, weight loss is encouraged. A weight loss of 5-10% has been shown to improve symptoms. The most common medications that are used to treat IIH are acetazolamide (Diamox), topiramate (Topamax), and furosemide (Lasix). These medications have been shown to decrease the intracranial pressure and/or improve headache symptoms. Sometimes, other types of headache, such as migraine or other chronic headache, can occur at the same time as IIH headaches, and patients need to follow up with their primary care physician or neurologist to manage those headaches. Finally, in patients with very high pressures or significant vision loss, surgical treatment may be necessary. These surgical procedures include an optic nerve sheath fenestration, performed by an ophthalmologist, or a CSF shunt procedure, performed by a neurosurgeon.