Idiopathic Intracranial Hypertension (IIH) is a medical condition that results from increased spinal fluid pressure around the brain, in the absence of a tumor or other brain disorder. It is formerly known as pseudotumor cerebri. IIH is most common in women who are obese and in their childbearing years.
Headache and vision changes are the classic symptoms seen in IIH. On an eye examination, healthcare providers will see papilledema, a condition characterized by optic disc swelling of the eye due to increased pressure in the brain. It is diagnosed by a healthcare provider during an ophthalmoscopic examination.
Other common (but not exclusive) symptoms seen in patients with IIH include:
- transient visual changes
- pulsatile tinnitus (hearing a pulsating or throbbing noise in your head)
- photopsia (perceiving flashes of light)
- pain behind the eyes
- double vision
- vision loss
What It Feels Like
A headache from IIH can mimic that of a migraine or tension-type headache, making the diagnosis tricky. Plus, there is not one specific “type of headache” that people with IIH have. That being said a classic IIH headache is severe and throbbing, like a migraine. The pain can be intermittent or constant and may be associated with nausea and/or vomiting. Sometimes, people with an IIH headache will note pain behind their eyes and/or pain with eye movement.
If a healthcare provider suspects IIH and sees papilledema on an eye exam, he will order an MRI of the brain with and without contrast to check for an underlying cause of your increased intracranial pressure. Secondary causes of intracranial hypertension include (but not limited to):
- Cerebral venous thrombosis
- Brain tumor or abscess (collection of infected fluid)
- Obstructive hydrocephalus
- Subarachnoid hemorrhage
- Malignant hypertension
If there is no cause seen on the MRI, you will undergo a lumbar puncture to confirm the elevated pressure and to rule out infection through analysis of the cerebrospinal fluid or CSF. In IIH, there is no infection, so the composition of the CSF is normal. But the lumbar puncture will show an opening pressure greater than 250 mmH2O, which is diagnostic for an elevated intracranial pressure.
Treatment of IIH requires close followup with a neurologist and ophthalmologist. The mainstay therapy for IIH is a prescription medication called acetazolamide, which reduces the rate of CSF production. Your healthcare provider may consider other therapies if you have a sulfa allergy or are pregnant. Sometimes, serial lumbar punctures and corticosteroids are used in the short-term to help lower the CSF pressure but these are not long-term solutions.
If an individual’s headache is resistant to medical therapy and/or she is suffering from progressive vision loss, surgery is needed. Surgical intervention entails procedures called optic nerve sheath fenestration (ONSF) and/or a CSF shunting procedure.
In optic nerve sheath fenestration, a slit or window is made in the optic nerve sheath. This allows CSF to drain, alleviating pressure on the nerve, permitting vision to be partially or fully restored. Studies show that ONSF is particularly effective at improving vision loss, especially when performed earlier than later. In CSF shunting, spinal fluid is diverted to other parts of the body, again alleviating pressure on the brain.
A Word From Get Meds Info
The headache of IIH is variable and is produced by increased intracranial pressure, signified by papilledema on an eye examination. Treatment is critical and urgent to prevent vision loss and requires close-followup with your neurologist and ophthalmologist.