Idiopathic intracranial hypertension

Alternate names

Intracranial hypertension, idiopathic; Pseudotumor cerebri; IIH

Definition

Idiopathic intracranial hypertension (IIH), formerly known as pseudotumor cerebri, is a condition that affects the brain. Pseudotumor cerebri literally translates to "false brain tumor." This term was used because symptoms of IIH resemble those of brain tumors despite no tumor being present. It is likely due to high pressure within the skull caused by the buildup or poor absorption of cerebrospinal fluid (CSF). The disorder is most common in women between the ages of 20 and 50.

Cause

• Experts don’t know what causes IIH.
• While the exact underlying cause of IIH is not known (idiopathic), it is likely due to high fluid pressure within the skull that is due to buildup, or poor absorption, of cerebrospinal fluid (CSF).
• IIH most often (but not always) occurs in young, overweight, females.
• When symptoms of intracranial pressure have an identifiable cause such as another underlying disease or medication, the condition is instead referred to as secondary IH.

Risk factors

• IIH is rare, but some people are at higher risk.
• It’s most common in women ages 20 to 50.
• Being overweight or obese also makes IIH more likely.
• You may be at higher risk if you have a body mass index (BMI) greater than 30, or if you recently gained weight.

Signs and symptoms

• Symptoms of IIH may include severe headache, nausea and vomiting, altered vision, and pulsating sounds within the head.
• A person with IIH may also have symptoms such as a stiff neck, back or arm pain, eye pain, and memory problems.
• If the condition remains untreated, permanent visual loss or blindness may develop.

Symptoms can include:

• Headaches
• Tinnitus (ringing in the ears)
• Temporary blindness
• Double vision
• Blind spots
• Neck and shoulder pain
• Peripheral (side) vision loss

Clinical presentation

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 80%-99% of people have these symptoms

• Increased intracranial pressure(Rise in pressure inside skull)

30%-79% of people have these symptoms

• Allergy
• Obesity(Having too much body fat)
• Papilledema

5%-29% of people have these symptoms

• Abnormal emotion/affect behavior
• Blurred vision
• Diplopia(Double vision)
• Nausea
• Photophobia(Extreme sensitivity of the eyes to light)
• Scintillating scotoma
• Sleep disturbance(Difficulty sleeping)
• Visual loss(Loss of vision)
• Vomiting(Throwing up)

1%-4% of people have these symptoms

• Back pain
• Depressivity(Depression)
• Focal sensory seizure with olfactory features
• Lethargy
• Migraine(Intermittent migraine headaches)
• Pulsatile tinnitus
• Vertigo(Dizzy spell)

Diagnosis

The neurologist will check to make sure your symptoms aren’t happening because of another health problem, like a brain tumor. The neurologist may do tests including:

• A physical exam
• Brain imaging, like a CT or MRI scan
• A spinal tap (lumbar puncture) to test your CSF

Treatment

Treatments include:

• Weight loss
• For people who are overweight or obese and have IIH, weight loss is usually the first treatment.
• Losing about 5 to 10 percent of your body weight can help lessen your symptoms — for example, if you weigh 200 pounds, that means losing about 10 to 20 pounds.

Medicine

• Your doctor may recommend a medicine called acetazolamide (Diamox) in addition to weight loss.

This medicine helps your body make less CSF. Surgery

• If other treatments don’t work, your doctor might suggest surgery to help relieve the pressure.
• In shunt surgery, doctors make a small hole and add a thin tube, called a shunt, to help extra fluid drain from around your brain into the rest of your body.
• There is also an eye surgery where doctors make a small hole in the covering around the optic nerve.

Prognosis

The disorder may cause progressive, permanent visual loss in some patients. In some cases, pseudotumor cerebri recurs.

NIH genetic and rare disease info

• NIH info on Idiopathic intracranial hypertension

Idiopathic intracranial hypertension is a rare disease.

Idiopathic intracranial hypertension Resources
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