Idiopathic intracranial hypertension (IIH), previously known as pseudotumor cerebri and benign intracranial hypertension, is a condition characterized by increased intracranial pressure (pressure around the brain) without a detectable cause. The main symptoms are headache, vision problems, ringing in the ears, and shoulder pain. Complications may include vision loss.This condition is idiopathic, meaning there is no known cause. Risk factors include being overweight or a recent increase in weight. Tetracycline may also trigger the condition. The diagnosis is based on symptoms and a high opening pressure found during a lumbar puncture with no specific cause found on a brain scan.Treatment includes a healthy diet, salt restriction, and exercise. The medication acetazolamide may also be used along with the above measures. A small percentage of people may require surgery to relieve the pressure.About 2 per 100,000 people are newly affected per year. The condition most commonly affects women aged 20–50. Women are affected about 20 times more often than men. The condition was first described in 1897. Signs and symptoms
The most common symptom of IIH is headache, which occurs in almost all (92–94%) cases. It is characteristically worse in the morning, generalized in character and throbbing in nature. It may be associated with nausea and vomiting. The headache can be made worse by any activity that further increases the intracranial pressure, such as coughing and sneezing. The pain may also be experienced in the neck and shoulders. Many have pulsatile tinnitus, a whooshing sensation in one or both ears (64–87%); this sound is synchronous with the pulse.
Given the reduced need for revisions in ventricular shunts, it is possible that this procedure will become the first-line type of shunt treatment.It has been shown that in obese people, bariatric surgery (and especially gastric bypass surgery) can lead to resolution of the condition in over 95%. Prognosis
It is not known what percentage of people with IIH will remit spontaneously, and what percentage will develop chronic disease.IIH does not normally affect life expectancy. The major complications from IIH arise from untreated or treatment-resistant papilledema. In various case series, the long-term risk of ones vision being significantly affected by IIH is reported to lie anywhere between 10 and 25%. Epidemiology
On average, IIH occurs in about one per 100,000 people, and can occur in children and adults. The median age at diagnosis is 30. IIH occurs predominantly in women, especially in the ages 20 to 45, who are four to eight times more likely than men to be affected. Overweight and obesity strongly predispose a person to IIH: women who are more than ten percent over their ideal body weight are thirteen times more likely to develop IIH, and this figure goes up to nineteen times in women who are more than twenty percent over their ideal body weight. In men this relationship also exists, but the increase is only five-fold in those over 20 percent above their ideal body weight.Despite several reports of IIH in families, there is no known genetic cause for IIH. People from all ethnicities may develop IIH.