Idiopathic intracranial hypertension – Where are we now?

Abstract

Idiopathic intracranial hypertension (previously known as pseudotumor cerebri or benign intracranial hypertension) is a syndrome of increased intracranial pressure of unclear etiology that most often occurs in women of childbearing age, although it can also occur in men, children, and older adults. Most patients are overweight or obese with a history of recent weight gain. Papilledema is the hallmark clinical sign and, if severe and untreated, can result in irreversible vision loss. Recent population studies in the USA have found that the annual incidence of idiopathic intracranial hypertension is increasing in association with obesity rates, while recent scientific studies indicate a possible role for androgen sex hormones and adipose tissue in the pathogenesis of the disease. The main goals of treatment are to preserve vision and alleviate symptoms, which can usually be achieved using a combination of weight loss, medical therapies, and surgical interventions. Prospective clinical trials have found benefit from weight loss, acetazolamide, and topiramate in the management of mild to moderate severity disease. However, the indications for surgical intervention, optimal timing and choice of intervention, and their long-term outcomes remain unclear due to a lack of prospective and controlled clinical trials evaluating surgical interventions for idiopathic intracranial hypertension.