Idiopathic intracranial hypertension (IIH, pseudotumor cerebri) is a syndrome of 
elevated intracranial pressure of unknown cause that occurs predominantly in 
obese women of childbearing age. It is a diagnosis of exclusion and, therefore, 
other causes of increased intracranial pressure must be sought with history, 
imaging, and cerebrospinal fluid examination before the diagnosis can be made. 
IIH produces symptoms and signs of increased intracranial pressure, including 
papilledema. If untreated, papilledema can cause progressive irreversible visual 
loss and optic atrophy. The treatment approach depends on the severity and time 
course of symptoms and visual loss, as determined by formal visual field 
testing. The main goals of treatment are alleviation of symptoms, including 
headache, and preservation of vision. All overweight IIH patients should be 
encouraged to enter a weight-management program with a goal of 5-10 % weight 
loss, along with a low-salt diet. When there is mild visual loss, medical 
treatment with acetazolamide should be initiated. Other medical treatments can 
be added or substituted when acetazolamide is insufficient as monotherapy or 
poorly tolerated. When visual loss is more severe or rapidly progressive, 
surgical interventions, such as optic nerve sheath fenestration or cerebrospinal 
fluid shunting, may be required to prevent further irreversible visual loss. The 
choice of intervention depends on the relative severity of symptoms and visual 
loss, as well as local expertise. At present, the role of transverse venous 
sinus stenting remains unclear. Although there are no evidence-based data to 
guide therapy, there is an ongoing randomized double-blind placebo-controlled 
treatment trial, investigating diet and acetazolamide therapy for IIH.