Idiopathic Intracranial Hypertension

Idiopathic intracranial hypertension, formerly called pseudotumor cerebri, is a syndrome of elevated intracranial pressure in the absence of an intracranial mass, hydrocephalus, vascular lesion or meningeal pathology. It primarily affects women of reproductive age with overweight or recent rapid weight gain.

Patients present with daily headache that is often pulsatile and worse on awakening, transient visual obscurations, pulsatile tinnitus, neck and back pain and progressive visual loss. Funduscopy reveals bilateral papilledema. MRI may show empty sella, flattening of the posterior globe, distended optic nerve sheaths and transverse sinus stenosis.

Diagnosis follows the modified Dandy criteria with confirmation by lumbar puncture showing opening pressure above 25 cm H2O and normal cerebrospinal fluid composition. Management combines weight reduction, oral acetazolamide, topiramate in selected cases, and serial ophthalmologic follow-up. Surgical options including optic nerve sheath fenestration, cerebrospinal fluid shunting and venous sinus stenting are reserved for fulminant or treatment-resistant disease.