Idiopathic intracranial hypertension

Idiopathic intracranial hypertension (IIH), also known as primary pseuotumor cerebri, is a syndrome of elevated intracranial pressure (ICP) of unidentified etiology, that predominantly affects young obese women. The diagnostic criteria are based on the revised Dandy criteria, and requires the presence of elevated opening pressure on lumbar puncture, presence of papilledema and normal neurological examination, neuroimaging, and CSF composition. Symptoms commonly included headaches, visual loss, impaired visual fields, diplopia and pulsatile tinnitus. Others symptoms can occasionally occur such as rhinorrhea and cranial nerve palsy. Neuroimaging is required and a brain MRI with gadolinium is the study of choice to exclude underlying disease such as a space-occupying mass lesion, abnormal meningeal enhancement or venous sinus thrombosis and to demonstrate typical MRI findings of IIH such as empty sella turcica, flattening of the posterior aspect of the globe, optic nerve protrusion, distension of the perioptic subarachnoid space and transverse sinus stenosis. Multiple medical and surgical therapies are employed for treatment of IIH, but there are currently no established guidelines to standardize the treatment of IIH. Investigators of the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) reported beneficial effects of acetazolamide and weight loss in patients with mild visual loss. Surgical procedures are based in optic nerve sheath fenestration or cerebrospinal fluid diversion. More recently, venous sinus stenting has matured as a promising treatment for IIH patients with associated venous sinus stenosis.