Idiopathic Intracranial Hypertension: Clinical Syndrome

Idiopathic intracranial hypertension (IIH) was first described by Quincke in 1897. Nonne called it pseudotumor cerebri in 1904. It has also been named otitic hydrocephalus, hypertensive meningeal hydrops and toxic hydrocephalus. The disorder was not well delineated as a clinical entity until the 1940's when cerebral angiography with water soluble contrast agents was added to pneumoencephalography as sensitive techniques to diagnose cases of mass lesions. Foley coined the term benign intracranial hypertension in 1955. Weisberg's large series further defined the entity but it was not until studies done in the 1980's in which the high incidence of visual loss was pointed out by Corbett that necessitated the term benign being dropped. Many disease associations have been reported but only few are linked to IIH when the case-control method is used. The etiology of IIH has remained elusive.