Walsh & Hoyt: Pseudotumor Cerebri: Definition

The pseudotumor cerebri syndrome was first described by Quincke in 1897. The terminology used historically in the literature gives a sense of the various conditions associated with pseudotumor cerebri (PTC): toxic hydrocephalus, otitic hydrocephalus, hypertensive meningeal hydrops, pseudoabscess, ICP without brain tumor, brain swelling of unknown cause, and papilledema of indeterminate etiology. The most popular and enduring term pseudotumor cerebri was first used in 1914. Noting that the condition was not caused by a tumor or malignancy, Foley introduced the term benign intracranial hypertension in 1955. However, the term benign also implies a favorable process or the absence of harm. As significant visual morbidity may result from PTC, the use of the prefix benign was challenged and is no longer considered acceptable terminology. The disorder is most correctly termed idiopathic intracranial hypertension (IIH) or intracranial hypertension from a secondary cause, depending upon whether or not n etiology is identified. The older term, PTC, describes the clinical syndrome without reference to the underlying cause.