The historical development of the pseudotumor concept

Abstract

<jats:p>The author outlines the history of the development of the pseudotumor concept, first specifically formulated by Nonne in 1904 but implicit in earlier descriptions. A scheme of periodization is applied, identifying otological, neurosurgical, neurological, and neuroophthalmological periods; he relates theoretical and practical developments in the understanding of the condition to the concerns of the dominant speciality involved in diagnosis and management. Key studies from each period are discussed in relation to the issues of mechanism, origin, nomenclature, diagnosis, and treatment. During the otological period, approaches to the condition were heavily influenced by its common association with middle ear infection, often with venous sinus involvement, and this led to the idea of disturbed cerebrospinal fluid dynamics as the cause. The advent of neuroradiological systems ushered in the neurosurgical period in which the significant but unfinished debate began: which intracranial compartment is primarily implicated in the intracranial hypertension? By the time of the neurological period cerebral edema was implicated as the cause, and this coincided with the availability of appropriate treatment methods and steroidal and diuretic agents. Toward the end of this period additional major advances in investigative methods again brought the issue of mechanism into contention, although a resolution was not to follow. The neuroophthalmological period is characterized by an increased awareness of the significance of visual loss and a return to one of the original methods of treatment. Finally, the possible significance of impaired cranial venous outflow, a thread running through all periods, is considered.</jats:p>

Pseudotumor Cerebri: Brief Review of Clinical Syndrome and Imaging Findings

“…1 Heinrich Quincke, an early pioneer in the use of lumbar puncture, reported the first recorded cases of intracranial hypertension of unknown cause in what he described as "meningitis serosa" in 1893; at that time, he posited that inadequate CSF resorption was responsible for the syndrome, a theory that is still entertained by some researchers. 2 The term "PTC" was coined in 1904 by Nonne to describe a condition characterized by symptoms associated with intracranial tumors with an unusual course of remission and subsequently termed "benign intracranial hypertension" by Foley in 1955. 1 The absence of a clear identifiable etiology for a clinical syndrome characterized by elevated ICP exists in nearly 90% of cases, and this ambiguity inevitably has led to the replacement of the misnomer "benign" intracranial hypertension with IIH in light of the incidence of vision loss resulting from this condition.…”

More Than Meets The Eye? Redefining Idiopathic Intracranial Hypertension

“…The history of IIH is quite unique, featuring only limited advancements in evidenced-based treatments, but boasting literally countless changes in its nomenclature, proposed etiology, and conceptual approach. Although all these variations may simply underscore its elusive pathogenesis, a review of the literature over the last century does reveal a significant evolution in our clinical approach to IIH that can be sequentially traced through identifiable periods (Johnston, 2001). Contemporary research suggests that we are now heralding a new phase in our ap-HISTORY IIH has been known by various names over the years, and diagnostic limitations challenge precisely establishing the first description of this clinical entity in history.…”

“…At least two of these patients likely fit the modern concept of PTC, and he postulated many of these patients had a sinus venous thrombosis secondary to otitis media. This otologic concept was accentuated by the frequency of middle ear infections at the time, and the clinical entity was eventually referred to as "otitic hydrocephalus" by Simmonds in the 1930s (Johnston, 2001;Symonds, 1931). Simmonds suggested excessive secretion from the choroid plexus or defective absorption through the arachnoid villi may be involved in the etiology as well, and CSF drainage by lumbar puncture was the mainstay of treatment.…”

“…This also triggered the neurologic period, as management trans- (Johnston, 2001). Etiology was now being attributed mostly to cerebral edema, and management included agents to counter cerebral edema, including acetazolamide, diuretics, and dexamethasone (Joynt & Sahs, 1956).…”

“…The development of ventriculography and decompressive surgery in the 1930s ushered in the neurosurgical period (Johnston, 2001 (Dandy, 1937). Given the normal CSF composition and the normal ventricular size, Dandy postulated that CSF was not involved in the etiology of PTC and thought that transient cerebral blood volume changes were the culprit.…”