The Historical Development of the Pseudotumor Concept

Ian Johnston M.D., Ph.D., F.R.C.S., Department of Surgery, University of Sydney, Australia

Neurosurg Focus. 2001;11(2) 

In This Article

Abstract and Introduction

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.

The disease now most commonly referred to either as PTC or IIH has been known by various names since its first clinical descriptions, which are usually, but incorrectly, attributed to Quincke[67,68] and Nonne[65] over a century ago. This variation in nomenclature reflects the continuing uncertainty about the precise nature of the condition, an uncertainty also quite clearly reflected in the variations in ideas of origin and treatment. From a historical standpoint the initial recognition of the disease depended on two critical developments of much broader significance than their relevance to PTC alone. The first was the invention in 1851 of the ophthalmoscope by von Helmholtz and its application to neurology pioneered by von Graefe, Albutt, Hughlings Jackson and others,[54] and the second was the introduction of lumbar puncture by Quincke around the turn of the century, which allowed objective measurement of the CSF pressure and analysis of its content. Likewise, the subsequent development of ideas regarding various aspects of the condition is closely linked to other important advances of wider application and it is of interest to examine the interrelationship between these advances and approaches to the disease. Whereas the history is of undoubted intrinsic interest, a historical consideration of the disease itself provides, perhaps, the most comprehensive insight into the problems still associated with an understanding of the condition. Thus, although the intention of the present paper is to review the historical development of the pseudotumor concept over the 120 years since the first descriptions, implicit in this historical survey is an attempt to address some of the outstanding issues of nomenclature, mechanism, origin, and treatment by setting them within a historical context. A scheme of periodization will be applied which, although admittedly arbitrary, aims to reflect the importance of the interrelated factors of ideas of mechanism, methods of treatment and the speciality most involved in diagnosis and management, to the understanding of, and approach to, this still perplexing condition.