Pseudotumor cerebri syndrome

Deborah I Friedman MD MPH (Dr. Friedman of the University of Texas Southwestern Medical Center has no relevant financial relationships to disclose.)
Jonathan D Trobe MD, editor. (Dr. Trobe of the University of Michigan has no relevant financial relationships to disclose.)
Originally released March 30, 1994; last updated February 15, 2016; expires February 15, 2019

This article includes discussion of pseudotumor cerebri syndrome, benign intracranial hypertension, pseudotumor cerebri. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Pseudotumor cerebri syndrome continues to be a diagnostic and therapeutic challenge, and the incidence is rising as obesity becomes more prevalent. The author updates the clinical features of pseudotumor cerebri syndrome in adults and children, secondary causes, current therapies, and the role of cerebral venous sinus disease.

Key points

 

• Headaches, transient obscurations of vision, and pulsatile tinnitus are the most frequent early manifestations of pseudotumor cerebri syndrome in adults.

 

• Obese women of childbearing age are most commonly affected by idiopathic intracranial hypertension, but the syndrome may occur from a secondary cause in children, nonobese patients, and those over 45 years of age.

 

• The manifestations of pseudotumor cerebri syndrome in children differ from those in adults and include prominent neck or back pain, diplopia, torticollis, other focal neurologic signs, headache, visual loss, and asymptomatic papilledema.

 

• Visual acuity reduction at presentation is an ominous sign, requiring aggressive intervention.

 

• A team approach to management is ideal, and a team leader (generally a neurologist or neuro-ophthalmologist) is critical.

 

• The Idiopathic Intracranial Hypertension Treatment Trial provides evidence-based therapy for patients who have mild visual loss.

Historical note and terminology

In 1897, Quincke described a syndrome of elevated intracranial pressure and bilateral papilledema due to impaired cerebrospinal fluid circulation. Seven years later, Nonne recognized that this group of conditions mimicked an intracranial tumor and named them "pseudotumor cerebri." In 1937, Dandy suggested that pseudotumor cerebri resulted from increased cerebral blood volume. In 1955, Foley popularized the term "benign intracranial hypertension." Recognition of the complication of visual loss by Corbett in the 1980s resulted in substitution of the descriptor "idiopathic" for "benign." The diagnostic criteria were updated in 2002 to reflect advances in neuroimaging techniques and to incorporate atypical presentations (Friedman and Jacobson 2002). Revised criteria in 2013 defined the diagnosis of pseudotumor cerebri syndrome in adults and children, including criteria for those in whom papilledema is absent (Friedman et al 2013).

“Idiopathic intracranial hypertension” is the term applied when no secondary cause is found, generally in obese women of childbearing age.

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