Idiopathic intracranial hypertension

Erica L Archer MD (

Dr. Archer of Kennedy Ophthalmology Associates, St. Peter’s Health Partners, and Albany 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 January 17, 2019; expires January 17, 2022

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

Overview

Idiopathic intracranial hypertension (synonymous with 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 idiopathic intracranial hypertension in adults and children, primary and secondary etiologies, diagnostic evaluation and criteria, and current therapeutic options.

Key points

 

• Headaches, transient visual obscurations, and pulsatile tinnitus are the most frequent early manifestations of idiopathic intracranial hypertension in adults.

 

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

 

• The manifestations of idiopathic intracranial hypertension in children differ from those in adults and include asymptomatic papilledema, prominent neck or back pain, diplopia, torticollis, and other focal neurologic signs.

 

• Visual acuity reduction or significant visual field loss at presentation is an ominous sign, requiring aggressive intervention.

 

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

 

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

Historical note and terminology

In 1897, Quincke first 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 visual loss as a possible complication resulted in substitution of the descriptor "idiopathic" for "benign" in 1982 (Corbett 1982). 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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