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Idiopathic intracranial hypertension (pseudotumor cerebri): Prognosis and treatment

Authors
Andrew G Lee, MD
Michael Wall, MD
Section Editor
Paul W Brazis, MD
Deputy Editor
Janet L Wilterdink, MD

INTRODUCTION

Idiopathic intracranial hypertension (IIH) is also called pseudotumor cerebri. It is a disorder defined by clinical criteria that include symptoms and signs isolated to those produced by increased intracranial pressure (eg, headache, papilledema, vision loss), elevated intracranial pressure with normal cerebrospinal fluid composition, and no other cause of intracranial hypertension evident on neuroimaging or other evaluations [1].

While once called benign intracranial hypertension, to distinguish it from secondary intracranial hypertension produced by a neoplastic malignancy, it is not a benign disorder. Many patients suffer from intractable, disabling headaches, and there is a risk of severe, permanent vision loss.

Recommendations for the treatment of IIH are limited by a limited number of randomized controlled trials [2,3]. Moreover, the natural history of untreated IIH is uncertain

This topic will discuss the prognosis and treatment of IIH. The epidemiology, pathogenesis, clinical features and diagnosis of this disorder are discussed separately. (See "Idiopathic intracranial hypertension (pseudotumor cerebri): Epidemiology and pathogenesis" and "Idiopathic intracranial hypertension (pseudotumor cerebri): Clinical features and diagnosis".)

PROGNOSIS

No large prospective series describe the natural history of idiopathic intracranial hypertension (IIH). A protracted course lasting months to years appears to be common [4-8]. In most patients, symptoms worsen slowly. With treatment, there is usually gradual improvement and/or stabilization, but not necessarily recovery; many patients have persistent papilledema, elevated intracranial pressure as documented on lumbar puncture, and residual visual field deficits.

                

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Literature review current through: Jun 2016. | This topic last updated: Jun 17, 2015.
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