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Idiopathic intracranial hypertension (pseudotumor cerebri): Epidemiology and pathogenesis

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 commonly 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.

While the pathogenesis of IIH is unknown, risk factors have been identified and many theories advanced as to its cause. The epidemiology and pathogenesis of IIH will be reviewed here. The clinical features, diagnosis, prognosis, and treatment of this disorder are discussed separately. (See "Idiopathic intracranial hypertension (pseudotumor cerebri): Clinical features and diagnosis" and "Idiopathic intracranial hypertension (pseudotumor cerebri): Prognosis and treatment".)

EPIDEMIOLOGY

Incidence — The annual incidence of IIH is 1 to 2 per 100,000 population [2,3]. There is a higher incidence in obese women between the ages of 15 and 44 years (4 to 21 per 100,000) [3,4]. With the increasing obesity epidemic in the United States and in other parts of the world, the incidence and prevalence of IIH is rising [5].

Risk factors — IIH is a disorder that primarily affects women of childbearing age who are overweight [3,4,6-10]. In a prospective study of 50 consecutively-diagnosed IIH patients, 92 percent were women with a mean age of 31 years (range 11 to 58 years) and 94 percent were obese [9]. Other case series in different geographic areas and ethnic groups report consistent findings [4,10-15].

        

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Literature review current through: Nov 2016. | This topic last updated: Tue Aug 25 00:00:00 GMT 2015.
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