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Idiopathic Intracranial Hypertension

Abstract

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NORD is very grateful to Bradley K. Farris, MD, Dean A. McGee Eye Institute and Professor, Departments of Ophthalmology, Neurology & Neurosurgery, University of Oklahoma School of Medicine, for assistance in the preparation of this report.

Synonyms of Idiopathic Intracranial Hypertension

  • benign intracranial hypertension
  • pseudotumor cerebri

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Intracranial Hypertension (IH) is characterized by increased pressure inside the skull. Intracranial means inside the skull and hypertension means high fluid pressure. Intracranial hypertension means that the pressure of the fluid that surrounds the brain (cerebrospinal fluid or CSF) is too high. Elevated CSF pressure often produces severe headache with visual difficulties, which, if left untreated can result in permanent loss of vision or blindness.

Pseudotumor cerebri and benign intracranial hypertension are both former names for IH, which are now considered inaccurate. These names do not adequately describe the disorder and downplay the seriousness of IH. There are two categories of IH: primary intracranial hypertension and secondary intracranial hypertension.

Primary intracranial hypertension, now known as idiopathic intracranial hypertension (IIH), occurs without known cause. This form most often occurs in young, overweight, females in their reproductive years (ages 20-45).

Secondary intracranial hypertension has an identifiable, causative agent, including drugs
(such as tetracycline, lithium, Vitamin A-derived oral acne medications or excessive ingestion of Vitamin A, and oral or intrathecal steroids, growth hormone treatments), sleep apnea and certain systemic diseases such as lupus, leukemia, kidney failure (uremia), meningitis and dural venous sinus thrombosis. There is an association of IH and Chiari type I malformation. Many other causes have been suggested in the medical literature but have not yet been confirmed as true causes. It is critical in these patients to rule out an intracranial space occupying mass by neuro-imaging (CT or MRI).

Although many factors are known to trigger the disease, the mechanism by which IH occurs, in either primary or secondary forms, is not known. In many cases, either type of IH may be chronic.

Organizations related to Idiopathic Intracranial Hypertension

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