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Idiopathic Intracranial Hypertension

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Copyright 1989, 1996, 1997, 1998, 2002, 2008, 2012

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

Disorder Subdivisions

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.

Symptoms

The most common symptom is often an unbearably painful headache, sometimes associated with nausea and vomiting that is not relieved by medication. The headache often awakens the patient from sleep. Some patients are treated in the emergency room where a lumbar puncture (spinal tap) is done as a last resort, to temporarily ease the headache. Measurement of the opening pressure is encouraged during these procedures.

The diagnosis is also confirmed by detecting a high spinal CSF pressure reading, usually greater than 250 mmH2O (200-250 considered borderline high) and normal laboratory and imaging studies including CT scans and MRIs. There is generally a normal neurological examination as well, although abnormal findings may be detected on eye examination. The eye findings may be subtle, and not noted in an emergency room evaluation. It is not uncommon to misdiagnose a patient with IH as simply having a refractory migraine headache, and be treated as such. Unlike Primary IH, Secondary IH patients may have abnormal scans and laboratory tests.

The high CSF pressure may cause the optic nerves to swell (papilledema). The optic nerve connects the interior of each eye, the retina, to the vision centers of the brain. The optic nerve transmits impulses from the retina to these brain centers. The earliest sign of papilledema on a visual field test is known as an enlarged blind spot. Abnormal CSF pressure can also affect the eye muscles controlling eye movements producing double vision, but this is an infrequent event. (All patients with presumed IH should have a thorough eye examination including visual field tests by an ophthalmologist or neuro-ophthalmologist).

Other common symptoms include transient altered vision, particularly on movement or bending over, intracranial noise (pulse synchronous tinnitus), stiff neck, back and arm pain, pain behind the eye, exercise intolerance, and memory difficulties.

Causes

In the idiopathic or primary type (IIH), obesity is a significant factor in women aged 20-35.

The many potential causes of secondary intracranial hypertension have been noted above. Note that in secondary IH, unlike IIH, obesity, gender, age and race are NOT risk factors, but may be present.

The mechanism by which IH occurs is not known, but several possibilities have been suggested. Most research supports the theory that there is resistance or obstruction to CSF outflow through the normal existing pathways in the brain.

Affected Populations

The incidence if IIH in the general population is thought to be about 1 per 100,000. In obese young females the incidence of IIH is about 20 per 100,000. IIH occurs in men and children as well, but with substantially lower frequency. Weight is not usually a factor in men and in children under 10 years of age.

The true incidence of secondary IH remains unknown because of the wide range of underlying causes and the lack of published surveys on the subject. Current statistics are not available on how many people have secondary intracranial hypertension.

Related Disorders

Symptoms of the following disorders can be similar to those of IIH. Comparisons may be useful for a differential diagnosis:

Arachnoiditis is a progressive inflammatory disorder affecting the middle membrane surrounding the spinal cord and brain (arachnoid membrane). It may affect both the brain and the spinal cord and may be caused by foreign solutions (such as dye) being injected into the spine or arachnoid membrane. Symptoms may include severe headaches, vision disturbances, dizziness, nausea and/or vomiting. If the spine is involved, pain, unusual sensations, weakness and paralysis can develop. (For more information on this disorder, choose "Arachnoiditis" as your search term in the Rare Disease Database.)

Epiduritis is characterized by inflammation of the outer tough canvas- like covering surrounding the brain and spinal cord known as the dura mater. Symptoms of this disorder can be similar to IIH.

Meningitis is an inflammation of the membranes around the brain and the spinal cord. It may occur as three different forms; adult, infantile and neonatal. It may also be caused by a number of different agents such as infectious bacteria, virus, or fungi, and malignant tumors. Meningitis may develop suddenly or have a gradual onset. Symptoms may include fever, headache, a stiff neck, and vomiting. The patient may also be irritable, confused and go from drowsiness, to stupor to coma. (For more information on this disorder, choose "Meningitis" as your search term in the Rare Disease Database.)

Brain tumors may also cause symptoms similar to IIH. Neuroimaging will help with this diagnosis.

Standard Therapies

Medical treatment consists of using drugs called carbonic anhydrase inhibitors to suppress the production of CSF. The most commonly used of the carbonic anhydrase inhibitors is acetazolamide. These drugs inhibit the enzyme system needed to produce CSF and control the pressure (by controlling the volume) to some degree. These drugs do not work in all cases and can have potentially serious side effects.

When medical treatment fails and vision is at risk, one of two types of surgery may be performed. Optic nerve fenestration is a procedure in which a small opening is made in the sheath around the optic nerve in an attempt to relieve swelling (papilledema). Implantation of neurological shunts (internal tubes) is used to drain CSF into other areas of the body.

Investigational Therapies

Studies of the treatment of intracranial hypertension, begun at SUNY Upstate Medical Center are being continued in the laboratories of:

Deborah I. Friedman, MD
Department of Ophthalmology
University of Rochester School of Medicine & Dentistry
Rochester, NY 14642

The Intracranial Hypertension Research Foundation together with the Casey Eye Institute of the Oregon Health Sciences University maintain the IH Registry in which IH patients may voluntarily register. Registrants may participate (only with informed consent) in surveys and receive almost immediate news of clinical trials in which registrants may participate.

Because IH is a rare disorder affecting so few patients a central register is key to gathering epidemiological data and to notifying patients of a clinical trial of interest.

Interested persons can receive more information by contacting:

The Intracranial Hypertension Research Foundation
6517 Buena Vista Drive
Vancouver, WA 98661

Tel: 360-693-4473
Fax: 360-694-7062
Web: http//www.IHRFoundation.org

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Organizations related to Idiopathic Intracranial Hypertension

References

REVIEW ARTICLES
Friedman DI, Rausch EA. Headache diagnoses in patients with treated idiopathic intracranial hypertension. Neurology. 2002;58:1551-3.

Digre KB. Idiopathic intracranial hypertension headache. Curr Pain Headache Rep. 2002;6:217-25.

Shin RK, Balcer LJ. New developments in idiopathic intracranial hypertension. Curr Neurol Neurosci Rep. 2001;1:463-70.

Kosmorsky G. Pseudotumor cerebri. Neurosurg Clin N Am. 2001;12:775-97.

JOURNAL ARTICLES
Digre KB, Corbett JJ. Idiopathic intracranial hypertension (pseudotumor cerebri): a reappraisal. The Neurologist. 2001;7:2-67.

Banta JT, Farris BK. Pseudotumor cerebri and optic nerve sheath decompression. Ophthalmology. 2000;107:1907-12.

Corbett JJ. Pseudotumor cerebri by any other name. Arch Ophthalmol. 2000;118:850-51.

Kleinschmidt J, Digre KB, Hanover R. Idiopathic intracranial hypertension: relationship to depression, anxiety and quality of life. Neurology. 2000;54:319-24.

Zemeck G, Romner B. Seven years of clinical experience with the programmable Codman-Hakin valve: a retrospective study of 583 patients. J Neurosurg. 2000;92:941-48.

Cinciripini GS, Donahue S, Borchert MS. Idiopathic intracranial hypertension in prepubertal pediatric patients: characteristics, treatment and outcomes. Am J Ophthalmol. 1999;127:178-82.

Friedman DI. Pseudotumor cerebri. Neurosurg Clin N Am. 1999;10:609-21.

Brodsky MC, Vaphiades MD. Magnetic resonance imaging in pseudotumor cerebri . Ophthalmology. 1998;105:1686-93.

Friedman DI, Streeten DH. Idiopathic intracranial hypertension and orthostatic edema may share a common pathogenesis. Neurology. 1998;50:1099-1104.

Sismanis A. Pulsatile tinnitus. A 15 year experience. Am J Otol. 1998;19:472-77.

Burgett RA, Purvin VA, Kawasaki A. Lumboperitoneal shunting for pseudotumor cerebri. Neurology. 1997;49:734-39.

Lee AG. Pseudotumor cerebri after treatment with tetracycline and isotretinoin for acne. Cutis. 1995;55:165-68.

Rosenberg ML, Corbett JJ, Smith C, et al. Cerebrospinal fluid diversion procedures in pseudotumor cerebri. Neurology. 1993;43:1071-72.

Spoor TC, McHenry JG. Long term effectiveness of optic nerve sheath decompression for pseudotumor cerebri. Arch Ophthalmol. 1993;111:632-35.

Wall M, George D. Idiopathic intracranial hypertension. Brain. 1991;114:155-80.

Wall M. The headache profile of idiopathic intracranial hypertension. Cephalalgia. 1990;10:331-35.

Corbett JJ, Thomson HS. The rational management of idiopathic intracranial hypertension. Arch Neurol. 1989;46:1049-51.

Digre KB, Corbett JJ. Pseudotumor cerebri in men. Arch Neurol. 1988;45:866-72.

Round R, Keane JR. The minor symptoms of increased intracranial pressure: 101 patients with benign intracranial hypertension. Neurology. 1988;38:1461-64.

Report last updated: 2012/03/02 00:00:00 GMT+0