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National Organization for Rare Disorders, Inc.
- Benign Intracranial Hypertension
- Idiopathic Intracranial Hypertension (Primary Intracranial Hypertension)
- Secondary Intracranial Hypertension
Intracranial Hypertension (Pseudotumor cerebri) is characterized by increased pressure inside the skull. Intracranial means inside the skull and hypertension means high fluid pressure. Basically, the pressure of the fluid that surrounds the brain (cerebrospinal fluid or CSF) is too high. Elevated CSF pressure produces severe headache and often visual difficulties, which, if left untreated can result in loss of vision or blindness.
Pseudotumor Cerebri and Benign Intracranial Hypertension are both former names for Intracranial Hypertension (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, also 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, and steroids, especially during withdrawal), growth hormone treatments, excessive ingestion of Vitamin A, sleep apnea and certain systemic diseases such as lupus, leukemia, kidney failure (uremia), meningitis and dural venous sinus thrombosis. Many other causes have been suggested in the medical literature but have not yet been confirmed as true causes.
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.
NIH/National Institute of Neurological Disorders and Stroke
P.O. Box 5801
Bethesda, MD 20824
Intracranial Hypertension Research Foundation
6517 Buena Vista Dr
Vancouver, WA 98661
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
PO Box 241956
Los Angeles, CA 90024
Chiari & Syringomyelia Foundation
290 Broadhollow Road, Suite 210E
Melville, NY 11747
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email email@example.com
Last Updated: 11/11/2008
Copyright 1989, 1996, 1997, 1998, 2002, 2008 National Organization for Rare Disorders, Inc.