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Idiopathic intracranial hypertension

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Other Names:
Intracranial hypertension, idiopathic; Pseudotumor cerebri; IIH
Categories:
Idiopathic intracranial hypertension (IIH), formerly known as pseudotumor cerebri, is a condition that affects the brain. Pseudotumor cerebri literally translates to "false brain tumor." This term was used because symptoms of IIH resemble those of brain tumors depsite no tumor being present. Symptoms of IIH may include severe headache, nausea and vomiting, altered vision, and pulsating sounds within the head. A person with IIH may also have symptoms such as a stiff neck, back or arm pain, eye pain, and memory problems. If the condition remains untreated, permanent visual loss or blindness may develop.

While the exact underlying cause of IIH is not known (idiopathic), it is likely due to high fluid pressure within the skull that is due to buildup, or poor absorption, of cerebrospinal fluid (CSF). IIH most often (but not always) occurs in young, overweight, females. When symptoms of intracranial pressure have an identifiable cause such as another underlying disease or medication, the condition is instead referred to as secondary IH.

Treatment for IIH may include medications called carbonic anhydrase inhibitors to control the production of CSF. In some cases, weight loss may also be recommended. However, medications and/or weight loss are not effective in all cases. In severe cases that do not respond to medication and weight loss, surgery may be needed to relieve symptoms and/or preserve vision.[1][2]
Last updated: 4/12/2018

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Increased intracranial pressure
Rise in pressure inside skull
0002516
30%-79% of people have these symptoms
Allergy 0012393
Obesity
Having too much body fat
0001513
Papilledema 0001085
5%-29% of people have these symptoms
Abnormal emotion/affect behavior 0100851
Blurred vision 0000622
Diplopia
Double vision
0000651
Nausea 0002018
Photophobia
Extreme sensitivity of the eyes to light
Light hypersensitivity
[ more ] 		0000613
Scintillating scotoma 0010822
Sleep disturbance
Difficulty sleeping
Trouble sleeping
[ more ] 		0002360
Visual loss
Loss of vision
Vision loss
[ more ] 		0000572
Vomiting
Throwing up
0002013
1%-4% of people have these symptoms
Back pain 0003418
Depressivity
Depression
0000716
Focal sensory seizure with olfactory features 0011161
Lethargy 0001254
Migraine
Intermittent migraine headaches
Migraine headache
Migraine headaches
[ more ] 		0002076
Pulsatile tinnitus 0008629
Vertigo
Dizzy spell
0002321
Percent of people who have these symptoms is not available through HPO
Autosomal recessive inheritance 0000007
Hypertension 0000822
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Last updated: 7/1/2020
Management options for idiopathic intracranial hypertension (IIH) can vary from person to person and may involve regular eye exams to monitor vision changes, medications, weight loss, and in some cases, surgery.

Medications that may be used to reduce CSF build-up and relieve intracranial pressure include acetazolamide and furosemide. Weight loss through dieting or weight loss surgery may also be recommended for people with IIH who are overweight or obese, although this does not lead to improvement of symptoms in all cases. If the above management options are not successful and symptoms are severe or permanent vision loss is possible, surgery may be needed. Types of surgery that may be needed may include a procedure to remove pressure on the optic nerve (optic nerve sheath fenestration), and CSF shunting, which involves surgically inserting a tube to drain cerebrospinal fluid.[1][2]

Detailed information about management options for IIH are available on Medscape Reference's website by clicking here.

Last updated: 4/12/2018
The outlook (prognosis) associated with idiopathic intracranial hypertension (IIH) is quite variable and difficult to predict in each person. In some cases, it goes away on its own within months. However, symptoms may return.[3] It has been reported that regaining weight that was previously lost has been associated with symptoms returning in some people.[4] Some individuals with IIH experience progressive worsening of symptoms, leading to permanent vision loss.[1][3] Symptoms that cause pain and loss of visual function cause much of the burden associated with IIH.[5]

IIH is not known to be associated with any specific mortality risk, but surgical procedures used to treat severe cases may affect quality of life or lead to a shortened lifespan due to complications.[5]
Last updated: 4/12/2018

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Idiopathic intracranial hypertension. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • The Mayo Clinic Web site provides further information on Idiopathic intracranial hypertension.
  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The Merck Manual provides information on this condition for patients and caregivers. 
  • The National Eye Institute (NEI) was established by Congress in 1968 to protect and prolong the vision of the American people. Click on the link to view information on this topic. 
  • The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Merck Manual for health care professionals provides information on Idiopathic intracranial hypertension.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Idiopathic intracranial hypertension. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

  1. NINDS Pseudotumor Cerebri Information Page. National Institute of Neurological Disorders and Stroke (NINDS). May 24, 2017; https://www.ninds.nih.gov/Disorders/All-Disorders/Pseudotumor-Cerebri-Information-Page.
  2. Idiopathic Intracranial Hypertension. National Organization for Rare Disorders (NORD). 2015; https://rarediseases.org/rare-diseases/idiopathic-intracranial-hypertension/.
  3. Pseudotumor cerebri. MedlinePlus. February 3, 2015; http://www.nlm.nih.gov/medlineplus/ency/article/000351.htm.
  4. Wakerley B, Tan M, Ting E. Idiopathic intracranial hypertension. Cephalalgia. March, 2015; 35(3):248-261. http://www.ncbi.nlm.nih.gov/pubmed/24847166.
  5. Gans MS. Idiopathic Intracranial Hypertension. Medscape Reference. May 17, 2017; http://emedicine.medscape.com/article/1214410.