Idiopathic intracranial hypertension (IIH) is a condition where people have high cerebrospinal fluid pressure, but the cause is unknown. Cerebrospinal fluid (CSF) is a fluid that surrounds the brain and spinal cord. It cushions these organs, carries nutrients and waste to and from them, and provides them with immune system protection. When there is too much CSF surrounding the brain, it causes increased pressure on the brain. This can cause chronic headaches, nausea, vomiting and progressive vision loss.^1,2

IIH can affect both males and females of all ages and any body type, but is more common in young women who are obese.²

Synonyms: benign intracranial hypertension, pseudotumor cerebri, primary intracranial hypertension.^2,3

Universal rare disease classifications provide a common language for recording, reporting and monitoring diseases. Please visit the Rare Disease Classifications page for more information about these internationally recognised classifications.

ORPHA:238624; Idiopathic intracranial hypertension

ICD-11: 8D60.Y Other specified increased intracranial pressure

Symptoms of intracranial hypertension may include:^1-6

• Headaches that are extreme, frequent, and/or long lasting
• Nausea or vomiting
• Brain fog or feeling ‘spaced out’
• Gradual loss of vision (edges of vision are lost first)
• Whooshing, ringing, or pulsating sounds in the ears (tinnitus)
• Some people also experience sore neck, back, arms, and eyes
• Some people have problems with memory

Please speak to your medical team to learn more about the signs and symptoms of idiopathic intracranial pressure.

Rare diseases are often serious and progressive, exhibiting a high degree of symptom complexity, leading to significant disability. Majority of the estimated two million Australians living with a rare disease meet the Australian Government’s definition for disability (in accordance to the Australian Public Service Commission and Australian Bureau of Statistics), and many experience severe and permanent disability impacts. If you or someone you care for is experiencing disability-related impacts from a rare condition, please speak with a health or disability professional for advice. Information about relevant disability support can be found at the RARE Portal’s Disability Support Information page.

There is no known cause of idiopathic intracranial hypertension.^1-4 It does not typically run in families and is not believed to be inherited.¹

In cases where people experience intracranial hypertension due to another condition, disease, medication, or injury, this is known as secondary intracranial hypertension.²

Diagnosis of idiopathic intracranial hypertension may be based on clinical examination, tests to confirm that the CSF pressure around the brain and spinal cord is high, as well as tests to rule out other possible causes.^2,4-6

This may involve a lumbar puncture (sometimes called a spinal tap) to measure CSF pressure, and an eye examination to look for swelling of the optic nerve at the back of the eye (papilledema). Brain imaging such as magnetic resonance imaging (MRI) may be used to rule out other potential causes of high CSF pressure.

As part of the diagnosis process, doctors may rule out other conditions that have similar symptoms (differential diagnosis), such as an intracranial mass, arachnoiditis, epiduritis, meningitis, subarachnoid haemorrhage, cerebral venous sinus thrombosis, and other conditions that can lead to increased intracranial pressure.^2,4

Please speak to your medical team to learn more about the available pathways for diagnosis of idiopathic intracranial hypertension.

Treatment of idiopathic intracranial hypertension is aimed at managing or reducing the symptoms and may involve a multidisciplinary care team.

In obese or overweight individuals, management strategies may include lifestyle and dietary modification, as weight loss often reduces or eliminates the symptoms of idiopathic intracranial hypertension.^2,4-7

For people who experience vision loss, surgery may be able to prevent damage to the optic nerve in the short term.^2,4-6 There are also medications which reduce the production of CSF and these may lessen the symptoms in some individuals.^2,4-6 Pain medications and management strategies may be recommended for chronic headaches.

Please speak to your medical team to learn more about the possible treatment or management options for your condition. Treatment will depend on an individual’s specific condition and symptoms. It is also important to stay connected to your medical team so that you can be made aware of any upcoming clinical trial opportunities.

Healthcare professionals involved in the care of individuals with idiopathic intracranial hypertension may include general practitioners (GP), neurologists, neurosurgeons, neuroradiologists, ophthalmologists, nurses, neuro-ophthalmologist, dieticians and occupational therapists. The need for different healthcare professionals may change over a person’s lifetime and extend beyond those listed here.

Clinical care for rare diseases often involves a multidisciplinary team of medical, care and support professionals. Please note that the information provided here is as a guide and that RVA does not necessarily monitor or endorse specific clinics or health experts.

For many rare diseases, palliative care services may be relevant and useful. Palliative care services are available for people (adults, children and their families) living with a life-limiting illness and is not only for end-of-life care. It can also help at any stage of illness from diagnosis onwards, and will look different for different people. Palliative care services provide assistance, support, resources and tools to help people manage their illness and the symptoms, ease pain, and improve comfort and quality of life. If this is relevant to you and you wish to find out more information about palliative care and how it can help you, please visit:

• Australian Institute of Health and Welfare: Palliative care Overview
• Australian Government Department of Health, Disability and Ageing: Palliative care
• Palliative Care Australia

We are not aware of any clinical care guidelines for idiopathic intracranial hypertension in Australia. If you know of any relevant care guidelines, please let us know via the Contribute page.

The following guidance is available from international experts outside Australia; however, there may be information that is not relevant or applicable to the Australian context, and may not be up to date:

• Idiopathic intracranial hypertension: consensus guidelines on management developed in 2017, and included input from United Kingdom-based neurologists, neurosurgeons, neuroradiologists, ophthalmologists, nurses, primary care doctors and patient representatives. An international panel of experts reviewed the recommendations.

• Diagnosis, treatment and monitoring of idiopathic intracranial hypertension: Consensus recommendations of the Austrian IIH network (AN4IH) developed in 2020, and included input from Austria-based neurologists, ophthalmologists, radiologists, neurosurgeons and endocrinologists.

Individuals living with rare diseases may have complex medical issues and disabilities, which are not always visible. It is often useful to refer to their medical history as well as personal information such as a medical card, doctor’s letter, or if available, a rare disease passport, for relevant information.

In addition, individuals, their parents, families and carers often develop extensive expertise on their specific rare disease. It is important to recognise that they can contribute valuable knowledge about their rare condition. Rare diseases often impact individuals differently, so it’s important to consider a person’s lived experience.

There are specific considerations around participating in rare disease research, including clinical trials. It is important to be mindful of issues such as data privacy, research ethics, consent and differences in research regulations between Australia and other countries. For more information, please visit the RARE Portal’s Considerations for Participating in Health and Medical Research page.

If you are interested in finding clinical trials for your condition, please visit the following websites; however, there may not be any clinical trials available:

• Australian Clinical Trials
• ClinicalTrials.gov

It is best to discuss your interest in research, including clinical trials,  with your medical team to determine suitability and eligibility.

Australian Organisation:
We are not aware of any rare disease organisations for idiopathic intracranial hypertension in Australia. If you are aware of any relevant Australian organisations, please let us know via the Contribute page. 

International Organisation:
Idiopathic Intracranial Hypertension UK (United Kingdom)
Website: https://www.iih.org.uk/

Please note that RVA does not monitor or endorse each group/organisation’s operational governance and activities. When engaging with a group, please consider the information on the RARE Portal’s Finding Helpful Peer and Community Supports page.

Idiopathic intracranial hypertension varies between individuals, and each person’s experience is unique.

Brain Foundation: Patient Stories has personal stories of individuals living with idiopathic intracranial hypertension.

If you would like to share your personal story with RVA, please visit the Rare Voices Australia: Share Your Story page. RVA will consider your story for publishing on our website and inclusion on the RARE Portal.

For information on available government and social services that provide support for individuals with a rare disease, please visit the National and State Services pages.

People living with a rare disease often face unique challenges such as diagnostic delays, misdiagnoses, limited treatment options, and limited access to rare disease specialists and support. These challenges may impact people’s emotional wellbeing and quality of life. Many find it helpful to seek mental health and wellbeing support to cope with ongoing stress and uncertainty. Connecting with people who have shared experiences through a support group may also be helpful. Information about relevant mental health and wellbeing support can be found at:

• Mental Health and Wellbeing Support for Australians Living with a Rare Disease
• The National and State Services pages underneath the ‘Mental Health’ sections listed

Further information on idiopathic intracranial hypertension can be found at:

• Genetic and Rare Diseases (GARD) Information Center: Benign intracranial hypertension
• National Organization for Rare Disorders (NORD): Idiopathic Intracranial Hypertension

Online Mendelian Inheritance in Man, OMIM^®: 243200; Intracranial Hypertension, Idiopathic

Orphanet: Idiopathic intracranial hypertension

Human Phenotype Ontology (HPO): Idiopathic intracranial hypertension

This page has been developed by Rare Voices Australia (RVA)’s RARE Portal team.

If you are aware of any additional information that may benefit stakeholders with an interest in this page, or if you notice any broken links or inaccurate information, please let us know via the Contribute page.