Overview
Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri or benign intracranial hypertension, is a condition where the pressure of the fluid surrounding the brain (called cerebrospinal fluid or CSF) becomes abnormally high without an obvious cause such as a tumor, infection, or blood clot. The word 'idiopathic' means the exact cause is unknown. This increased pressure pushes on the brain and the nerves behind the eyes, leading to a range of symptoms. The most common symptom is severe, persistent headaches that may worsen with eye movement or straining. Many people also experience visual problems, including blurred vision, double vision, brief episodes of vision going dark (called visual obscurations), and in some cases, permanent vision loss if the condition is not treated. Other symptoms can include pulsatile tinnitus (a whooshing sound in the ears that matches your heartbeat), neck pain, back pain, and nausea. IIH most commonly affects women of childbearing age, particularly those who are overweight or obese. Treatment focuses on lowering the pressure inside the skull to protect vision and relieve symptoms. This typically includes weight loss, medications like acetazolamide (Diamox) to reduce fluid production, and in more severe cases, surgical procedures such as optic nerve sheath fenestration or CSF shunting. With proper treatment, many patients can manage their symptoms effectively, though some may experience recurrences.
Also known as:
Key symptoms:
Severe persistent headachesBlurred or dimmed visionBrief episodes of vision going dark (visual obscurations)Double visionWhooshing or pulsing sound in the ears (pulsatile tinnitus)Pain behind the eyesNeck stiffness or painBack painNausea and vomitingDizzinessSwelling of the optic nerve (papilledema)Difficulty with peripheral (side) visionFatigueDifficulty concentrating or brain fogShoulder pain
Clinical phenotype terms (21)— hover any for plain English
Multifactorial
Caused by a mix of several genes and environmental factors
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
10 eventsDuke University — PHASE4
University Hospital, Montpellier — NA
Thomas Jefferson University
Assiut University — NA
King's College Hospital NHS Trust
Bnai Zion Medical Center
Assiut University
Medical University of Vienna
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Idiopathic intracranial hypertension.
16 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Idiopathic intracranial hypertension.
Community
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Start the conversation →Latest news about Idiopathic intracranial hypertension
Disease timeline:
New recruiting trial: Multifocal Chromatic Pupilloperimetry in Patients With Pseudotumor Cerebri and Healthy Subjects.
A new clinical trial is recruiting patients for Idiopathic intracranial hypertension
New recruiting trial: Stent Implantation Versus Medical Therapy for Idiopathic IntracraniaL Hypertension (SIMPLE)
A new clinical trial is recruiting patients for Idiopathic intracranial hypertension
New recruiting trial: Ocular Perfusion in Patients With Idiopathic Intracranial Hypertension
A new clinical trial is recruiting patients for Idiopathic intracranial hypertension
New recruiting trial: Biomarkers in the Etiology of Idiopathic Intracranial Hypertension
A new clinical trial is recruiting patients for Idiopathic intracranial hypertension
New recruiting trial: Diagnostic and Prognostic Biomarkers of Idiopathic Intracranial Hypertension
A new clinical trial is recruiting patients for Idiopathic intracranial hypertension
New recruiting trial: Prospective Ocular Imaging for Intracranial Pressure Evaluation
A new clinical trial is recruiting patients for Idiopathic intracranial hypertension
Caregiver Resources
NORD Caregiver Resources
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Mental Health Support
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.How severe is the swelling of my optic nerves, and is my vision currently at risk?,What is my current cerebrospinal fluid pressure, and what is the target we are aiming for?,What are the side effects of acetazolamide, and what should I do if I cannot tolerate it?,How much weight loss is recommended for my situation, and can you refer me to a weight management program?,How often do I need follow-up eye exams and visual field tests?,At what point would surgery be considered, and what are the options?,Are there any activities or medications I should avoid that could worsen my condition?
Common questions about Idiopathic intracranial hypertension
What is Idiopathic intracranial hypertension?
Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri or benign intracranial hypertension, is a condition where the pressure of the fluid surrounding the brain (called cerebrospinal fluid or CSF) becomes abnormally high without an obvious cause such as a tumor, infection, or blood clot. The word 'idiopathic' means the exact cause is unknown. This increased pressure pushes on the brain and the nerves behind the eyes, leading to a range of symptoms. The most common symptom is severe, persistent headaches that may worsen with eye movement or straining. Many people also ex
How is Idiopathic intracranial hypertension inherited?
Idiopathic intracranial hypertension follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Idiopathic intracranial hypertension typically begin?
Typical onset of Idiopathic intracranial hypertension is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Idiopathic intracranial hypertension?
Yes — 16 recruiting clinical trials are currently listed for Idiopathic intracranial hypertension on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Idiopathic intracranial hypertension?
25 specialists and care centers treating Idiopathic intracranial hypertension are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.