A health condition that causes increased pressure in the skull and happens for unknown reasons is called idiopathic intracranial hypertension (IIH). People who develop it may experience vision problems, headaches, nausea, and tinnitus (ringing in the ears).
In other words, IIH (formerly known as pseudotumor cerebri) is a condition that increases pressure around the brain. It occurs when cerebrospinal fluid (CSF) cushions the spinal cord and brain, which causes it to build up in the skull, and may affect the optic nerve. That’s why people with IIH have vision problems. Do not hesitate to see a doctor if you experience tinnitus along with vision changes.
According to some research, this condition occurs rarely, and it affects about 2 out of 100,000 people worldwide. While IIH is not a life-threatening condition, it may cause permanent vision changes that negatively affect your quality of life.
What Are The Symptoms of Idiopathic Intracranial Hypertension?
People who develop this condition often experience the following symptoms. These include:
- Headaches that are usually sudden and severe
- Fatigue (extreme tiredness)
- Double vision
- Nausea
- Vomiting
- Reduced or loss of peripheral vision
- Neck or shoulder pain
- Temporary blindness or blind spots in vision
- Ringing in the ears
There are a lot of other conditions that may cause the previous symptoms. That’s why, if any of them occur, it is recommended to visit a doctor for a diagnosis and prompt treatment. Otherwise, it may lead to serious complications.
Causes and Risk Factors
Experts do not fully understand what exactly causes this condition. However, they think a blockage in the path where CSF goes to the brain or a large vein in the brain becoming narrower contributes to IIH. These problems may cause fluid or blood to return when they try to exit the brain. As a result, the intracranial pressure increases.
Nowadays, researchers continue to learn more about whether this condition is hereditary (meaning IIH runs in families) because some people with IIH report family members with the same disorder. Moreover, doctors have identified some factors that may increase your risk. Examples include:
- Sex – IIH tends to occur more in females than males.
- Age – Most diagnosed cases are in people between the ages of 20 and 45.
- Obesity (excessive body weight)
- Endocrine disorders – These include hypoparathyroidism (underactive parathyroid glands), Addison’s disease, polycystic ovary syndrome (PCOS), and others.
- Obstructive sleep apnea (OSA)
- Anemia (low red blood cell count)
In addition, there are some medicines that are often associated with IIH. For example, tetracycline antibiotics (including Minocycline and Doxycycline), retinoids (such as Isotretinoin), growth hormones (including Lithium carbonate), and corticosteroids (a group of medicines used to reduce inflammation).
Complications
Those who ignore the symptoms and do not get treatment may also experience some complications. Check some of them below:
- Permanent loss of vision – In most cases, doctors prescribe medicines to treat vision problems in people with IIH. However, if it is left untreated, you may experience permanent blindness.
- Optic atrophy – This is permanent damage to the optic nerve fibers.
- Papilledema (swelling of the optic disc)
- Transient visual obscurations
- Peripheral vision loss
- Chronic headaches
- Cranial nerve palsy – IIH often affects the sixth cranial nerve and causes weakness or even paralysis, which often causes double vision (also known as diplopia).
- Pulsatile tinnitus
This document does not list all possible complications of IIH. In any case, if you get treatment in the early stages, you may avoid any of the previous complications. In addition, there is no way to prevent this condition because doctors do not fully understand what exactly causes it. To reduce the risk, physicians may recommend getting or maintaining a healthy weight, quitting smoking, limiting or avoiding alcoholic drinks, and managing chronic health conditions (including hyperlipidemia, diabetes, hypertension, and others).
Diagnosis
Like the diagnosis of many other health conditions, the diagnosis of IIH starts with a physical and neurological examination. Physicians may also ask some questions about your symptoms and medical history to get more information about your disease. They may also perform the following tests and procedures to exclude other health conditions that may cause symptoms similar to IIH. Examples include:
- Visual field test – It is used to check for blind spots in the vision.
- Brain CT (computerized tomography) or MRI (magnetic resonance imaging) scans
- Lumbar puncture (also known as a spinal tap)
Treatment
Generally, the treatment goal is to reduce pressure on the brain and prevent vision loss. Usually, doctors recommend medicines, surgery, and weight management to treat IIH and its complications.
Medicines
These include:
- Topiramate and Acetazolamide to reduce the production of CSF (cerebrospinal fluid)
- Diuretics (also known as water pills) to reduce fluid retention
- Over-the-counter (OTC) and prescription pain relievers
It is also important to inform your doctor if you are pregnant, plan to become pregnant, or are taking birth control pills.
Surgery
Doctors usually recommend this treatment for people with severe idiopathic intracranial hypertension. It often includes:
- Shunt – This is a thin and long tube that is placed in the brain to drain excess cerebrospinal fluid.
- Stent – This is another small tube that is used in the venous sinus to widen it.
- Optic nerve sheath fenestration – This is an eye surgery that doctors may perform in people with IIH. It involves small cuts (incisions) around the optic nerve to allow CSF drainage.
Lifestyle Changes
Along with the previous treatments, doctors may also recommend regular physical exercise, dietary changes, quitting smoking, and avoiding alcoholic drinks to get or maintain a healthy weight. According to some research, excessive fat in the chest and abdominal area makes it difficult for blood to reach these areas. Thus, the fluid begins to collect in the brain, causing IIH.
Frequently Asked Questions
Is idiopathic intracranial hypertension a brain tumor?
No, idiopathic intracranial hypertension (IIH) is not a brain tumor. It causes increased pressure around the brain, often because of problems involving cerebrospinal fluid (CSF) circulation or drainage. It was previously called pseudotumor cerebri because its symptoms, particularly headaches and swelling of the optic nerve, can resemble those caused by a brain tumor.
Can idiopathic intracranial hypertension cause blindness?
Yes. Increased pressure can damage the optic nerves and cause progressive or permanent vision loss. Prompt diagnosis and treatment are important because vision loss can sometimes become permanent if the condition is not managed in the early stages.
What does an IIH headache feel like?
An IIH headache can feel like a pressure or throbbing pain and may be severe. It can sometimes become worse when coughing, sneezing, bending over, or straining. Headaches vary from person to person and may resemble migraine or other types of headaches.
Can idiopathic intracranial hypertension go away?
IIH can improve or go into remission in some people, particularly when contributing factors are addressed. However, symptoms can return, so ongoing medical follow-up may be necessary. Regular monitoring is especially important for protecting vision.
How is idiopathic intracranial hypertension diagnosed?
Doctors usually diagnose IIH by evaluating symptoms, examining the eyes and nervous system, and ruling out other causes of increased pressure. Tests may include brain MRI or CT imaging, a visual field examination, and a lumbar puncture to measure CSF pressure and analyze the fluid.
Can weight loss help treat idiopathic intracranial hypertension?
For people with IIH who are overweight or obese, weight loss can significantly improve symptoms and may help lower intracranial pressure. A healthcare professional can recommend a safe and appropriate weight-management plan.
What is papilledema in idiopathic intracranial hypertension?
Papilledema is swelling of the optic disc caused by increased pressure around the brain. It is an important sign of IIH because prolonged swelling can damage the optic nerve and affect vision.
Can idiopathic intracranial hypertension cause ringing in the ears?
Yes, IIH can cause pulsatile tinnitus, which is a rhythmic ringing, whooshing, or pulsing sound that may seem to follow the heartbeat. New tinnitus accompanied by vision changes or severe headaches should be evaluated by a healthcare professional.
How long does treatment for idiopathic intracranial hypertension take?
Treatment duration varies depending on the severity of IIH, symptoms, vision changes, and response to treatment. Some people improve within months, while others require longer-term treatment and monitoring. The main goals are to control symptoms and prevent permanent vision loss.
When should someone with suspected idiopathic intracranial hypertension see a doctor?
Anyone experiencing persistent or severe headaches together with vision changes, blind spots, double vision, or pulsatile tinnitus should seek medical evaluation. Sudden or significant vision loss requires urgent medical attention because untreated increased intracranial pressure can permanently damage the optic nerve. Ask your healthcare professional if you have additional questions.
