Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)

Pseudotumor cerebri, also known as idiopathic intracranial hypertension, is a condition where the pressure inside the skull rises unexpectedly. This increased pressure isn't caused by a tumor, but rather, for unknown reasons.

The symptoms often resemble those of a brain tumor. The extra pressure can swell the optic nerve, which is crucial for vision. This swelling can lead to vision problems, including blurry vision or even vision loss.

Doctors usually try to reduce the pressure with medication. These medications can help ease headaches and other symptoms associated with the increased pressure. However, sometimes surgery is needed to lower the pressure.

Pseudotumor cerebri can affect both children and adults, but it's more frequently diagnosed in women of childbearing age who are overweight or obese. This connection between weight and the condition is something doctors are still studying.

Pseudotumor cerebri, sometimes called intracranial hypertension, can cause a variety of symptoms. These symptoms often include:

• Headaches: These headaches are frequently intense and often start behind the eyes. Imagine a throbbing pain centered in the back of your head, or around your temples.
• Head Noise: A pulsing, whooshing sound in your head, that matches your heartbeat, can be a sign. This is sometimes described as a humming or buzzing.
• Upset Stomach: Nausea, vomiting, and dizziness are common. These feelings can be quite unpleasant and might make you feel unwell.
• Vision Problems: Vision loss is another possible symptom. This can range from blurry vision to complete loss of sight in one or both eyes.
• Temporary Blindness: Brief episodes of blindness, lasting only a few seconds, can occur. This might feel like a sudden blackout in your vision. This can happen in one or both eyes.
• Side Vision Issues: You might have trouble seeing things to the side of you. This can make it harder to see objects or people that are positioned to the side of your direct line of sight.
• Double Vision: Seeing double is another possible symptom. This means you see two images of the same object.
• Light Flashes: You might see flashes of light, like lightning, in your vision.
• Neck, Shoulder, or Back Pain: Some people with pseudotumor cerebri also experience pain in their neck, shoulders, or back.

Importantly, the symptoms may go away for a while and then return months or even years later.

The exact reason why pseudotumor cerebri happens is still a mystery. If we ever figure out a specific cause, it's then called secondary intracranial hypertension, not idiopathic pseudotumor cerebri.

Your brain and spinal cord are protected by a fluid called cerebrospinal fluid. This fluid acts like a cushion, protecting them from harm. Your body makes this fluid in your brain, and it normally gets absorbed back into your bloodstream at a steady rate. This keeps the pressure inside your head stable.

Pseudotumor cerebri might be linked to a problem with how this fluid is absorbed back into your bloodstream. This disrupted absorption could lead to a buildup of cerebrospinal fluid, causing the pressure inside your head to rise.

Pseudotumor cerebri, sometimes called idiopathic intracranial hypertension, is a condition where pressure builds up inside the skull. Several things are linked to this increased pressure, including:

• Obesity: Being significantly overweight or obese is a strong risk factor for pseudotumor cerebri. Extra weight puts extra strain on the whole body, including the delicate structures within the skull. This increased pressure can lead to the build-up of fluid and the symptoms associated with the condition.

• Certain Medications: Some medications, like birth control pills containing certain hormones, and steroids, can sometimes raise pressure inside the skull. If you're taking medications and start experiencing symptoms like headaches or vision problems, talk to your doctor.

• Vitamin A Supplements: High doses of vitamin A can also contribute to the condition. Your body needs vitamin A, but too much can be harmful. If you take vitamin A supplements, be sure to follow the recommended dosage and discuss any concerns with your doctor.

• Other Medical Conditions: Conditions like polycystic ovary syndrome (PCOS) and thyroid problems have also been connected to pseudotumor cerebri. These underlying conditions can sometimes affect the balance of fluids and other factors that can contribute to increased pressure in the brain.

• Pregnancy: Pregnancy itself can sometimes raise intracranial pressure. This is often temporary and resolves after childbirth. However, it's important to monitor for symptoms.

It's crucial to remember that these factors are associated with, not necessarily the cause of, pseudotumor cerebri. Many people with these factors never develop the condition. If you're concerned about any of these factors or experience symptoms like headaches, vision changes, or ringing in the ears, it's essential to see a doctor for proper diagnosis and treatment.

In some cases of pseudotumor cerebri, the condition can cause vision problems that get progressively worse, potentially resulting in blindness.

To figure out what's wrong, your doctor will first talk to you about your symptoms and past health, do a physical exam, and order some tests.

If your doctor thinks you might have pseudotumor cerebri, a specialist who treats eye problems (an ophthalmologist) will check for specific swelling around the optic nerve, which is at the back of your eye.

They'll also test your vision to see if you have any blind spots beyond the normal blind spot in each eye (where the optic nerve connects to the back of your eye). Pictures of your eyes and a test called optical coherence tomography (OCT) might also be done. This test measures the thickness of the layers in your eye.

Your doctor will probably order an MRI (magnetic resonance imaging) or CT (computed tomography) scan. These tests help rule out other possible problems that can cause similar symptoms, such as brain tumors or blood clots.

Your doctor may also recommend a lumbar puncture. In this test, a medical professional inserts a needle between two bones in your lower back to remove a small amount of fluid surrounding your brain and spinal cord. This fluid is then tested in a lab to measure the pressure inside your skull.

Pseudotumor cerebri is a condition that causes pressure in the brain, often leading to headaches and vision problems. The goal of treatment is to relieve these symptoms and prevent vision loss.

Lifestyle Changes:

If you have pseudotumor cerebri and are overweight, losing weight is a crucial part of treatment. A doctor might recommend a low-sodium diet and suggest working with a registered dietitian to create a personalized weight loss plan. Weight loss programs or even surgery, like gastric bypass, can also be helpful in some cases.

Medications:

One of the first medications often tried is acetazolamide, a drug also used for glaucoma. This medicine helps by reducing the amount of cerebrospinal fluid (CSF) produced in the brain. Lowering CSF levels can often ease symptoms. However, it can cause side effects like stomach upset, tiredness, tingling in the fingers, toes, and mouth, or even kidney stones.

If acetazolamide isn't enough, another type of diuretic (a medication that increases urine output) might be added to the treatment plan. These medications help the body get rid of extra fluid. Sometimes, migraine medications can help with the severe headaches often associated with pseudotumor cerebri.

Surgical Options:

If medication doesn't work or vision problems worsen, surgery may be necessary. These surgeries aim to reduce the pressure around the optic nerve and in the skull.

• Optic Nerve Sheath Fenestration: This surgery involves creating a small opening in the tissue surrounding the optic nerve. This allows excess cerebrospinal fluid to drain away, often improving or stabilizing vision. While this procedure usually helps both eyes, it's not always successful and can sometimes worsen vision problems.

• Spinal Fluid Shunt: This surgery is typically a last resort, used only after other treatments have failed. A thin tube (shunt) is placed in the brain or lower spine and connected to the abdomen. The shunt drains excess cerebrospinal fluid from the brain into the abdomen. However, shunts can sometimes become blocked, requiring further surgery, and can lead to complications like headaches or infections.

• Venous Sinus Stenting: This newer procedure involves placing a tiny tube (stent) in a large vein in the head to improve blood flow. More research is needed to fully understand the benefits and risks of this procedure.

Ongoing Monitoring:

Even after treatment, regular eye exams are crucial to monitor for any changes in vision. This is essential to catch any potential problems early on.

Being overweight significantly raises the risk of pseudotumor cerebri in young women. This condition can cause vision problems. Even a moderate amount of weight gain in women who aren't already obese can increase their risk.

Losing excess weight and maintaining a healthy weight are important steps in potentially lowering the risk of this condition. Keeping a healthy body weight can help protect your vision.