Pseudotumor cerebri


Pseudotumor cerebri

Description, Causes and Risk Factors:

Pseudotumor cerebri literally means "false brain tumor." It is likely due to high pressure within the skull caused by the buildup or poor absorption of cerebrospinal fluid (CSF). The disorder is most common in women between the ages of 20 and 50.

The exact cause of pseudotumor cerebri in most individuals is unknown, but it may be linked to an excess amount of cerebrospinal fluid (CSF) within the bony confines of your skull.

Your brain and spinal cord are surrounded by cerebrospinal fluid, which acts like a cushion to protect these vital tissues from injury. This fluid is produced in the brain and eventually is absorbed into the bloodstream. The increased intracranial pressure of pseudotumor cerebri may be a result of a problem in this absorption process.

In general, your intracranial pressure increases when the contents of your skull exceed its capacity. For example, a brain tumor typically increases your intracranial pressure because there's no room for it.

The same thing happens if your brain swells or if you have too much cerebrospinal fluid. Recent evidence indicates that the majority of people with pseudotumor cerebri have a narrowing (stenosis) in two large sinuses in the brain (transverse sinuses), but it's not clear whether the narrowing is a cause or effect of the condition.

Other Risk Factors:

Medical conditions associated with IIH:

    Addison's disease.

  • Head injury.

  • Kidney disease.

  • Lupus.

  • Lyme disease.

  • Mononucleosis.

  • Polycystic ovary syndrome.

  • Sleep apnea.

  • Underactive parathyroid glands.

Medications that can cause this include:

    Growth hormone.

  • Oral contraceptives.

  • Tetracycline.

  • Discontinuation of steroids.

  • Excess vitamin A.

Symptoms:

Symptoms of pseudotumor cerebri include symptoms that closely mimic large brain tumors and they may include:

    Headache.

  • Transient light flashes.

  • Intracranial noises (pulsatile tinnitus).

  • Photopsia.

  • Retrobulbar pain.

  • Diplopia.

  • Sustained visual loss.

  • Nausea.

  • Vomiting.

  • Pulsating sounds within the head.

Diagnosis:

Because the headache features are nonspecific, a thorough exam of the back of the eye (the fundus) is critical to identify patients with Pseudotumor cerebri. When papilledema is present, this suggests elevated intracranial pressure, which can have many etiologies in addition to Pseudotumor cerebri.You will also undergo a visual fields test to see if there are any blind spots in your vision besides your so-called normal blind spot in each eye where the optic nerve enters the retina.

Brain imaging: CT or MRI scans can rule out other problems that can cause similar symptoms, such as brain tumors and blood clots.

Ultrasonography: In the emergency department, bedside ultrasonography has been used to identify intracranial hypertension by measuring the diameter of the optic nerve sheath.

Spinal tap (lumbar puncture): A lumbar puncture — which involves inserting a needle between two vertebrae in your lower back — can determine how high the pressure is inside your skull.

Other Tests:

    Complete blood count.

  • Erythrocyte sedimentation rate (ESR).

  • Serum iron and iron binding capacity.

  • Anti-cardiolipin antibodies/lupus anticoagulant.

  • Antinuclear antigen (ANA) profile (eg, anti-dsDNA, anti-ssDNA).

  • Full procoagulant profile including protein S, protein C, homocysteine levels, antithrombin III, factor V Leiden variant, anti-phospholipid/anti-cardiolipin antibodies, lupus anticoagulant, and platelet aggregation studies (in patients with previous history of thrombosis or MRI evidence of dural venous sinus occlusion).

  • Lyme screening test (enzyme-linked immunosorbent assay [ELISA]) in patients who have a history of exposure to Lyme in areas of endemic disease.

Treatment:

Pseudotumor cerebri treatment typically begins with medications to control the symptoms. Weight loss is recommended for obese individuals. If your vision worsens, surgery to reduce the pressure around your optic nerve or to decrease the intracranial pressure may be necessary. Once you have had pseudotumor cerebri, you should have your vision checked regularly.

Medications:

    Migraine medications. Medications typically prescribed to relieve migraines can sometimes ease the severe headaches that often accompany pseudotumor cerebri.

  • Glaucoma drugs. One of the first drugs usually tried is acetazolamide (Diamox), a glaucoma drug that reduces the production of cerebrospinal fluid by at least 50 percent. Possible side effects include stomach upset, fatigue, tingling of fingers, toes and mouth, and kidney stones.

  • Diuretics. If acetazolamide alone isn't effective, it's sometimes combined with furosemide (Lasix), a potent diuretic that reduces fluid retention by increasing urine output.

Surgeries:

    Optic nerve sheath fenestration. This procedure cuts a window into the membrane that surrounds the optic nerve. This allows excess cerebrospinal fluid to escape. Vision stabilizes or improves in more than 85 percent of cases. Most people who have this procedure done on one eye notice a benefit for both eyes. However, this surgery isn't always successful and may even increase vision problems.

  • Spinal fluid shunt. Another type of surgery inserts a long, thin tube — called a shunt — into your brain or lower spine to help drain away excess cerebrospinal fluid. The tubing is burrowed under your skin to your abdomen, where the shunt discharges the excess fluid. Symptoms improve for more than 80 percent of the people who undergo this procedure. But shunts can become clogged and often require additional surgeries to keep them working properly. Complications can include low-pressure headaches and infections.

NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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