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Pseudotumor cerebri, which literarily means “false brain tumor”, is a potential sight-stealing disorder that occurs when the pressure inside the skull (intracranial pressure) increases for no obvious reason. It's also called idiopathic intracranial hypertension or simply intracranial hypertension. Symptoms of this disorder mimic those of a brain tumor.
What is it?
Pseudotumor cerebri, which literary means “false brain tumor”, is a potential sight-stealing disorder that occurs when the pressure inside the skull (intracranial pressure) increases for no obvious reason. It's also called idiopathic intracranial hypertension or simply intracranial hypertension.
Symptoms of this disorder mimic those of a brain tumor. The increased pressure inside the skull can cause compression of the optic nerve and result in vision loss. Medications often can reduce this pressure and the headache, but in some cases, surgery is necessary.
Pseudotumor cerebri can occur in children and adults, but it's most common in women of childbearing age who are obese.
How do I know I have this disorder?
Some of the indicators that you may have pseudotumor cerebri might include:
Sometimes, symptoms may disappear only to recur months or years later.
What causes it?
The cause is unknown.
When a cause is identified for increase in pressure within the skull, the condition is called secondary intracranial hypertension, rather than idiopathic.
The brain and spinal cord are surrounded by cerebrospinal fluid, a special clear and watery fluid which cushions these vital tissues from injury. This fluid is produced in the brain and eventually absorbed into the bloodstream at a rate that usually allows the pressure in the brain to remain constant.
The increased intracranial pressure of pseudotumor cerebri might result from a problem in the absorption process of the cerebrospinal fluid into the blood stream.
What are the risk factors?
The following factors have been associated with pseudotumor cerebri:
Obesity. Obese women of childbearing age are more likely to develop the disorder.
Medications: some types of substances or medications are linked with secondary intracranial hypertension. Examples include:
Interestingly, in 2002, doctors at the University of Port Harcourt and University Teaching Hospital at Port Harcourt, in Nigeria1 reported the case of a young obese 14-year-old school girl who developed psudotumor cerebri (benign intracranial hypertension) while being treated with an oral antibiotics (perflacine) for the treatment of septic left knee joint infection. She responded satisfactorily to the administration of water losing drug (oral acetazolamide) with complete resolution of her symptoms and signs within 12 days of hospitalization and 2 weeks of follow-up.
Health problems: Conditions and diseases that have been linked to secondary intracranial hypertension include:
For many people with pseudotumor cerebri, the commonest complication of the disorder is worsening of vision loss that may lead to blindness.
How is it diagnosed?
If you suspect you have some or all of the symptoms of pseudotumor cerebri as identified above, it is best to see your doctors in order to obtain a proper diagnosis. The doctor will take a good history, review your symptoms, and conduct a complete or focused physical examination.
Your doctor will also likely order some tests including:
I. Eye exams
If pseudotumor cerebri is suspected, your doctor may send you to another doctor trained in eye conditions (ophthalmologist). The specialist eye doctor will:
(i) Look for distinctive evidence of damage to a structure at the back of your eyes called the optic disc. The disc consist of the optic nerves that carry information from your eyes into the brain. The optic nerves are damaged in pseudotumor cerebri.
(ii) Carry out a visual field confrontation test on you to see if there are abnormal blind spots in your vision besides the normal blind spot in each eye that is associated with the optic disc.
(iii) Likely take photos of your eyes and perform an imaging test to measure the thickness of the layers of your retina (optical coherence tomography).
II. Brain imaging
Your doctor is likely to order an MRI or CT scan. These tests can identify other problems that can cause similar symptoms, such as brain tumors and blood clots.
III. Spinal tap (lumbar puncture)
Your doctor may perform or send you to another specialist doctor for the performance of a lumbar puncture to measure the pressure inside your skull and analyze your spinal fluid. In this test, a specialist inserts a needle between two vertebrae in your lower back and removes a small amount of cerebrospinal fluid for testing in the laboratory.
In view of the strong association of obesity or even moderate amount of weight gain in women with the risk of developing pseudotumor cerebri, losing extra pounds and maintaining a healthy weight might help reduce your chances of developing this potentially sight-stealing disorder.
The goals of treatment for pseudotumor cerebri treatment are:
(i) To relieve you of your symptoms, and
(ii) Keep your eyesight from deteriorating.
If obese, your doctor may recommend that you work with a dietician to plan a low-sodium weight-loss diet to help improve your symptoms. Some people benefit from weight-loss programs or gastric surgery and/or medications.
Possible side effects include stomach upset, fatigue, tingling of fingers, toes and mouth, and kidney stones.
If acetazolamide alone isn't effective, it's sometimes combined with another diuretic, which reduces fluid retention by increasing urine output.
If your vision worsens, surgery to reduce the pressure around your optic nerve or to decrease the intracranial pressure might be necessary.
Vision stabilizes or improves in most cases. However, this surgery isn't always successful and can increase vision problems.
A shunt is generally considered only if other treatments haven't relieved your condition. Shunts can clog and often require other surgeries to keep them working. Complications can include low-pressure headaches and infections.
Regular vision check is required to monitor changes during treatment for pseudotumor cerebri.
Pseudotumor cerebri is a potential sight-stealing disorder that occurs when the pressure inside the skull (intracranial pressure) increases for no obvious reason. Headache, nausea, vomiting and vision problems are common symptoms of the disorder. Obesity in women is a high risk factor. Proper diagnosis of the condition may require a visit with a specialist doctor (neurologist). Losing and maintaining a healthy weight is of importance in the prevention of the disorder. Treatment options include water losing medications and /or surgery.
Created: October 27, 2022
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