What causes pseudotumor cerebri?
The exact cause of PTC is unknown, although it may be associated with an excess of cerebrospinal fluid in the skull. The brain and spinal cord are protected from injuries by cerebrospinal fluid, which is produced and simultaneously absorbed by the blood vessels such that the amount of fluid remains the same.
Sometimes, the body may produce an excessive amount of cerebrospinal fluid, or the brain blood vessels may not reabsorb enough fluid, causing the quantity of fluid to increase. This excess fluid gets collected in the skull and exerts pressure on the brain, which feels like a lesion occupying space.
What are the signs and symptoms of pseudotumor cerebri?
Signs and symptoms of pseudotumor cerebri (PTC) may include:
- Severe headache characterized by dull, pulsating, or throbbing pain that may originate behind the eyes or above the eyes, back of the head, and neck.
- Headaches worsen at night or upon waking up from sleep.
- Other common headaches, such as migraines or tension headaches, may coexist with PTC.
- Any activity that raises abdominal pressure, especially coughing, bearing down, or leaning over, aggravates the headache.
- With the increase in cerebral pressure, the optic nerve enlarges and disrupts information transfer to the brain from the nerve, leading to vision problems. On physical examination, papilledema (swelling of the optic nerve) is found.
- There may be a brief loss of vision in one or both eyes that lasts a few seconds, which may later progress to complete blindness.
- With increased pressure, the sixth cranial nerve (nerves abducens) that controls eyeball movements may not function properly and cause diplopia (double vision).
- Peripheral vision gets blurry or black.
- There may be sudden bursts of light called flashes (a condition called photopsia).
What are risk factors for pseudotumor cerebri?
Risk factors for PTC include:
- Sex (women are 9 times more likely to have PTC than men)
- Medications, such as lithium, tetracycline, and some steroids
- Hypervitaminosis A
- Blood vessel abnormalities, such as narrowing of veins that drain blood and cerebrospinal fluid from the brain, increasing intracranial hypertension
- Sleep apnea
Pressure in the skull may increase with exertion, so symptoms of PTC worsen with exercise or physical activity.
Symptoms may resolve without any intervention, but sometimes may recur after a few months or years.
How is pseudotumor cerebri diagnosed?
The primary goal of treatment is to rule out other health issues, including the presence of an actual tumor. A physical exam and tests can help diagnose pseudotumor cerebri (PTC). Tests include:
- Magnetic resonance imaging (MRI) or computer tomography (CT) of the brain is done to identify tumors and brain shifts.
- MRI scans may reveal tiny ventricles or a flattened pituitary gland (both of which suggest increased pressure in the head), constricted draining veins or indirect indicators of unusually high spinal fluid pressure.
- A CT scan may indicate smaller-than-normal cerebrospinal fluid gaps in the brain (ventricles).
- A lumbar puncture (spinal tap) is performed to confirm the increased pressure (normal is less than 25 cm), and a sample of fluid is collected from around the spine and tested to rule out viral and inflammatory reasons for elevated pressure.
- The fluid drainage can provide rapid but short-lived relief from headaches. However, if other indicators such as increased pressure or eye issues are not present, this test alone cannot confirm the diagnosis of PTC.
- Examination of the eye is done to identify any abnormalities in the movements of the eyeball, vision changes, and possible vision loss.
- An eye exam may identify papilledema (optic nerve swelling) at the rear of the eye because it causes vision loss.
What are treatment options for pseudotumor cerebri?
It is very important to treat PTC as early as possible to prevent any potential vision loss.
Losing weight is one of the most effective methods to reduce discomfort. Salt intake should be limited, but only under medical supervision.
The primary therapies for pseudotumor cerebri are medications and surgery to relieve pressure in the head.
- Steroids to decrease intracranial hypertension
- Glaucoma drugs to decrease the production of cerebrospinal fluid
- Diuretics, such as furosemide, to increase urination and reduce fluid levels in the body
- Migraine medicines to relieve headache
Surgery may be required in severe cases where medicines fail to decrease intracranial pressure:
- Shunting: Surgical procedure to place a long, thin tube called a shunt into the brain or spinal cord to drain excess fluid.
- Optic nerve sheath fenestration: Surgical incision is made in the tissue around the optic nerve to drain excess fluid.
With the removal of excess cerebrospinal fluid, the intracranial pressure decreases and the brain can function normally.
Wall M, Lee AG. Idiopathic Intracranial Hypertension (Pseudotumor Cerebri): Prognosis and Treatment. UpToDate. https://www.uptodate.com/contents/idiopathic-intracranial-hypertension-pseudotumor-cerebri-prognosis-and-treatment#H2194494066
Nationwide Children’s Hospital. Intracranial Hypertension (Pseudotumor Cerebri). https://www.nationwidechildrens.org/conditions/intracranial-hypertension-pseudotumor-cerebri
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