Children with slowly worsening headaches and vision loss, blurry vision or double vision may be experiencing increased pressure inside the skull. Sometimes there’s no obvious cause. Often, the condition is pseudotumor cerebri.
Pseudotumor cerebri in adults is associated with obesity and is much more frequent in women. In children who haven’t started puberty, pseudotumor cerebri happens equally among boys and girls and is not associated with weight, according to Darren M. Farber, D.O., neurologist with Norton Children’s Hospital and UofL Physicians – Child Neurology.
Primary care physicians may look for signs of optic nerve swelling and refer patients to a neurologist. A neurologist will check the pressure in the skull and order an MRI to rule out a tumor.
As the name suggests, pseudotumor cerebri symptoms mimic those of an actual tumor. Another name for it is idiopathic intracranial hypertension.
Pseudotumor cerebri symptoms
Headaches associated with pseudotumor cerebri start as dull pain and frequently occur at the back of the head or behind the eyes. The headaches worsen with eye movement and tend to be worse first thing in the morning or at night. Concurrent migraines or tension headaches can complicate the diagnosis.
Other symptoms of pseudotumor can include:
- Nausea, vomiting or dizziness
- Flashes of light or auras
- Difficulty seeing to the side
- Blurred or dimmed vision
- Rhythmic thumping or whooshing sound that’s often in time with the heartbeat
- Brief episodes of blindness in one or both eyes
- Neck stiffness or neck, shoulder or back pain
- Forgetfulness and/or depression
- Double vision
Physical activity can make the symptoms worse.
The cause of pseudotumor cerebri typically is unknown. It may stem from a problem absorbing brain and spinal fluid into the bloodstream. Too much of the fluid confined in the skull could cause the pressure.
“Obesity by far and away is the biggest risk factor in older teenagers and adults,” Dr. Farber said. “Obesity is a huge concern in many parts of the South, including Kentucky.”
Teenagers taking the anti-acne medications tetracycline, Accutane and other oral or topical retinoic acid products also are at higher risk, as are children who are on chronic steroids for other conditions. Growth hormone disorders such as acromegaly — enlargement of the face, hands and feet — also may have a pseudotumor connection.
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Other medical conditions associated with pseudotumor include:
- Addison’s disease
- Polycystic ovary
- Behcet’s syndrome
- Blood vessel irregularities
- Sleep apnea
- Underactive parathyroid glands
- Blood-clotting disorders
- Kidney disease
Untreated pseudotumor cerebri possibly can lead to permanent vision loss. Delayed treatment also can lead to permanent optic nerve damage.
Diuretics may be prescribed and can take three to nine months to lower the pressure.
Surgical options include:
- Cutting slits into the membrane surrounding the optic nerve to relieve pressure
- Using shunts to redirect fluid from the brain
- Implanting stents to widen narrowed drainage paths
Even when symptoms resolve, they can recur months or years later, making regular follow-up important. The risk of recurrence is higher in obese patients who do not lose weight following diagnosis, according to Dr. Farber.