Pseudotumor cerebri

Children with slowly worsening headaches and vision loss, blurry vision or double vision may have pseudotumor cerebri.

Pseudotumor in adults is associated with obesity and is much more frequent in women. In prepubertal children, pseudotumor 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 University of Louisville Physicians – Child Neurology.

Primary care physicians who suspect pseudotumor should perform a funduscopic exam to look for papilledema. If there’s evidence of optic nerve swelling, the patient should be referred to a neurologist. Pseudotumor is a serious condition.

“The optic nerve is being squeezed,” Dr. Farber said. “If we do not treat that pressure, even if we treat it later on, you can have permanent injury.”

A neurologist will perform a lumbar puncture to check the intracranial pressure and order an MRI to rule out a tumor.

As the name suggests, pseudotumor cerebri symptoms mimic those of an actual tumor. Pseudotumor, also called idiopathic intracranial hypertension, occurs when intracranial pressure increases for no apparent reason.

Symptoms

Headaches associated with pseudotumor frequently occur at the back of the head or behind the eyes and start as a dull pain. 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
  • Photopsia
  • Difficulty seeing to the side
  • Blurred or dimmed vision
  • Pulsatile tinnitus
  • Brief episodes of blindness in one or both eyes
  • Neck stiffness or neck, shoulder or back pain
  • Forgetfulness and/or depression
  • Diplopia

Physical activity can exacerbate the symptoms by increasing intracranial pressure.

The cause of pseudotumor typically is unknown. It may stem from a problem absorbing cerebrospinal fluid into the bloodstream, resulting in an excess of cerebrospinal fluid in the bony confines of the skull.

Risk factors

“Obesity by far and away is the biggest risk factor in older teenagers and adults,” Dr. Farber said. “Obesity is a huge problem 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 are also linked to pseudotumor.

Other medical conditions associated with pseudotumor include:

  • Addison’s disease
  • Lupus
  • Anemia
  • Polycystic ovary
  • Behcet’s syndrome syndrome
  • Blood vessel irregularities
  • Sleep apnea
  • Underactive parathyroid glands
  • Blood-clottingdisorders
  • Uremia
  • Kidney disease

Treatment

Untreated pseudotumor can lead to progressive and possibly permanent vision loss. Delayed treatment also can lead to permanent damage to the optic nerve.

According to Dr. Farber, there are medical and nonmedical treatments. Acetazolamide, sold as Diamox and other brand names, is a diuretic typically used for high blood pressure. It affects cerebrospinal fluid flow dynamics, though it can take three to nine months to lower the intracranial pressure sufficiently.

Surgical options include:

  • Optic nerve sheath fenestration, where slits are cut into the meninges surrounding the optic nerve to relieve intracranial pressure
  • Placing shunts to redirect fluid from the brain
  • Placing stents in narrowed draining venous sinuses

Recurrence

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.

For more information or to refer a patient, call (502) 629-KIDS.


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