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Pseudotumor cerebri

Idiopathic intracranial hypertension; Benign intracranial hypertension

 

Pseudotumor cerebri is a condition in which the pressure inside the skull is increased. The brain is affected in a way that the condition appears to be, but is not, a tumor.

Causes

 

The condition occurs more often in women than men, especially in young obese women 20 to 40 years old. It is rare in infants, but can occur in children.

The cause is unknown.

Certain medicines can increase the risk of this condition. These medicines include:

  • Birth control pills
  • Cyclosporine
  • Isotretinoin
  • Minocycline
  • Nalidixic acid
  • Nitrofurantoin
  • Phenytoin
  • Steroids (starting or stopping them)
  • Sulfa drugs
  • Tamoxifen
  • Tetracycline
  • Vitamin A

The following factors are also related to this condition:

  • Addison disease
  • Chronic kidney failure
  • Cushing disease
  • Hypoparathyroidism
  • Iron deficiency anemia
  • Obesity
  • Onset of menstruation
  • Pregnancy

 

Symptoms

 

Symptoms may include any of the following:

  • Headache
  • Blurred vision
  • Buzzing sound in the ears (tinnitus)
  • Dizziness
  • Double vision (diplopia)
  • Nausea
  • Vision loss

Symptoms may get worse during physical activity, especially when you tighten the stomach muscles.

 

Exams and Tests

 

The doctor will perform a physical exam. Signs of this condition include:

  • Bulging anterior fontanelle in infants
  • Increased head size
  • Swelling of the optic nerve in the back of the eye (papilledema)

Even though there is increased pressure in the skull, there is no change in alertness.

Tests that may be done include:

  • CT scan of the head
  • Eye exam, including visual field testing
  • MRI of the head with MR venography
  • Lumbar puncture (spinal tap)

Diagnosis is made when other health conditions are ruled out. These include conditions that may cause increased pressure in the skull, such as:

  • Hydrocephalus
  • Tumor
  • Venous sinus thrombosis

 

Treatment

 

Treatment is aimed at the cause of the pseudotumor.

A lumbar puncture can help relieve pressure in the brain and prevent vision problems.

Other treatments may include:

  • Fluid or salt restriction
  • Medicines such as corticosteroids, acetazolamide, and furosemide
  • Shunting procedures to relieve pressure from spinal fluid buildup
  • Surgery to relieve pressure on the optic nerve
  • Weight loss

People will need to have their vision closely monitored. There can be vision loss, which is sometimes permanent. Follow-up MRI or CT scans may be done to rule out hidden cancer.

 

Outlook (prognosis)

 

Sometimes the condition disappears on its own within 6 months. Symptoms can return in some people. A small number of people have symptoms that slowly get worse and lead to blindness.

 

Possible Complications

 

Vision loss is a serious complication of this condition.

 

When to Contact a Medical Professional

 

Call your health care provider if you or your child has any of the symptoms listed above.

 

 

Open References

References

Ferri FF. Idiopathic intracranial hypertension. In: Ferri FF. Ferri's Clinical Advisor 2015 . Philadelphia, PA: Elsevier Mosby; 2015:640-641.

Pless ML. Pseudotumor cerebri. In: Kliegman RM, Stanton BF, St. Geme JW III, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 597.

Rosenberg GA. Brain edema and disorders of cerebrospinal fluid circulation. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC, eds. Bradley's Neurology in Clinical Practice. 6th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 59.

 
  • Central nervous system

    Central nervous system - illustration

    The central nervous system is comprised of the brain and spinal cord. The peripheral nervous system includes all peripheral nerves.

    Central nervous system

    illustration

    • Central nervous system

      Central nervous system - illustration

      The central nervous system is comprised of the brain and spinal cord. The peripheral nervous system includes all peripheral nerves.

      Central nervous system

      illustration

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        Review Date: 2/3/2015

        Reviewed By: Amit M. Shelat, DO, FACP, Attending Neurologist and Assistant Professor of Clinical Neurology, SUNY Stony Brook, School of Medicine, Stony Brook, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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