Pseudotumor cerebri

Answer 1

Definition

Pseudotumor cerebri is a process affecting the brain that appears to be -- but is not -- a tumor. It is often reversible.

Alternative Names

Idiopathic intracranial hypertension; Benign intracranial hypertension

Causes, incidence, and risk factors

The condition occurs more frequently in women than men, particularly in premenopausal obese women. It is rare in infants.

The cause is unknown. Certain medicines can increase your risk for this condition. These medicines include:

• Birth Control pills
• Tetracycline
• Nitrofurantoin
• Phenytoin
• Sulfa drugs
• Isotretinoin
• Minocycline
• Tamoxifen
• Nalidixic acid
• Lithium
• Steroids (starting or stopping them)

The following factors also associated with this condition:

• Anemia from iron deficiency
• Being overweight or obese
• Chronic kidney failure
• Cushing's disease
• Hypoparathyroidism
• Hypothyroidism
• Onset of menstruation (menarche)
• Pregnancy
• Vitamin A (too much or too little)

Symptoms

The major symptom is increased pressure within the skull (increased intracranial pressure). There is no evidence of tumor, infection, blocked drainage of the fluid surrounding the brain, or any other cause.

Symptoms include:

• Headache
• Buzzing sound in the ears (tinnitus)
• Dizziness
• Nausea
• Double vision(diplopia)
• Partial or permanent loss of vision. Patients often complain of blurred vision.

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

Signs and tests

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

• Swelling of a small area in the back of the eye (papilledema)
• Increased head size
• Bulging anterior fontanelle in babies whose cranial sutures are not yet closed

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

Tests that may be done include:

• CT Scan
• MRI
• Lumbar puncture (spinal tap)
• Eye exam, including formal visual field testing

The diagnosis is made when other health conditions are ruled out. Several conditions may cause increased intracranial pressure, including venous sinus thrombosis, hydrocephalus, and an intracranial mass (such as a tumor).

Treatment

Treatment must be directed at the specific cause of the pseudotumor.

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

Other treatments may include:

• Fluid or salt restrictions
• Medications such as corticosteroids, glycerol, acetazolamide, and furosemide
• Shunting procedures to relieve pressure due to spinal fluid retention
• Surgery to relieve pressure on the optic nerve
• Weight loss

The patient will need their vision closely monitored, since there is potential for progressive and sometimes permanent visual loss. Follow-up MRI or CT scans may be done to rule out hidden cancer.

Expectations (prognosis)

Sometimes the condition disappears on its own within 6 months. About 10-20% of persons have their symptoms return. A small number of patients have symptoms that slowly get worse and lead to blindness.

Complications

Vision loss is a serious complication of this condition.

Calling your health care provider

Call your health care provider if you or your child experience the symptoms listed above.

References

Jonnalagadda J. Lithium, minocycline, and pseudotumor cerebri. J Am Acad Child Adolesc Psychiatry. March 1, 2005; 44(3): 209.

Behrman RE. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, Pa: WB Saunders; 2004; 2048-2049.

Answer 2

Definition

Pseudotumor cerebri is a process affecting the brain that appears to be -- but is not -- a tumor. It is often reversible.

Alternative Names

Idiopathic intracranial hypertension; Benign intracranial hypertension

Causes, incidence, and risk factors

The condition occurs more often in women than men, especially in obese women who are about to go through menopause. It is rare in infants, but can occur in children.

The cause is unknown.

Certain medicines can increase your risk for 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's disease
• Chronic kidney failure
• Cushing's disease
• Hypoparathyroidism
• Iron deficiency anemia
• Obesity
• Onset of menstruation (menarche)
• Pregnancy

Symptoms

Symptoms include:

• 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.

Signs and tests

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

• Bulging anterior fontanelle
• 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, incluiding visual field testing
• MRI of the headwith MR venography
• Lumbar puncture(spinal tap)

The diagnosis is made when other health conditions are ruled out. Several conditions may cause increased pressure in the skull, including:

• 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
• Medications 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

Patients 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.

Expectations (prognosis)

Sometimes the condition disappears on its own within 6 months. About 10-20% of persons have their symptoms return. A small number of patients have symptoms that slowly get worse and lead to blindness.

Complications

Vision loss is a serious complication of this condition.

Calling your health care provider

Call your health care provider if you or your child experiences the symptoms listed above.

References

DeAngelis LM. Tumors of the central nervous system and intracranial hypertension and hypotension. In: Goldman L, Ausiello D, eds. Cecil Textbook of Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 199.

Haslam RHA. Pseudotumor cerebri. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics.18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 604.

Rosenberg GA. Brain edema and disorders of cerebrospinal fluid circulation. In: Bradley WG, Daroff RB, Fenichel GM, Jankovic J, eds. Bradley: Neurology in Clinical Practice. 5th ed. Philadelphia, Pa: Butterworth-Heinemann Elsevier; 2008:chap 63.

Dhungana S, Sharrack B, Woodroofe N. Idiopathic intracranial hypertension.Acta Neurol Scand. 2010;121(2):71-82. Epub 2009 Nov 23.

Reviewed By

Review Date: 02/05/2011

David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Also reviewed by Joseph V. Campellone, MD, Division of Neurology, Cooper University Hospital, Camden, NJ. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.