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Pseudotumor cerebri syndrome

Idiopathic intracranial hypertension; Benign intracranial hypertension

Pseudotumor cerebri syndrome 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.

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Central nervous system and peripheral nervous system

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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. Before puberty, it occurs equally in boys and girls.

The cause is unknown.

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

The following factors are also related to this condition:

Symptoms

Symptoms may include any of the following:

Headaches may get worse during physical activity, especially when you tighten the stomach muscles during coughing or straining.

Exams and Tests

The health care provider will perform a physical exam. Signs of this condition include:

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

Tests that may be done include:

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

Treatment

Treatment is aimed at the cause of the pseudotumor. The main goal of treatments is to preserve vision and reduce the severity of headaches.

A lumbar puncture (spinal tap) can help relieve pressure in the brain and prevent vision problems. Repeat lumbar punctures are helpful for pregnant women in order to delay surgery until after delivery.

Other treatments may include:

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 problems such as tumors or hydrocephalus (buildup of fluid inside the skull).

In some cases, the pressure inside the brain remains high for many years. Symptoms can return in some people. A small number of people have symptoms that slowly get worse and lead to blindness.

Outlook (Prognosis)

The condition sometimes 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 provider if you or your child has any of the symptoms listed above.

References

Miller NR. Pseudotumor cerebri. In: Winn HR, ed. Youmans and Winn Neurological Surgery. 7th ed. Philadelphia, PA: Elsevier; 2017:chap 164.

Rosenberg GA. Brain edema and disorders of cerebrospinal fluid circulation. In: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. Bradley's Neurology in Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016:chap 88.

Varma R, Williams SD. Neurology. In: Zitelli BJ, McIntire SC, Nowalk AJ, eds. Zitelli and Davis' Atlas of Pediatric Physical Diagnosis. 7th ed. Philadelphia, PA: Elsevier, 2018:chap 16.

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Review Date: 9/17/2019  

Reviewed By: Alireza Minagar, MD, MBA, Professor, Department of Neurology, LSU Health Sciences Center, Shreveport, LA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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