What is pseudotumor cerebri syndrome (PTCS)?

Pseudotumor cerebri syndrome (PTCS) is a condition caused by an abnormal increase of cerebrospinal fluid pressure inside the skull, without presence of a brain tumor. This pressure causes symptoms such as headaches, nausea, double vision and vision loss.

Pseudotumor cerebri literally means “false brain tumor” because the signs and symptoms in affected patients are similar to those of a large brain tumor. The cause, however, is an abnormal increase of cerebrospinal fluid pressure inside the skull without a brain tumor.

The body produces cerebrospinal fluid, which surrounds the brain and spinal cord. In people with PTCS, for unclear reasons, the cerebrospinal fluid pressure becomes abnormally high around the brain.

PTCS can occur in children and adults but is most common in overweight and obese women of child-bearing age. When no cause can be found, the condition is labeled primary PTCS — also known as idiopathic intracranial hypertension (IIH).

Cause of PTCS

While the cause is sometimes unknown, in many instances PTCS can be a symptom of an underlying hormone problem or undiagnosed medical condition. PTCS has been linked with certain factors, including:

• Obesity or recent substantial weight gain
• Anemia
• Polycystic ovary syndrome
• Renal failure

In addition, the following medications may play a role in the development of PTCS:

• Growth hormone
• Tetracycline antibiotics (e.g., minocycline)
• Excess vitamin A
• Withdrawal from chronic corticosteroids

Signs and symptoms of PTCS in children

Common symptoms of pseudotumor cerebri syndrome include headaches, nausea, double vision and vision loss. Your child may also hear ringing in the ears that pulses in time with his or her heartbeat. Some children may experience neck, shoulder and back pain.

Some children have no symptoms, yet findings suggestive of PTCS are discovered during a routine eye examination.

Testing and diagnosis of PTCS

Because PTCS often affects vision, an ophthalmologist or neuro-ophthalmologist will examine your child’s optic nerves for signs of swelling and check for blind spots in your child’s field of vision.

Your child’s doctor will also order an MRI or CT scan to exclude other possible causes, and a lumbar puncture to measure the cerebrospinal fluid pressure in your child’s skull and to make sure there is no infection.

An evaluation with an endocrinologist can also diagnose any underlying hormonal or medical problems that may be causing PTCS.

Treatment for PTCS

The optimal treatment of children with pseudotumor cerebri syndrome requires a multidisciplinary approach that addresses its causes as well as its symptoms. At CHOP’s Pediatric Pseudotumor Cerebri Collaborative Clinic, children with PTCS are seen by a team that includes a neuro-ophthalmologist, endocrinologist, and in many instances, a dietitian. Our team will work closely with you to evaluate, treat, and manage your child’s PTCS.

Since permanent vision loss is the most concerning potential complication of PTCS, a neuro-ophthalmologist will monitor your child’s optic disc swelling and vision over time and will manage your child’s PTCS medications.

Your child also will be cared for by an endocrinologist who will determine the best treatment approach for any hormonal or medical causes of the PTCS. If your child’s weight is believed to be causing the PTCS, our dietitian will work with your family to help your child achieve a healthy weight.

Most patients will be treated with an oral medical medication called “acetazolamide,” which decreases the production of cerebrospinal fluid to lower the cerebrospinal fluid pressure inside the skull. When vision loss is severe or gets worse despite acetazolamide treatment, surgeries such as optic nerve sheath fenestration or ventriculoperitoneal shunting may be recommended.

Long-term outlook for PTCS

In most instances, acetazolamide treatment for six to nine months, along with weight loss if PTCS occurs with obesity, are sufficient for a full recovery, including resolution of the elevated cerebrospinal fluid pressure inside the skull, headaches, optic disc swelling, and vision loss.

Patients with PTCS generally are seen every two to three months until the condition resolves, and long-term follow-up appointments and repeat lumbar punctures after this are not necessary. For those patients who lose weight as part of their treatment, maintaining a healthy weight in general prevents the disorder from recurring.

Why choose CHOP for PTCS treatment

At CHOP’s Pediatric Pseudotumor Cerebri Collaborative Clinic, your child is cared for by an internationally-recognized multidisciplinary team comprised of pediatric neuro-ophthalmologists, an endocrinologist, and a dietitian, all with special expertise in treating children with PTCS. We meet regularly to review our patients’ cases and collaborate in medical decision-making.

Our team is also actively engaged in research projects with the goal of improving our understanding of the causes of PTCS and thereby optimizing our management of affected patients.

Our team members are leaders in the field and are frequently invited speakers at national and international medical conferences. Dr. Liu, for instance, was one of the co-authors of the diagnostic criteria for PTCS (Friedman et al. 2013).

Support PTCS research

We are constantly searching for ways to advance the science of PTCS thanks to our grateful families who support our research. If you are interested in supporting our clinic or research, please feel free to email us.