Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)

Pseudotumor Cerebri is a rare disease that affects only a few individuals across the world every year. However, it may lead to permanent vision loss if not treated in time. Read and know all about the causes, symptoms, diagnosis, treatment and prognosis of this condition.

What is Pseudotumor Cerebri Syndrome?

It is a condition that affects the brain. The name of this disease literally means “false tumor” as it appears as a tumor and its symptoms are also the same as a brain tumor. However, it is actually a tumor of the brain which is a far more damaging syndrome and even leads to the death of sufferers.

The condition primarily affects women of childbearing age who are predominantly obese and also women who are passing through menopause. It affects women more than men and is rare in infants. However, Pseudotumor Cerebri in children is more frequent.

This disease is also known by other names like Benign Intracranial Hypertension and Idiopathic Intracranial Hypertension.

Pseudotumor Cerebri Causes

This condition can arise due to pregnancy, onset of menstruation and obesity. It may also occur as a result of conditions like :

• Iron deficiency anemia
• Cushing’s disease
• Addison’s disease
• Hypoparathyroidism
• Chronic kidney failure

It may also develop as a side effect of intake of medicines like :

• Cyclosporine
• Isotretinoin
• Minocycline
• Birth control pills
• Nitrofurantoin
• Tamoxifen
• Tetracycline
• Nalidixic acid
• Sulfa drugs
• Phenytoin

Consumption of Vitamin A capsules and starting or stopping of steroid medications may also lead to this disease.

Pseudotumor Cerebri Symptoms

The symptoms of Pseudotumor Cerebri involve physical discomforts like :

• Dizziness
• Blurred vision
• Vision loss
• Loss of hearing
• Attacks of migraine without any apparent triggers
• Nausea
• Severe headaches, not relieved by medications
• Pulsating intracranial sounds
• Neck pain
• Shoulder pain
• Buzzing sound in the ears (tinnitus)
• Double vision (diplopia)

Physical activity may increase the intensity of these symptoms. This mainly occurs when a person tightens the stomach muscles.

Pseudotumor Cerebri Diagnosis

Diagnosis is usually carried out to detect presence of physical symptoms like :

• Increase in size of head
• Inflammation of the optic nerve located in the posterior position of the eye (Papilledema)
• Protruding anterior fontanelle

However, there is no change in the level of alertness even though there is increase in pressure in the skull. However, conditions like Venous sinus thrombosis, Tumor and Hydrocephalus may elevate pressure in the skull. These diagnostic tests are carried out when other health disorders are ruled out.

Medical tests performed for diagnosing this condition involve Visual Field Testing and other exams for the eye, CT scan of the head, Lumbar puncture (spinal tap) and MRI of the head by using MR Venography.

After normal neuroimaging is performed, a lumbar puncture (LP) is required to eliminate presence of meningitis. The CSF opening pressure needs to be documented. For demonstrating the diagnosis of this condition, the opening pressure of CSF (Cerebrospinal Fluid) should be higher than 250 mm H20, which is the topmost limit of normal pressure for most non-obese and obese adults (75). The glucose and cell count should be usual and the level of protein should be low or normal.

Pseudotumor Cerebri Treatment

Treatment for Pseudotumor Cerebri involves a number of procedures. These include :

Medications

Drugs such as furosemide, acetazolamide and corticosteroids are commonly used by healthcare providers to give patients fast relief from this condition.

Shunting

In patients of Pseudotumor Cerebri Shunting is used to provide patients with relief from pressure due to accumulation of fluids in the spine.

Surgery

In extreme cases, surgical operation is also required to ease pressure over the optic nerve of the eye.

Fluid restriction

Intake of salts and fluids may be restricted in sufferers to prevent aggravation of the disease and future complications.

Exercise

Patients of this disease should exercise regularly to keep their weight in control. As aforesaid, excessive body weight can make obese people highly susceptible to this disorder.

Lumbar Puncture

In some cases, it becomes necessary to carry out a Lumbar Puncture in sufferers. The process helps ease pressure build-up inside the brain and prevents problem in vision. In Pseudotumor Cerebri Lumbar Puncture (Spinal Tap) is also the only dependable diagnostic method. The process involves passing a needle between the lower back bones to reach the cerebrospinal fluid that lies around the spinal nerves. It helps in measuring the pressure of this fluid.

Shunt For Pseudotumor Cerebri

Pseudotumor Cerebri Shunt involves surgical insertion of a draining tube within the spinal fluid area in the lower spine in the cavity of the abdomen. This is usually performed to drain excess fluid and ease pressure.

Pseudotumor Cerebri Management

Management of this condition involves dietary modifications. Salts and fluids may need to be restricted, but as per the specifications laid down by the doctor. The vision of patients needs to be closely monitored. In acute cases, loss of vision may become permanent in the absence of proper monitoring. CT scans and MRI scans may be required as follow-up examinations. In Pseudotumor Cerebri MRI findings may help rule out underlying cancerous conditions.

Pseudotumor Cerebri Complications

Vision loss is the main complication of this disorder. It may also lead to Papilledema (a condition leading to an inflammation of the optic disc that arises from a rise in intraocular pressure) which may be refuse to go away despite treatment.

Pseudotumor Cerebri Prognosis

The condition may naturally go away within six months. Approximately 10 to 20% of affected individuals may suffer from a recurrence of symptoms. In rare cases, the symptoms slowly worsen leading to complete blindness of the patient.

Minocycline and Pseudotumor Cerebri

Minocycline medicines are frequently used to cure acne problems. Use of this medicine can lead to Pseudotumor Cerebri in some individuals, often after 2 to 3 weeks of use. This can also lead to further complications like abduction deficit in the right eye and Bilateral Papilledema. Treatment may require stopping of this medicine. Doctors may refer an alternate drug to such patients.

Is Pseudotumor Cerebri Hereditary?

This condition is often regarded to be a hereditary one even though it is actually not so. It is also a non-contagious disease.

You should immediately get in touch with a doctor if you or anyone in your family is experiencing symptoms of this disease. Diagnosing and curing this condition earlier will allow an early recovery for a sufferer and keep his or her vision intact.

References:

http://www.nlm.nih.gov/medlineplus/ency/article/000351.htm

http://emedicine.medscape.com/article/1143167-overview

http://www.medicinenet.com/pseudotumor_cerebri/article.htm

http://www.mayoclinic.com/health/pseudotumor-cerebri/DS00851