Pseudotumor Cerebri

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What is pseudotumor cerebri 

Pseudotumor cerebri is a Latin term that means “false brain tumor” in which cerebrospinal fluid (CSF) that surrounds the brain and spinal cord builds up inside the skull and can cause a severe headache, nausea and vomiting, ringing in the ears, and problems with vision — symptoms that mimic those of a brain tumor. It is a condition now more commonly known as idiopathic intracranial hypertension (IIH), which literally means high pressure (hypertension) in the skull (intracranial) due to an unknown reason (idiopathic).

Symptoms of Pseudotumor Cerebri

Symptoms of pseudotumor cerebri develop gradually and are caused by elevated intracranial pressure. The most common symptoms include:

• Daily or near-daily headache, often behind the eyes, worsening when lying down or first thing in the morning
• Blurred or double vision, sometimes progressing to complete vision loss if untreated
• Transient visual obscurations: brief episodes (seconds to minutes) of vision darkening in 1 or both eyes
• Pulsatile tinnitus: a rhythmic whooshing or pounding sound in the ears that synchronizes with heartbeat
• Neck and back pain, sometimes worsening with movement
• Nausea or vomiting
• Papilledema: swelling of the optic nerve at the back of the eye, visible only during an eye exam with dilated pupils

Not every patient has all of these symptoms, and severity varies. Some patients have only a headache. Others have primarily vision problems. What unites them is the underlying pressure increase, which is what distinguishes pseudotumor cerebri from other headache disorders.

When to See a Specialist

You should seek a consultation with a neurovascular specialist if you have:

• New or worsening headache paired with vision changes
• Symptoms of elevated intracranial pressure noted on an eye exam (papilledema)
• Pulsatile tinnitus accompanied by headache or visual changes
• A diagnosis of pseudotumor cerebri that has not improved with initial treatment
• Symptoms that have worsened or returned after initial improvement

Causes and Risk Factors

Pseudotumor cerebri is called idiopathic because in most cases no single cause can be identified. However, certain conditions and medications increase risk substantially. Understanding these factors helps guide treatment decisions.

Key Risk Factors

• Obesity or rapid weight gain: The strongest preventable risk factor. Obese women are particularly at risk.
• Female sex and reproductive age: Women of childbearing age are 8 to 9 times more likely to develop pseudotumor cerebri than men.
• Certain medications: Tetracyclines (antibiotics), vitamin A derivatives (isotretinoin, tretinoin), corticosteroid use or withdrawal, hormonal birth control, and lithium.
• Polycystic ovary syndrome (PCOS): Associated with obesity and hormonal changes, increases risk in women.
• Sleep apnea: Oxygen disruption and increased nighttime intracranial pressure contribute to risk.
• Menstrual irregularities or thyroid disease: Hormonal factors play a role in some patients.

The exact mechanism by which these factors lead to elevated intracranial pressure is not fully understood. What is clear is that weight loss, if applicable, and discontinuation of offending medications can lead to symptom improvement or complete resolution in many patients.

What causes pseudotumor cerebri? How is it Diagnosed?

The build-up of CSF fluid and resulting pressure can happen suddenly because of a head injury or stroke, which is called acute, or it can also develop over time, which experts refer to as chronic. The exact cause for why IIH occurs is unknown; however, factors such as obesity; certain medications like birth control pills, Vitamin A supplements, and steroids; and other health conditions like chronic kidney disease, metabolic conditions, and sleep apnea have been linked to its development.

Pseudotumor cerebri can also affect anyone at any age, but it is more commonly diagnosed in women between the ages of 20 and 50 years. 

While not life threatening, the condition can be serious. The pressure that builds up in the skull can cause swelling of the optic nerve — the bundle of nerve fibers that transmit visual information between the eye and the brain. If the swelling isn’t treated, it can lead to nerve damage and permanent vision loss; therefore, timely diagnosis and treatment is critical.

Can I prevent pseudotumor cerebri?

Since the cause of pseudotumor cerebri is unknown, it’s not fully preventable. However, maintaining a healthy lifestyle, getting enough sleep, and managing health conditions can play a role in lowering the chances of developing the condition. 

What are the treatment options?

Because pseudotumor cerebri can severely impact vision if left untreated, it’s critical to work with specialists like those at NJBS who can ensure proper management of the condition. 

The primary goals of treatment are to reduce pressure within the skull, minimize headaches, and prevent permanent vision loss. This may require a combination of treatments that oftentimes begins with lifestyle changes and medications. 

Weight loss has been established as a first-line treatment if the underlying cause is not attributed to a traumatic injury. Research has shown that women with obesity who followed a low-calorie diet for 3 months lost an average of 30 pounds, which was associated with a decrease in intracranial pressure and fewer headaches and visual problems as reported by the patients in the study.

Medications may be prescribed at the same time to lower the pressure inside the skull. These may include the glaucoma medications, diuretics, migraine drugs, and steroids that work to reduce fluid in the skull, ease headaches, and prevent visual loss.  

Many patients respond to weight loss, medication management, and lifestyle changes. However, if there is rapid or severe visual loss, this may require more aggressive treatment or surgery to more quickly relieve the pressure around the optic nerve. 

Possible procedures may include:

• Shunting can be used to drain excess cerebrospinal fluid, reducing pressure on the brain. It can be done in several ways: a thin tube is placed into the ventricles of the brain (ventriculoperitoneal, or VP shunt) or the lower spine (lumboperitoneal, or LP shunt) to redirect fluid away from the brain. 
• Venous sinus stent is a procedure in which a stent is placed in a larger vein in the head to widen the area, improve drainage, and reduce pressure.
• Optic Nerve Sheath Fenestration is a procedure that relieves pressure around the optic nerve.
  
  

Can pseudotumor cerebri be cured?

Pseudotumor cerebri is considered a chronic condition. Most people improve with treatment, but some can have symptoms that worsen over time and lead to blindness. it is also important to understand that the condition can occur again after treatment. 

Regular follow-up appointments with a neurologist are important to detect or manage symptoms to prevent further or future vision loss.

NJBS is ranked among the top 3 neurosurgery practices in the country. Our subspecialty expertise allows us to provide highly focused compassionate care for patients with chronic conditions like pseudotumor cerebri. Contact us for more information and to learn how we can partner with you to prevent long-term complications.

Why Choose NJBS?

Pseudotumor cerebri requires expertise in neurovascular imaging, intracranial pressure management, and both minimally invasive and surgical options. NJBS is uniquely positioned to provide comprehensive care.

The neurovascular team at NJBS includes fellowship-trained specialists with expertise in intracranial hypertension, venous stenosis, and minimally invasive endovascular procedures. 

“We see many patients who have suffered for months with pseudotumor cerebri before reaching a diagnosis. Once we confirm elevated intracranial pressure, we work closely with the patient to establish a treatment plan. For many, medical management combined with lifestyle change is effective. For those who do not respond, we have the full range of minimally invasive and surgical tools.”

Frequently Asked Questions (FAQs) About Pseudotumor Cerebri (IIH)

Source: NIH / StatPearls