Recognizing and Treating Idiopathic Intracranial Hypertension

What is Idiopathic Intracranial Hypertension/Pseudotumor Cerebri?

Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a condition characterized by elevated pressure in the fluid surrounding the brain, spinal cord, and optic nerves. This can cause headaches and vision problems. While the cause(s) of the disease are not fully understood (the definition of "idiopathic"), we know a great deal about the disease itself.

Who Can Get IIH?

Anyone can get IIH, but it's more common in women (90%) than men. While adolescents and young women are most affected, people of all ages can be affected. Furthermore, it is particularly common in people who are overweight, obese, or who have recently gained a significant amount of weight.

What is Idiopathic Intracranial Hypertension?

Idiopathic intracranial hypertension is a condition characterized by increased intracranial pressure in the absence of space-occupying brain masses and apparent causes of CSF blockage. Idiopathic intracranial hypertension presents with symptoms similar to those of a brain tumor (both pathologies involve increased intracranial pressure) and is often associated with obesity. This condition can affect people of all age groups, but it is more common in women aged 20-40, especially if obese.

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What Causes Idiopathic Intracranial Hypertension?

To date, the underlying reasons for idiopathic intracranial hypertension remain partially unclear. Nevertheless, several factors, with obesity at the forefront, are closely linked to the emergence of this disease. In this sense, we can say that the main causes of idiopathic intracranial hypertension are:

• Possible increase in cerebral venous pressure in case of increased endocranial blood volume;
• Alteration of CSF flow due to increased protein content in CSF or obstruction of the cranio-cervical passage;
• Alteration of venous flow due to thrombosis of cerebral veins/sinuses or increased venous pressure in case of arteriovenous malformation;
• Increased venous pressure in case of right heart failure, chronic obstructive bronchopneumopathy;
• Overproduction of CSF;

Risk factors for idiopathic intracranial hypertension are:

• Women of childbearing age.
• Obesity, especially with particularly rapid weight gain;
• Endocrine disorders (thyroid metabolism, hypo- and hypercortisolism, Addison's disease, hypoparathyroidism);
• Hypervitaminosis A, iron deficiency anemia, chronic obstructive bronchopneumopathy;
• Taking medications such as corticosteroids, antibiotics (especially tetracyclines), phenothiazines, lithium;

How to Recognize Idiopathic Intracranial Hypertension?

Symptoms of Idiopathic Intracranial Hypertension

This pseudotumor cerebri often manifests as a headache that persists daily, or occurs frequently and affects both sides of the head. Initially, the headache may be mild, but its intensity varies and may be accompanied by:

• Nausea;
• Double or blurred vision;
• Noise in the head that occurs with each heartbeat (pulsatile tinnitus);
• Some individuals, however, are asymptomatic;

To improve your health, here's some important information: Have you heard of papilledema? This is a condition potentially caused by intracranial hypertension that affects the optic nerve near the eye. Your doctor can easily detect it by examining the back of your eye with an ophthalmoscope.

The first sign of vision problems is loss of peripheral (side) vision. This loss sometimes goes unnoticed at first, and affected individuals may stumble over objects for no apparent reason. In the advanced stage of the disease, vision becomes blurry and blindness can quickly set in. At least one-third of people with pseudotumor cerebri lose their sight, either partially or completely. Vision loss is usually permanent, even if the pressure around the brain is reduced.

Diagnosis of Idiopathic Intracranial Hypertension

In order to diagnose idiopathic intracranial hypertension, a neurological examination is fundamental and may reveal:

• Enlargement of the optic nerves, due to CSF pressure;
• Decreased vision;
• Double vision (diplopia);

In addition to visual symptoms, other main symptoms of idiopathic intracranial hypertension are:

• Headaches;
• Nausea;
• Vomiting;
• Pulsatile tinnitus (sounds perceived in the ears, which are heard at the same rate as the pulse);

During the evaluation, the doctor thoroughly examines the visual field, peripheral vision, and the inside of the eye using an ophthalmoscope.

To ensure an accurate diagnosis of idiopathic intracranial hypertension, a brain MRI is necessary to rule out any other possible cause, as well as a lumbar puncture to measure the pressure inside the skull.

Simply put, MRI provides images of the main veins (called venous sinuses), responsible for transporting blood in the brain. These images allow doctors to detect any possible narrowing, a phenomenon frequent in patients suffering from idiopathic intracranial hypertension.

In cases of idiopathic intracranial hypertension, the increased fluid pressure often reaches extremely high levels. However, a lumbar puncture reduces the pressure inside the head, allowing the sinuses to widen and blood to circulate better in the brain, thus relieving headaches. This process restores balance and alleviates symptoms.

These tests also help rule out other factors that can cause increased pressure inside the skull, such as a brain tumor obstructing the venous sinuses.

Treatment of Idiopathic Intracranial Hypertension

Medical Treatment

Medical treatment of idiopathic intracranial hypertension involves the use of oral diuretics. Short-term treatment with oral or intravenous corticosteroids is sometimes recommended to reduce intracranial pressure.

It is important to stop taking medications known to contribute, such as tetracycline. For headache relief, aspirin, paracetamol, or migraine medications can be helpful.

Topiramate can be beneficial for weight loss and reducing intracranial pressure.

Healthy Lifestyle

Adopting a healthy lifestyle (with a balanced diet and regular physical activity) is often crucial in the fight against obesity. In some cases, this may even be sufficient as the only treatment needed. Indeed, overweight individuals must absolutely lose weight to reduce the pressure inside their skull. A loss of only 10% of their body weight may be enough to resolve idiopathic intracranial hypertension. Unfortunately, the proposed weight loss programs are often ineffective.

Vision Treatment

When idiopathic intracranial hypertension affects your vision, surgery may be crucial to reduce the pressure inside your skull and potentially preserve your visual acuity.

Procedures include:

• Optic nerve sheath fenestration;
• A shunt;
• A stent;

In optic nerve sheath fenestration, incisions are made allowing cerebrospinal fluid to drain from the tissues around the eye, where it is absorbed.

A shunt is a plastic tube used to create permanent drainage. It can be surgically placed to remove cerebrospinal fluid. The stent, on the other hand, is a tube that is placed in a narrowed venous sinus to widen it.

Idiopathic Intracranial Hypertension and Bariatric Surgery

For obese patients unresponsive to other measures, bariatric surgery, such as gastric bypass, can be considered to aid weight loss. If successful, this could resolve idiopathic intracranial hypertension.

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