Tolosa-Hunt Syndrome

What is Tolosa-Hunt syndrome?

Tolosa-Hunt syndrome is a rare condition that causes sudden, severe headaches and painful eye movements and/or a limited ability to move your eyes. Symptoms can come and go throughout your life (relapse) and usually start around age 40.

These symptoms can feel like having uninvited guests stop by your house. They interrupt you and stop you from doing what you planned on doing that day. Luckily, the guests don’t stay too long.

Research suggests it could be the result of inflammation (an immune system response) behind your eye.

You may hear your healthcare provider refer to Tolosa-Hunt syndrome as:

• Ophthalmoplegia.
• Recurrent ophthalmoplegia.
• Ophthalmoplegia syndrome.

How common is Tolosa-Hunt syndrome?

Tolosa-Hunt syndrome is rare. Studies estimate it affects 1 person per million throughout the world.

What are the symptoms of Tolosa-Hunt syndrome?

Symptoms of Tolosa-Hunt syndrome happen suddenly and include:

• A headache, with pain near your upper face and eyes (periorbital headache). This pain can be sharp or dull.
• Seeing two of something you look at (double vision).
• Weakness or not being able to move the muscles around your eyes and face.
• Feeling tired all the time (chronic fatigue).

In addition, you might also experience:

• Bulging eyes (proptosis).
• Droopy upper eyelids (ptosis).
• Low vision.

Symptoms usually affect one side of your face. They may come and go unexpectedly (spontaneous remission).

What causes Tolosa-Hunt syndrome?

We don’t know the exact cause of Tolosa-Hunt syndrome. Research suggests it could be the result of:

• An abnormal autoimmune response. Your immune system mistakenly attacks healthy tissue, specifically the cavernous sinus and orbital fissure, behind your eyes.
• Inflamed or narrow blood vessels. These block or slow blood from flowing to certain parts of your brain.

What are the risk factors for Tolosa-Hunt syndrome?

If you experience any of the following triggers (factors that can lead to symptoms), you may be more at risk of developing Tolosa-Hunt syndrome:

• A viral infection.
• A traumatic brain injury.
• A tumor.
• An aneurysm.

What are the complications of Tolosa-Hunt syndrome?

Severe cases of Tolosa-Hunt syndrome may affect your vision. This can happen if inflammation behind your eye (cavernous sinus and orbital fissure) extends into the back of your eye (optic nerve).

How is Tolosa-Hunt syndrome diagnosed?

A healthcare provider will diagnose Tolosa-Hunt syndrome after a physical exam to review your symptoms and take a complete medical history. Blood tests and radiology imaging tests can confirm a diagnosis and rule out possible conditions with similar symptoms. Imaging tests for Tolosa-Hunt syndrome include a:

• Computed tomography (CT) scan: A series of X-rays and a computer produce a 3D image of soft tissues and bones in your body.
• Magnetic resonance imaging (MRI) test: A large magnet, radio waves and a computer take pictures of the inside of your body.

A healthcare provider specifically looks for certain characteristics, like inflammation behind your eye, from imaging tests to make this diagnosis.

When does a Tolosa-Hunt syndrome diagnosis occur?

Tolosa-Hunt syndrome can affect anyone at any age, but a diagnosis is most common around age 40.

How do you treat Tolosa-Hunt syndrome?

Short-term steroid medications, like corticosteroids, treat Tolosa-Hunt syndrome symptoms. This medication reduces inflammation. Inflammation behind your eyes is what causes your symptoms.

Are there side effects of the treatment?

Talk to your healthcare provider about the possible side effects of steroids before starting a medication. Possible side effects of corticosteroids include:

• Swelling in your legs.
• Increased appetite.
• Mood changes.
• Bruising easily.
• Blurry vision.

How soon after treatment will I feel better?

After taking prescribed steroids, you may feel better in one to three days. Without treatment, your symptoms may go away on their own within 15 to 20 days. Even after your symptoms go away, they may come back unexpectedly.

Can Tolosa-Hunt syndrome be prevented?

There’s no known way to prevent Tolosa-Hunt syndrome.

Is Tolosa-Hunt syndrome serious?

Tolosa-Hunt syndrome isn’t a life-threatening condition. Symptoms can affect your ability to go about your daily routine normally due to pain and discomfort. Your prognosis (outlook) is very good with treatment. Steroids effectively reduce your symptoms within 24 to 72 hours after they appear, so you can feel better sooner.

Is Tolosa-Hunt syndrome curable?

There’s no known cure available for Tolosa-Hunt syndrome.

When should I see a healthcare provider?

Visit your healthcare provider if you experience symptoms of Tolosa-Hunt syndrome. They’ll treat your symptoms with short-term steroid medications that work quickly so the symptoms you experience go away.

What questions should I ask my healthcare provider?

• Will Tolosa-Hunt syndrome affect my vision?
• Do I need to take steroids to treat it?
• How and how often should I take steroid medications?
• What are the side effects of treatment?

A note from Cleveland Clinic

Tolosa-Hunt syndrome can stop you in your tracks due to sudden, uncomfortable symptoms. You may have severe headaches that make it difficult to go about your day normally. It can also affect your vision. These symptoms come and go throughout your life unexpectedly. Short-term use of steroid medications is effective to reduce your symptoms within a day to three days. Treatment quickly helps you get back to the things you enjoy with limited interruption.