What Is Tolosa Hunt Syndrome

Introduction

Tolosa Hunt syndrome derives its name from its discoverers, Tolosa, who in 1954 described the first case of the condition, and, Hunt, who described another six cases of the condition in 1961. Tolosa Hunt syndrome is an uncommon condition and its cause is not completely understood, though it is thought that it does involve abnormalities of structures in the brain, namely, inflammation in a segment of the brain which causes the weakening of its nerves. The condition is characterised by pain when moving the eyes and a one-sided headache around the region of the eye and eyebrow which can last approximately for weeks to a month at a time, with said episodes potentially recurring after several months or years. Whilst the condition cannot be cured, signs and symptoms can eventually resolve by themselves, though this process can be sped up with medication.^1,2,3,4

Causes and triggers of Tolosa Hunt syndrome

The cause of Tolosa Hunt syndrome is not completely understood. Inflammation in part of the brain, which causes weakening of a few of the brain’s nerves, known as cranial nerves, is thought to be involved in the condition.^1,2,3

Triggers of Tolosa Hunt syndrome are also not completely understood, but, research has pointed to several potential triggers of one with Tolosa Hunt syndrome experiencing an episode: ^5,6,7,8,9

• COVID-19 infection and COVID-19 vaccination
• Pregnancy
• Physical trauma
• Tumours
• Aneurysms

Signs and symptoms of Tolosa Hunt syndrome

The characteristic signs and symptoms of Tolosa Hunt syndrome are pain when moving the eyes and a one-sided headache around the region of the eye and eyebrow. However, other signs and symptoms can accompany these:¹

• Double vision
• Long-term fatigue
• Bulging eyes
• Drooping of the upper eyelid
• Worsened vision

Signs and symptoms can last approximately for weeks to a month at a time, with said episodes potentially recurring after several months or years. Signs and symptoms can occur randomly without a specific pattern.²

Diagnosis of Tolosa Hunt syndrome

Diagnostic criteria

The International Headache Society sets out criteria that should be fulfilled to confirm a diagnosis of Tolosa Hunt syndrome:¹

• Eye pain on one side of the head that persists for at least eight weeks if untreated
• Irritation or damage to the third, fourth, or sixth cranial nerves
• Pain relief within 48 hours of steroids being given
• Other conditions that may cause the signs and symptoms of Tolosa Hunt syndrome have been ruled out

Clinical history

The clinical history involves a doctor gathering information about you, your past health history, and your current health. This provides a doctor with an opportunity to understand you more, your condition, and what might have caused your condition. This also provides you with an opportunity to share any of your concerns or ask any questions.¹⁰

Scans

Scans, typically CT scans and MRI scans, may be used to confirm a diagnosis of Tolosa Hunt syndrome as these scans can indicate any inflammation of the brain that may be contributing to the cause of the condition.¹

CT scans are a type of X-ray that takes images of your body. They involve you having to lie back on a bed that passes through the CT scanner. The CT scanner consists of a small ring that the bed slowly passes through. Typically a CT scan will take 10 to 20 minutes to complete.¹¹

MRI scans use magnetic fields and radio waves to take images of your body. Similar to CT scans, you’ll be asked to lie back on a bed that passes through an MRI scanner. However, unlike CT scans, MRI scans are much larger and cover the majority of your body. MRI scans also take longer to complete, lasting approximately 20 to 90 minutes.¹²

Treatment of Tolosa Hunt syndrome

The pain caused by Tolosa Hunt syndrome can resolve without treatment, though this typically takes 15 to 20 days. However, with medication, the time it takes for pain to be alleviated can be vastly reduced, though medication cannot prevent further episodes from occurring. The medication typically used to relieve the pain caused by Tolosa Hunt syndrome is steroids.  They can be taken as a pill or an injection and can relieve pain within 24 to 72 hours.¹ However, steroids can cause several side effects:^{13 14}

• Heartburn
• Difficulty sleeping
• Feelings of anxiety and irritability
• Increased risk of infections
• High blood sugar
• High blood pressure
• Cushing’s syndrome
• Cataracts
• Glaucoma

FAQs

How common is Tolosa Hunt syndrome?

Tolosa Hunt syndrome is an uncommon condition, with it affecting about 1 to 2 people per million people each year.¹⁵ It affects those assigned female at birth and those assigned male at birth equally. The average age of onset is 41 years but cases have been reported in a wide range of ages, including children.¹ 

Can Tolosa Hunt syndrome reoccur after treatment?

Tolosa Hunt syndrome can reoccur after treatment and there is no treatment currently available to prevent episodes from recurring. However, steroids can be used to relieve pain quickly.¹

What triggers Tolosa Hunt syndrome?

What triggers episodes of Tolosa Hunt syndrome is not completely understood. Several potential risk factors have been suggested, and these include COVID-19 infection, COVID-19 vaccination, pregnancy, physical trauma, tumours, and aneurysms. ^5,6,7,8,9

Can Tolosa Hunt syndrome be prevented?

The cause of Tolosa Hunt syndrome is not currently known, and, therefore, ways to prevent the condition have not been established.¹

Does Tolosa Hunt syndrome occur in children or adults?

The average age of onset of Tolosa Hunt syndrome is 41 years but cases have been reported in a wide range of ages, including children.¹ 

Is Tolosa Hunt syndrome a hereditary condition?

The cause of Tolosa Hunt syndrome is not known and the condition has not been determined to be a hereditary condition.¹

Can Tolosa Hunt syndrome be cured?

Tolosa Hunt syndrome cannot be cured. The condition occurs in episodes which cause pain typically lasting several weeks to a month and can resolve by itself in about 15 to 20 days. But with medications, it can resolve in 24 to 72 hours.¹

Are there any long-term complications associated with Tolosa Hunt syndrome?

Tolosa Hunt syndrome is often treated with steroids to relieve the pain caused by the condition. Steroids can cause several long-term complications, including heartburn, difficulty sleeping, feelings of anxiety and irritability, increased risk of infections, high blood sugar, high blood pressure, Cushing’s syndrome, cataracts, and glaucoma.^13,14

Can Tolosa Hunt syndrome lead to permanent vision loss?

Tolosa Hunt syndrome can affect vision, causing it to worsen, and how much vision is worsened can differ depending on the person. Furthermore, this decline in vision can be worsened by steroids which can cause cataracts and glaucoma.^1,13,14

Can any lifestyle changes alleviate Tolosa Hunt syndrome?

The only established treatment for Tolosa Hunt syndrome is medication, mainly steroids for pain relief. Lifestyle changes have not been established as a treatment for the condition.¹

What specialists are involved in treating Tolosa Tunt syndrome?

Many different medical specialists may be involved in the care of patients with Tolosa Hunt syndrome. A general practitioner can coordinate your care, referring you to medical specialists equipped to deal with the specific signs and symptoms of your condition.¹⁶

Summary

Tolosa Hunt syndrome is an uncommon condition, which, though the cause is unknown, is thought to be related to inflammation of parts of the brain which disrupts the nerves. The condition is characterised by pain when moving the eyes and a one-sided headache around the region of the eye and eyebrow. These symptoms occur in episodes typically lasting several weeks to a month and may be separated by periods of no symptoms that can last several months to years. Diagnosing this condition involves ruling out other conditions with similar signs and symptoms, typically done through a clinical history and scans. This is not a curable condition, but episodes can resolve themselves and recovery can be achieved more quickly with medications, which are mostly steroids.