What are the other Names for this Condition? (Also known as/Synonyms)

• Cavernous Sinus Granulomatosis
• Superior Orbital Fissure Syndrome
• Tolosa-Hunt Ophthalmoplegia

What is Tolosa-Hunt Syndrome? (Definition/Background Information)

• Tolosa-Hunt Syndrome (THS) is a rare eye disorder with signs and symptoms such as headaches, eye pain, decreased eye movement, and blurred vision. Individuals are usually affected around middle-age, although the condition may appear at any stage in life
• The cause of Tolosa-Hunt Syndrome is currently unknown. Research experts believe that having a recent viral infection may increase the likelihood of developing the condition
• A healthcare provider can diagnose Tolosa-Hunt Syndrome by excluding other similar conditions and through specific guidelines that have been outlined for the condition. These include eye pain on one side of the head that persists for at least 8 weeks, if untreated, and associated irritation or damage to the 3rd, 4th, or 6th cranial nerves among others
• Treatment of Tolosa-Hunt Syndrome involves the use of oral corticosteroids. Individuals usually have a very good prognosis with the condition, because steroids have been proven to be an effective treatment measure
• Nevertheless, complications, such as the damage and abnormal repair of the cranial nerves, can worsen the prognosis. Also, currently, no prevention methods for Tolosa-Hunt Syndrome have been identified

Who gets Tolosa-Hunt Syndrome? (Age and Sex Distribution)

• Tolosa-Hunt Syndrome is usually observed in individuals around the age of 40 years
• There have been reported cases of individuals under the age of 30 years being affected; children have been diagnosed with THS, but it is a rare occurrence
• Males and females of different racial and ethnic backgrounds can be affected

What are the Risk Factors for Tolosa-Hunt Syndrome? (Predisposing Factors)

• The risk factors for Tolosa-Hunt Syndrome are not well-characterized. However, having had a recent viral infection may be a risk factor

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Tolosa-Hunt Syndrome? (Etiology)

The cause of Tolosa-Hunt Syndrome is currently unknown.

• Research experts theorize that it may be due to an abnormal autoimmune response, which is triggered by a recent viral infection
• Autoimmune disorders develop when the body’s defense system attacks its own tissues

What are the Signs and Symptoms of Tolosa-Hunt Syndrome?

The signs and symptoms of Tolosa-Hunt Syndrome may vary between individuals and may include the following:

• Usually only one eye is affected (unilateral condition), but rarely affect both eyes can be involved (bilateral condition)
• Headaches and dizziness
• Painful and decreased eye movement; the pain can be severe in nature (stabbing pain is felt)
• It is common to have nausea and vomiting during severe pain
• Ptosis (drooping eyelid)
• Double vision or blurred vision
• Light sensitivity and neck stiffness
• The signs and symptoms of Tolosa-Hunt Syndrome during an episode can last anywhere from days to weeks

The cranial nerves of the face are affected, which can lead to a set of associated signs and symptoms. The nerves involved are the oculomotor nerve (cranial nerve III), the trochlear nerve (cranial nerve IV), and the abducens nerve (cranial nerve VI).

How is Tolosa-Hunt Syndrome Diagnosed?

Tolosa-Hunt Syndrome is a diagnosis of exclusion, which means that other conditions with similar signs and symptoms have to be eliminated before arriving at a diagnosis. A healthcare professional may diagnose Tolosa-Hunt Syndrome by examining an individual who meet all the following criteria as proposed by the International Headache Society:

• Eye pain on one side of the head that is present for many weeks, when treatment is not provided. There may be more than one episode
• Signs and symptoms, such as paresis, associated with damage to the 3rd, 4th, and/or 6th cranial nerves. This may or may not be accompanied by the presence of a granuloma (seen through imaging studies or biopsy)
• The pain and muscle weakness due to nerve damage is decreased 2-3 days after treatment with suitable steroids
• Testing to rule out conditions that cause similar signs and symptoms have been undertaken

Additional diagnostic tools include:

• Complete physical examination
• Analysis of previous medical history
• Examination of the eye using an ophthalmoscope reveals abnormally swollen optic disc in the back of the eye
• Neurological examination
• Measurement of intraocular pressure
• Visual acuity test
• CT scan of the head and neck region; MRI scan of brain

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Tolosa-Hunt Syndrome?

The complications of Tolosa-Hunt Syndrome may include the following:

• The damaged cranial nerves may get repaired in an abnormal fashion resulting in cranial nerve dysfunction. The signs and symptoms due to such complications can vary from one individual to another
• Recurrence of the condition: The signs and symptoms may ‘come and go’ with time, despite treatment

How is Tolosa-Hunt Syndrome Treated?

The treatment of Tolosa-Hunt Syndrome is determined by a healthcare professional who may administer oral corticosteroids to treat the condition.

How can Tolosa-Hunt Syndrome be Prevented?

• Currently, no preventative measures have been identified for Tolosa-Hunt Syndrome
• However, frequent follow-up with a healthcare provider is strongly recommended

What is the Prognosis of Tolosa-Hunt Syndrome? (Outcomes/Resolutions)

• With appropriate treatment, the prognosis for Tolosa-Hunt Syndrome is good. The condition is treatable in a majority of cases with prompt diagnosis
• Pain normally begins to subside and disappear within 24-72 hours following treatment commencement. The other signs and symptoms also get better with time through appropriate treatment

Additional and Relevant Useful Information for Tolosa-Hunt Syndrome:

Usually only one side of the face is affected by Tolosa-Hunt Syndrome, but there have been reported cases of individuals with both sides being affected.