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Tolosa-Hunt Syndrome is a disease defined as a severe and unilateral periorbital headache associated with painful and restricted eye movements. It is a disease associated with painful sensations in your eyes that can be quite bothersome but there are therapies that may help you. 

This is an idiopathic disease that many researchers believe originates from inflammation of the cavernous sinuses or super orbital fissures. There could also be a history of traumatic injuries, tumors or aneurysms that may also lead to these symptoms. 

Thankfully, it is a very rare disease and is believed to affect only 1 in 1 million patients. If you are the unlucky one, however, this means very little for you. This is a disease that will typically manifest in older patients with the onset of symptoms occurring when patients are around 41 years old typically. 

Patients will come to the clinic with a history of severe pain that is often described as shooting, stabbing, boring and severe. In most people with the disease, it is a disease that is relapsing and remitting so symptoms will come and go. These attacks can occur even months or years apart so it can be a disease that is quite bothersome for patients. 

The oculomotor nerve (the nerve responsible for movements of the eye) will be affected in up to 80 percent of cases. Patients may also be affected by diseases like Horner Syndrome, another disease of the eye. 

Patients will be diagnosed typically based just on their presentation of symptoms. Neurological testing and imaging studies will also be necessary to help rule out some of the more serious causes of a similar disease. Laboratory testing and CSF analysis through lumbar punctures are also necessary but potentially more painful for the patients to tolerate. 

Patients will be given steroids in order to help reduce the inflammation of this disease. The symptoms can improve in as little as two or three days after the therapy. There are no specific guidelines to help determine the staging or the specific treatments that may be necessary but it is possible for patients to get over their symptoms after one treatment of steroids. The treatment will begin as an initial high-dose therapy that will be lowered after the first week. The dose will continue to be lowered gradually over the next few weeks to months. A follow-up MRI will be done to verify that the inflammation as stopped. 

In the few cases where this does not stop symptoms from occurring, patients will be given a more intense therapy. Azathioprine, methotrexate, and infliximab are some of the agents that are typically used to help with the immunosuppression. Even radiotherapy may be a necessary step in order to manage the recurrent flare-ups. 

Even with therapy, relapses are common in up to 50 percent of patients so you have to have patience with this type of disease. If you have relapses, steroids are going to be the first-line therapy again and more aggressive agents could be added to try to suppress the symptoms. [1]

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