Tolosa-Hunt Syndrome

Sudden severe eye pain

In Tolosa-Hunt syndrome, inflammation of the cavernous sinus (behind the eyes) causes severe eye pain and irritation or damage of the nerves of the face. Males and females are affected equally by Tolosa-Hunt syndrome, which usually affects people more than 20 years old.

Symptoms

Tolosa-Hunt syndrome begins with severe pain behind or around one eye that comes on suddenly. The pain can be constant and intense. As the sinus inflammation increases and spreads, nerves in the face can be affected, producing symptoms such as drooping eyelid (ptosis) of the affected eye or numbness and tingling in the forehead. Difficulty controlling eye movements (ophthalmoplegia) and the pupil may cause sensitivity to light and double or blurred vision. If left untreated, vision loss is possible.

Diagnosis

The International Headache Society criteria for Tolosa-Hunt syndrome are:

• One-sided eye pain for an average of 8 weeks if left untreated
• Associated irritation or damage to the third, fourth, or sixth cranial nerves
• The pain is relieved within 48 hours of starting to take steroid medication
• Other conditions have been ruled out by testing.

Often, the last condition is the most important, since many conditions can causes symptoms similar to Tolosa-Hunt syndrome. Magnetic resonance imagaing (MRI), angiography, or computed tomography (CT) can help determine if somthing else, such as a tumor, is causing the eye pain. If Tolosa-Hunt syndrome is present, the sinus inflammation can generally be seen during these tests as well.

Treatment

Fortunately, Tolosa-Hunt syndrome is not a fatal disorder, and can be treated with steroid medication such as prednisone. This usually provides pain relief within 24-72 hours of starting to take the medication. The vision problems and forehead numbness may take weeks or months to resolve, and sometimes the symptoms never go away completely.

As many as 30-40% of individuals may have a relapse (recurrence) of Tolosa-Hunt syndrome, generally on the same side. Because many disorders can have similar symptoms, individuals should report any new symptoms or side effects from treatment to their physicians.

Source:
Taylor, D. (2002). Tolosa-Hunt syndrome. eMedicine, accessed at http://www.emedicine.com/neuro/topic373.htm