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Tolosa Hunt syndrome

Other Names for this Disease
  • Nonspecific inflammation of the cavernous sinus or superior orbital fissure
  • Painful ophthalmoplegia
  • THS
  • Tolosa-Hunt syndrome
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What is Tolosa Hunt syndrome?

How might Tolosa Hunt syndrome be treated?

What is Tolosa Hunt syndrome?

Tolosa Hunt syndrome (THS) is a rare condition characterized by painful ophthalmoplegia (paralysis and/or weakness of the eye muscles). Onset can occur at any age.[1] Signs and symptoms include a constant pain behind the eye; decreased eye movements; and signs of cranial nerve paralysis such as drooping of the upper eyelid (ptosis), double vision (diplopia), large pupil, and facial numbness.[1][2] Although it is considered a benign condition, permanent neurologic deficits can occur and relapses are common. The features of THS are caused by inflammation of the cavernous sinus (an area at the base of the brain) but the underlying cause of the inflammation is unknown. Left untreated, symptoms may resolve spontaneously after an average of about eight weeks. Treatment may include use of glucocorticoids or other immunosuppressive therapies.[1]
Last updated: 3/25/2013

How might Tolosa Hunt syndrome be treated?

Left untreated, symptoms may resolve spontaneously after an average of about eight weeks. Glucocorticoids have long been the recommended treatment for Tolosa Hunt syndrome. Little consideration has been given to alternative therapies, probably due to the typical rapid response to glucocorticoids (pain usually resolves within 24 to 72 hours after starting treatment). However, there are little data other than case series to determine the most effective dose, the route and schedule of administration, or the length of therapy. Furthermore, while it is known that glucocorticoids relieve the pain associated with the condition, there is no definitive evidence that associated cranial neuropathies recover any faster with or without treatment.[1] A few affected individuals may need other immunosuppressive medications either to limit the complications of corticosteroid use or to keep the disorder in remission. These individuals typically undergo a biopsy to confirm the diagnosis of THS.[1]

Close follow-up with repeat MRI is typically recommended to confirm glucocorticoid treatment remains effective and no evidence of another cause for the symptoms develops. Improvement seen on MRI is often not seen until several weeks after signs and symptoms improve.[1]
Last updated: 3/25/2013

  1. Kenneth S Shindler. Tolosa-Hunt syndrome. UpToDate. Waltham, MA:; February 2013;
  2. Tolosa Hunt Syndrome. NORD. March 30, 2012; Accessed 3/25/2013.