National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Tolosa Hunt syndrome


Other Names:
THS; Painful ophthalmoplegia; Nonspecific inflammation of the cavernous sinus or superior orbital fissure; THS; Painful ophthalmoplegia; Nonspecific inflammation of the cavernous sinus or superior orbital fissure; Tolosa-Hunt syndrome See More
Categories:
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
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

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Patient Registry

  • The Autoimmune Registry supports research for Tolosa Hunt syndrome by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Organizations Providing General Support

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Tolosa Hunt syndrome. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • I find that there is very little info about the treatment of this condition. Any info that you can provide would be appreciated. See answer


  1. Kenneth S Shindler. Tolosa-Hunt syndrome. UpToDate. Waltham, MA: UpToDate.com; February 2013;
  2. Tolosa Hunt Syndrome. NORD. March 30, 2012; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/399/viewAbstract. Accessed 3/25/2013.