Tolosa Hunt Syndrome

Tolosa Hunt Syndrome

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Tolosa Hunt Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • ophthalmoplegia, painful
  • ophthalmoplegia, recurrent
  • ophthalmoplegia syndrome

Disorder Subdivisions

  • None

General Discussion

Tolosa-Hunt syndrome is a rare disorder characterized by severe periorbital headaches, along with decreased and painful eye movements (ophthalmoplegia). Symptoms usually affect only one eye (unilateral). In most cases, affected individuals experience intense sharp pain and decreased eye movements. Symptoms often will subside without intervention (spontaneous remission) and may recur without a distinct pattern (randomly). Affected individuals may exhibit signs of paralysis (palsy) of certain cranial nerves such as drooping of the upper eyelid (ptosis), double vision (diplopia), large pupil, and facial numbness. The affected eye often abnormally protrudes (proptosis). The exact cause of Tolosa-Hunt syndrome is not known, but the disorder is thought to be associated with inflammation of specific areas behind the eye (cavernous sinus and superior orbital fissure).

Symptoms

Many individuals with Tolosa-Hunt syndrome experience the sudden onset of severe periorbital headache, followed by painful and decreased eye movements (ophthalmoplegia). In some cases of severe ophthalmoplegia, the eye itself is unable to move or look in various directions (frozen globe).



The major symptoms of Tolosa-Hunt syndrome include chronic periorbital headache, double vision, paralysis (palsy) of certain cranial nerves, and chronic fatigue. Affected individuals may also exhibit protrusion of the eye (proptosis), drooping of the upper eyelid (ptosis) and diminished vision. In most cases, symptoms associated with Tolosa-Hunt syndrome affect only one side (unilateral). Symptoms will usually subside without intervention (spontaneous remission) and may recur without a distinct pattern (randomly).

Causes

While the exact cause of Tolosa-Hunt syndrome is unknown, one theory is an abnormal autoimmune response linked with an inflammation in a specific area behind the eye (cavernous sinus and superior orbital fissure). In some cases, inflammation may be due to a clumping of a certain type of cell (granulomatous inflammation). Autoimmune disorders are caused when the body's natural defenses against "foreign" or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons. Other possible causes may include generalized inflammation and constricted or inflamed cranial blood vessels.

Affected Populations

Tolosa-Hunt syndrome is a rare neuro-immunological disorder that occurs in males and females in equal numbers. The average age of onset is 41 years, but there have been cases reported among people younger than age 30. In rare cases, children under the age of 10 have been diagnosed with Tolosa-Hunt syndrome.

Standard Therapies

Diagnosis

The diagnosis of Tolosa-Hunt syndrome has been codified by the International Headache Society. The following criteria must be met for a definitive diagnosis of this disorder: Eye pain on one side of the head that persists for at least eight weeks if untreated; associated irritation or damage to the third, fourth, or sixth cranial nerves; relief of pain within 48 hours upon the administration of steroids; and specialized testing that rules out other conditions such as neoplasm, infection or aneurysm.



The diagnosis of Tolosa-Hunt syndrome is suspected based upon the presence of characteristic physical features (e.g., pain, headache, ophthalmoplegia). The diagnosis may be confirmed by a thorough clinical evaluation, detailed patient history, and a variety of specialized radiologic tests including computed tomography (CT) scan, and magnetic resonance imaging (MRI). These examinations may reveal characteristic enlargement or inflammation of the areas behind the eye (cavernous sinus and superior orbital fissure).



Treatment

In most cases, the pain associated with Tolosa-Hunt syndrome subsides with short-term use of steroid drugs. Pain is usually reduced in untreated cases within fifteen to twenty days. With steroid treatment, pain typically briskly subsides within twenty-four to seventy-two hours - and this brisk steroid response aids in the diagnosis. Affected individuals may be vulnerable to recurrent future attacks.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com



Researchers have been studying various additional treatment options for individuals who do not respond to, or develop resistance to, corticosteroid treatment. Radiation therapy has demonstrated long-term improvement in several subjects. Immunosuppressive drugs such as methotrexate are being studied as a potential treatment for individuals with Tolosa-Hunt syndrome. Some researchers have advocated the use of acupuncture. More research is necessary to determine the long-term safety and effectiveness of these potential treatments for individuals with Tolosa-Hunt syndrome.



Contacts for additional information about Tolosa-Hunt syndrome:



Roman Shinder, MD

Assistant Professor of Ophthalmology

Ophthalmic Plastic & Reconstructive Surgery

SUNY Downstate Medical Center

Director Oculoplastics

Kings County Hospital Center

451 Clarkson Ave, E Bldg, 8th Fl, Suite C

Brooklyn, NY 11203

Phone: 718-270-1961



and



Renelle Pointdujour, MD

SUNY Downstate Medical Center

Department of Ophthalmology

450 Clarkson Ave, Box 58

Brooklyn, NY 11203

Phone: 718-288-6818

References

TEXTBOOKS

Naderi A, Ahmadi MA, Esmaeli B. Tolosa Hunt Syndrome. In: NORD Guide to Rare Disorders. Philadelphia, PA: Lippincott Williams & Wilkins; 2003:664.



Rowland LP, ed. Merritt's Neurology. 10th ed. Philadelphia, PA: Lippincott Williams & Wilkins;. 2000.



Kanski JJ, ed. Clinical Ophthalmology. 4th ed. Oxford, UK: Butterworth-Heinemann; 1999.



Adams RD, Victor M, Ropper AA, eds. Principles of Neurology. 6th ed. New York, NY: McGraw-Hill Companies; 1997.



Newell FW, ed. Ophthalmology: Principles and Concepts. 7th ed. St. Louis, MO: Mosby Year Book; 1991



JOURNAL ARTICLES

Foubert-Samier A, Sibon I, Maire JP, Tison F. Long-term cure of Tolosa-Hunt syndrome after low-dose focal. Headache. 2005;45:389-91.



Haque TL, Miki Y, Kashii S, et al. Dynamic MR imaging in Tolosa-Hunt syndrome. Eur J Radiol. 2004;51:209-17.



Mandrioli J, Frank G, Sola P, et al. Tolosa-Hunt syndrome due to actinomycosis of the cavernous sinus: the infectious hypothesis revisited. Headache. 2004;44:806-11.



Gladstone JP, Dodick DW. Painful ophthalmoplegia: overview with a focus on Tolosa-Hunt syndrome. Curr Pain Heachache Rep. 2004;8:321-29.



Yeung MC, Kwong KL, Wong YC, et al. Paediatric Tolosa-Hunt syndrome. J Paediatr Child Health. 2004;40:410-13.



Chmielewska B, Leszek Kaminski M. Progression of preexisting trigem-inalgia to Tolosa-Hunt-like syndrome. The importance of neuroimaging for early differential diagnosis. Neurol Sci. 2003;24:281-85.



Nepp J, Jandrasits K, Schauersberger J, et al. Is acupuncture a useful tool for pain-treatment in ophthalmology? Acupunct Electrother Res. 2002;27:171-82.



Sugano H, Iisuka Y, Arai H, et al. Progression of Tolosa-Hunt syndrome to a cavernous dural arteriovenous fistula: a case report. Headache. 2003;43:122-26.



INTERNET

Taylor DC, Mankowski K. Tolosa-Hunt Syndrome. Emedicine. http://emedicine.medscape.com/article/1146714-overview. Updated April 29, 2010. Accessed March 19, 2012.

Resources

Lighthouse International

111 E 59th St

New York, NY 10022-1202

Tel: (800)829-0500

Email: info@lighthouse.org

Internet: http://www.lighthouse.org



National Headache Foundation

820 N. Orleans

Suite 411

Chicago, IL 60610-

USA

Tel: (312)274-2650

Fax: (312)460-9049

Tel: (888)643-5552

Email: info@headaches.org

Internet: http://www.headaches.org



American Autoimmune Related Diseases Association, Inc.

22100 Gratiot Ave.

Eastpointe, MI 48021

Tel: (586)776-3900

Fax: (586)776-3903

Tel: (800)598-4668

Email: aarda@aarda.org

Internet: http://www.aarda.org/



NIH/National Institute of Neurological Disorders and Stroke

P.O. Box 5801

Bethesda, MD 20824

Tel: (301)496-5751

Fax: (301)402-2186

Tel: (800)352-9424

TDD: (301)468-5981

Internet: http://www.ninds.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



AutoImmunity Community

Email: moderator@autoimmunitycommunity.org

Internet: http://www.autoimmunitycommunity.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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