NORD gratefully acknowledges Roman Shinder, MD FACS, Professor of Ophthalmology and Otolaryngology, Director of Oculoplastics, SUNY Downstate Medical Center; and Renelle Pointdujour, MD, Department of Ophthalmology, SUNY Downstate Medical Center, for assistance in the preparation of this report.
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).
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).
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.
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.
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).
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.
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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
Professor of Ophthalmology, Otolaryngology
Director of Oculoplastics
SUNY Downstate Medical Center
541 Clarkson Ave, E Bldg, 8th Fl, Suite C
Brooklyn, NY 11203
Renelle Pointdujour, MD
SUNY Downstate Medical Center
Department of Ophthalmology
450 Clarkson Ave, Box 58
Brooklyn, NY 11203
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