$Unique_ID{BRK04271} $Pretitle{} $Title{Tolosa-Hunt Syndrome} $Subject{Tolosa-Hunt Syndrome Ophthalmoplegia Painful Ophthalmoplegia Syndrome Orbital Cellulitis Cavernous Sinus Thrombosis Ophthalmoplegia Migraine Headache } $Volume{} $Log{} Copyright (C) 1987, 1989 National Organization for Rare Disorders, Inc. 399: Tolosa-Hunt Syndrome ** IMPORTANT ** It is possible the main title of the article (Tolosa-Hunt Syndrome) is not the name you expected. Please check the SYNONYMS listing on the next page to find alternate names, disorder subdivisions, and related disorders covered by this article. Synonyms Ophthalmoplegia, Painful Ophthalmoplegia Syndrome Information on the following diseases can be found in the Related Disorders section of this report: Orbital Cellulitis Cavernous Sinus Thrombosis Ophthalmoplegia Migraine Headache General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Tolosa-Hunt Syndrome is characterized by severe headaches often preceding painful eye muscle paralysis (ophthalmoplegia). Symptoms usually affect only one side of the head. Double vision, fever, vague feelings of discomfort, nausea and vomiting (which are symptoms often associated with migraine headaches) may also occur. With treatment, pain may subside in twenty-four to seventy-two hours. In untreated cases, acute attacks may ease within fifteen to twenty days. Symptoms The major symptoms of Tolosa-Hunt Syndrome include chronic headaches, mild fever and vision impairment followed by painful eye muscle paralysis. Swelling, protrusion of the eye, drooping eyelid, diminished vision and abnormal skin sensations around the eye may be associated with the paralysis. These symptoms usually occur on only one side of the head. Additionally, symptoms often associated with migraine headaches such as double vision, nausea, vomiting and a general feeling of discomfort may develop. Symptoms of Tolosa-Hunt Syndrome can recur spontaneously several times even after treatment in some cases. Causes Tolosa-Hunt Syndrome is thought to be caused by an abnormal autoimmune response linked with an inflammation in the area behind the eyes (cavernous sinus and superior orbital fissure). Autoimmune disorders are caused when the body's natural defenses against begin to attack healthy tissue for unknown reasons. Other possible causes include generalized inflammation and constricted or inflamed cranial blood vessels. Affected Population Tolosa-Hunt Syndrome occurs in males and females in equal numbers. Average age of onset is forty-one years of age although it may occur at any age. Related Disorders Symptoms of the following disorders can be similar to Tolosa-Hunt Syndrome. Orbital Cellulitis is characterized by inflammation of the tissues surrounding the cavity which holds the eyeball. Symptoms include extreme pain, impaired eye movement, swelling, fever and a general feeling of discomfort. Possible complications may include impaired vision, vein abnormalities and spread of the inflammation to the entire eye area, brain or the membranes surrounding the brain. Cavernous Sinus Thrombosis is an ophthalmologic disorder usually caused by infection and clotting in veins behind the eyeballs. It can be a complication of Orbital Cellulitis or infections of facial skin. Swelling and protrusion of the eye, fever, headache and possibly convulsions are symptoms of this disorder. Prompt treatment with antibiotics, intravenous fluids and bed rest is recommended. Migraine Headaches usually involve one side of the head like the Tolosa- Hunt Syndrome. Individuals who suffer from these intense headaches may have a genetic predisposition to them. Often associated with these painful attacks are irritability, nausea, vomiting, constipation or diarrhea, and sensitivity to light. Medical researchers believe constriction of the cranial arteries may precede migraine headaches in some cases. Fever and eye muscle paralysis are not symptomatic of migraine headaches and should alert physicians to the possibility of Tolosa-Hunt Syndrome. The following disorder may be associated with Tolosa-Hunt Syndrome as a secondary characteristic. This is not necessary for a differential diagnosis: Ophthalmoplegia is a symptom of Tolosa-Hunt Syndrome. It is defined as paralysis of the eye muscles. The eye itself is unable to move or look in various directions. Swelling, diminished clear vision, drooping eyelids, unusual skin sensations in the area around the eye or protrusion of the eyeball may be associated with the paralysis. This condition may accompany a variety of other disorders. Symptoms can range from mild to severe. Therapies: Standard The pain associated with Tolosa-Hunt Syndrome may improve with short term use of steroid drugs in many cases. Pain usually subsides in untreated cases within fifteen to twenty days. With drug treatment, pain may subside within twenty-four to seventy-two hours although attacks may recur at any time in the future. Therapies: Investigational This disease entry is based upon medical information available through April 1989. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Tolosa-Hunt syndrome, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 National Migraine Foundation 5252 North Western Avenue Chicago, IL 60625 (312) 878-7715 NIH/National Institute of Neurological Disorders & Stroke (NINDS) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5751 (800) 352-9424 References STEROID RESPONSIVE OPHTHALMOPLEGIA IN A CHILD. DIAGNOSTIC CONSIDERATIONS: R.S. Kandt, et al.; Arch Neurol (June 1985, issue 42(6)). Pp. 589-591. TRANSIENT UNILATERAL OCULOMOTOR PARALYSIS: E. Kattner, et. al.; Monatsschr Kinderheilkd (March 1985, issue 133(3)). Pp. 175-177. A NEW ETIOLOGY FOR VISUAL IMPAIRMENT AND CHRONIC HEADACHE. THE TOLOSA- HUNT SYNDROME MAY BE ONLY ONE MANIFESTATION OF VENOUS VASCULITIS: J. Hannerz, et al.; Cephalalgia (March 1986, issue 6(1)). Pp. 59-63.