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- $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.
-
-