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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.