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$Title{Meige Syndrome}
$Subject{Meige Syndrome Brueghel syndrome Blepharospasm Oromandibular Dystonic
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Copyright (C) 1986, 1990 National Organization for Rare Disorders, Inc.

235:
Meige Syndrome

** IMPORTANT **
It is possible the main title of the article (Meige Syndrome) is not the
name you expected.  Please check the SYNONYMS listing to find the alternate
names and disorder subdivisions covered by this article.

Synonyms

     Brueghel syndrome
     Blepharospasm Oromandibular Dystonic syndrome

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.


Meige syndrome is a neurological movement disorder.  It is characterized
by abnormal jerky movements of the muscles of the face, jaw, eyebrows and
spasm of the muscle around the eye.  There may also be tongue, throat, and
possible respiratory spasms.

Symptoms

Meige syndrome is characterized by impaired voluntary movements (dyskinesia),
ceaseless rapid involuntary jerky movements (chorea), and disordered tension
of all muscles (dystonia) of the facial musculature such as the jaws and the
eyebrows.  Rapid eye blinking and spasms of the muscles around the eye
(blepharospasm) can cause the inability to control movement of the eyelids,
resulting in involuntary closure of the eyes intermittently.  The trunk and
the extremities may be involved, but this is less common than facial
involvement.  Tongue, throat, and possible respiratory system spasms may also
occur.  Onset of Meige's syndrome is gradual, occurring usually in
individuals over 50 years of age.

Causes

The specific cause of this neurological movement disorder is not known.
Malfunction of brain chemicals (neurotransmitters) has been implicated in
Meige's syndrome.

Affected Population

Meige's syndrome usually affects people over 50 years of age although it can
occur at younger ages.

Related Disorders

Benign Essential Blepharospasm is a disorder affecting the circular muscle
around the eye.  It is characterized by strong involuntary contractions of
this muscle resulting in intermittent loss of control over eyelid movements.
The blepharospasm is similar, but other facial muscles are also involved in
Meige's syndrome.  (For more information on blepharospasm, choose Benign
Essential Blepharospasm as your search term in the Rare Disease Database.)

Therapies:  Standard

Drugs used to treat the blepharospasm of Meige syndrome include the
following:

     a.  diazepam, a tranquilizer
     b.  levodopa, an anti-parkinsonian agent
     c.  methyldopa, an antihypertensive
     d.  trihexyphenidyl, an anticholinergic
     e.  lithium (which is also used to treat manic depression)
     f.  baclofen (Lioresal), a muscle relaxant
     g.  clonazepam (Clonopin), an anticonvulsant

Therapies:  Investigational

Botulinum A Toxin is being used experimentally on Meige Syndrome.  This
orphan drug (brand name Oculinum) is injected into muscles in order to halt
spasms by temporarily paralyzing the muscle.  Treatment must be repeated
within a few months when the spasms return.

This disease entry is based upon medical information available through
April 1990.  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 Meige Syndrome, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     (203) 746-6518

     NIH/National Institute of Neurological Disorders & Stroke (NINDS)
     9000 Rockville Pike
     Bethesda, MD  20892
     (301) 496-5751
     (800) 352-9424

     Dystonia Medical Research Foundation
     8383 Wilshire Blvd.
     Beverly Hills, CA  90211
     (213) 852-1630

     Dystonia Medical Research
     1 E. Wacker Dr., Suite 2900
     Chicago, IL  60601-20998

     Benign Essential Blepharospasm Research Foundation, Inc. (BEBRF, Inc.)
     P.O. Box 12468
     Beaumont, TX 77726-2468
     (409) 832-0788

References

CECIL TEXTBOOK OF MEDICINE, 18th ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.:  W. B. Saunders Co., 1988.  Pp. 2150-1.

THE MERCK MANUAL 15th ed:  R. Berkow, et al:  eds; Merck, Sharp & Dohme
Research Laboratories, 1987.  P. 1421.