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$Unique_ID{BRK03830}
$Pretitle{}
$Title{Horner's Syndrome}
$Subject{Horner's Syndrome Oculosympathetic Palsy Bernard-Horner Syndrome Adie
Syndrome Wallenberg Syndrome }
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Copyright (C) 1991 National Organization for Rare Disorders, Inc.

864:
Horner's Syndrome

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

Synonyms

     Oculosympathetic Palsy
     Bernard-Horner Syndrome

Information on the following diseases can be found in the Related
Disorders section of this report:

     Adie Syndrome
     Wallenberg 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.

Horner's Syndrome is a disorder that consists of extreme contraction of
the pupil of the eye (miosis), drooping of the upper eyelid (ptosis), absence
of sweating of the face (anidrosis), and sinking of the eyeball into the bony
cavity that protects the eye (enophthalmos).  The disorder is caused by a
buildup of tissue (lesion or growth) or injury somewhere along the cervical
sympathetic chain.  The cervical sympathetic chain is a grouping of nerves
that involuntarily stimulate the muscles, the heart, and the sweat glands.
This chain governs the uncontrolled reflexes our body performs such as
contraction of the eye.

Symptoms

The symptoms of Horner's Syndrome are:

1.  Drooping of the upper eyelid (ptosis).  This is caused by paralysis
of the muscles of the eye that help the lens to be curved or rounded
(Muller's muscle).

2.  Sinking of the eyeball into the cavity that protects it
(enophthalmos).  This is caused by a narrowing of the fold on the eyelid due
to drooping and swelling of the upper eyelid.

3.  Swelling of the lower eyelid.  This is due to paralysis of the muscle
attached to the tissue that helps support the eyelid.

4.  An absence of sweating on the same side of the face as the affected
eye.  This is caused by a lesion in a nerve near the base of the skull.

5.  The pupil becomes smaller (contraction).

6.  An increase in vibration when the eye is adjusting to see things at
different distances.

7.  Each iris may be a different color, or two different colors within
one iris (heterochromia).  This is caused by congenital (inborn) or acquired
lesions.

Causes

Horner's Syndrome is caused by a partial interruption of the sympathetic
chain due to a lesion or growth.  The lesion develops somewhere along the
path from the eye to a point in the brain that controls involuntary
movements.  The lesion may be present at birth, acquired during infancy,
develop sporadically, or be caused by trauma.

The most common causes of Horner's Syndrome are:

     Inflammation or growth located in the lymph nodes located in the neck.
     Trauma to the neck caused by injury or surgery.
     A lesion or growth near the nerves in the cavity under the arm that
supplies nerves to the shoulder, chest, and arm.
     Fracture at the base of the skull.
     A lesion of the first and second segments of the spine caused by injury.
     A chronic, progressive disease of the spinal cord.
     A blood clot, air bubble in the blood, or lesion on either side of the
brainstem.
     A tumor in the lung.

When Horner's Syndrome occurs for no other apparent reason, it may be
inherited as an autosomal dominant genetic disorder.  (Human traits including
the classic genetic diseases, are the product of the interaction of two genes
for that condition, one received from the father and one from the mother.  In
dominant disorders, a single copy of the disease gene (received from either
the mother or father) will be expressed "dominating" the normal gene and
resulting in appearance of the disease.  The risk of transmitting the
disorder from affected parent to offspring is 50% for each pregnancy
regardless of the sex of the resulting child.)

Affected Population

Horner's Syndrome is a rare disorder that affects males and females in equal
numbers and may occur at any age.

Related Disorders

Symptoms of the following disorders can be similar to those of Horner's
Syndrome.  Comparisons may be useful for a differential diagnosis.

Adie Syndrome is a rare neurological disorder affecting the pupil of the
eye.  The symptoms of this syndrome are a large (dilated) pupil, and slow
reaction to light or focus on nearby objects.  In some patients the pupil may
be smaller than normal rather than dilated.  Absent or poor reflexes are also
associated with this disorder.  (For more information on this disorder,
choose "Adie Syndrome" as your search term in the Rare Disease Database).

Wallenberg Syndrome is a rare disorder caused by a blood clot.  It is
characterized by difficulty articulating words due to disease of the central
nervous system, difficulty swallowing, a staggering gait, dizziness, low
pressure of the fluid in the eyeball that gives it a round shape, lack of
coordination in voluntary movement, rapid involuntary movement of the
eyeball, signs of Horner's Syndrome on the side where the lesion is present,
and a loss of pain and temperature senses on the side of the body opposite
the lesion.

Therapies:  Standard

The treatment of Horner's Syndrome depends on the location and cause of the
lesion or tumor.  In some cases surgical removal of the lesion or growth may
be appropriate.  Radiation and chemotherapy may be beneficial to patients
with malignant tumors.

Genetic counseling may be of benefit for patients and their families if
they have the genetic form of this disorder.  Other treatment is symptomatic
and supportive.

Therapies:  Investigational

This disease entry is based upon medical information available through
September 1991.  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 Horner's Syndrome, please contact:

     National Organization for Rare Disorders
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     [phone]

     NIH/National Eye Institute
     9000 Rockville Pike
     Bethesda, MD  20892
     [phone]

For genetic information and genetic counseling referrals, please contact:

     March of Dimes Birth Defects Foundation
     1275 Mamaroneck Avenue
     White Plains, NY  10605
     [phone]

     Alliance of Genetic Support Groups
     35 Wisconsin Circle, Suite 440
     Chevy Chase, MD  20815
     (800) 336-GENE
     [phone]

References

MENDELIAN INHERITANCE IN MAN, 8th ed.:  Victor A. McKusick; Johns Hopkins
University Press, 1986.  P. 472.

CECIL TEXTBOOK OF MEDICINE, 18th ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.:  W.B. Saunders Co., 1988.  Pp. 2113-4.

PRINCIPLES OF NEUROLOGY, 4th Ed.:  Raymond D. Adams and Maurice Victor,
Editors; McGraw-Hill Information Services Co., 1989.  Pp. 20-21, 435-6.

CLINICAL OPHTHALMOLOGY, 2nd Ed.:  Jack J. Kanski, Editor; Butterworth-
Heinemann, 1990.  Pp. 474-5.