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$Unique_ID{BRK03597}
$Pretitle{}
$Title{Chorea, Sydenham's}
$Subject{Chorea, Sydenham's Chorea Minor Infectious Chorea St. Vitus Dance
Rheumatic Chorea Huntington's Disease Cerebral Palsy Tourette Syndrome
Wilson's Disease Rheumatic Fever Scarlet Fever}
$Volume{}
$Log{}
Copyright (C) 1989 National Organization for Rare Disorders, Inc.
687:
Chorea, Sydenham's
** IMPORTANT **
It is possible that the main title of the article (Sydenham's Chorea) is
not the name you expected. Please check the SYNONYM listing to find the
alternate names and disorder subdivisions covered by this article.
Synonyms
Chorea Minor
Infectious Chorea
St. Vitus Dance
Rheumatic Chorea
Information on the following diseases can be found in the Related
Disorders section of this report:
Huntington's Disease
Cerebral Palsy
Tourette Syndrome
Wilson's Disease
Rheumatic Fever
Scarlet Fever
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.
Sydenham's Chorea is a non-progressive neurological movement disorder
characterized by uncontrollable muscle spasms of the face, and limbs. It
occurs following a streptococcal infection such as Rheumatic Fever,
Meningitis or Scarlet Fever.
Symptoms
Sydenham's Chorea usually begins with jerky, uncontrollable, nonrepetitive
muscle movements on one or both sides of the body. All muscles, except those
of the eyes, can be affected. There may be facial contortions, grunting,
difficulty in speaking and impaired coordination. In some cases, the person
may simply appear clumsy, and have difficulties with feeding and dressing
while in others the movements may be so intense that the patient must be
protected from self-injury. The duration of this disorder is limited,
usually lasting from six to eight months, with symptoms subsiding gradually.
There is usually no long-term neurological or muscle strength impairment.
Causes
Sydenham's Chorea is rare due to the common use of antibiotics to treat
streptococcal infections. This disorder can occur as a consequence of
untreated strep infections. Approximately 10% of patients with Rheumatic
Fever may develop symptoms of Sydenham's Chorea. The onset of the symptoms
can sometimes be delayed for up to six months after the initial infection.
The disorder occurs more often during the late summer and early fall.
Affected Population
Sydenham's Chorea occurs more often in females than in males, usually between
the ages of five and fifteen. Early diagnosis of strep infections, and
treatment with antibiotics can avoid complications of streptococcal
infections such as Sydenham's Chorea.
Related Disorders
Symptoms of the following disorders can be similar to those of Sydenham's
Chorea. Comparisons may be useful for a differential diagnosis:
Huntington's Disease (also known as Huntington's Chorea) is an inherited
progressively degenerative neurological illness. Those affected experience
involuntary jerky movements, loss of motor control, changes in gait, loss of
memory, and eventual loss of both mental capability and physical control. In
general, Huntington's Disease affects adults between thirty and fifty years
of age and runs a progressive course, severely weakening patients usually
over a ten to twenty year period. (For more information on this disorder,
choose "Huntington" as your search term in the Rare Disease Database).
Cerebral Palsy is a disorder of muscle control or coordination resulting
from injury to the brain during its early stages of development (the fetal,
perinatal or early childhood stages). There may be problems associated with
involuntary movements, vision, hearing, communication skills, perception
levels, intellect and seizures. (For more information on this disorder,
choose "Cerebral Palsy" as your search term in the Rare Disease Database).
Tourette Syndrome is a hereditary neurological movement disorder which
begins in childhood between the ages of 2 and 16. The disease is
characterized by involuntary muscular movements, called tics, and
uncontrollable vocal sounds. Facial grimaces, head shaking, shoulder jerking
and rapid eye blinking are common motor symptoms. Tourette Syndrome is not a
degenerative disorder and those affected can expect to live a normal life
span. (For more information on this disorder, choose "Tourette" as your
search term in the Rare Disease Database).
Wilson's Disease is a rare genetic disorder characterized by excess
storage of copper in the body tissues, particularly in the liver, brain and
corneas of the eyes. It leads eventually to liver disease, neurological
abnormalities and a characteristic rusty-brown colored ring in the cornea of
the eyes known as Kayser-Fleischer rings. Involuntary jerky motor movements
and behavioral changes are early symptoms of this disorder. (For more
information on this disorder, choose "Wilson" as your search term in the Rare
Disease Database).
The following disorders may precede the development of Sydenham's Chorea.
They can be useful in identifying an underlying cause of some forms of this
disorder:
Rheumatic Fever can occur following a strep throat. Patients initially
experience moderate fever, a general feeling of ill health (malaise), a sore
throat and fatigue. Major complications can include valvular heart disease,
joint pain and arthritis, and possible skin symptoms. Treatment should begin
as soon as possible, and be maintained for months or even years to help
control serious complications. Rheumatic Fever can be avoided if strep
throat is vigorously treated and cured with antibiotics. (For more
information on this disorder, choose "Rheumatic Fever" as your search term in
the Rare Disease Database.)
Scarlet Fever is a serious and very contagious streptococcus infection.
The main characteristics are sore throat, fever, a fine scarlet red rash,
rapid pulse and a strawberry-colored tongue. This disorder can be cured with
antibiotic drugs.
Therapies: Standard
There is no known cure for Sydenham's Chorea. Treatment consists mainly of
bed rest and possibly sedative medication to help reduce the intensity of
involuntary muscle movements. Symptoms disappear even without treatment
after 6 to 8 months.
Therapies: Investigational
At the present time, studies are being conducted on the effectiveness of
neuroleptic drugs such as carbamazepine and tetrabenzine on different forms
of chorea. More research must be conducted to determine long-term safety and
effectiveness of these drugs as a treatment for chorea type involuntary
movements.
This disease entry is based upon medical information available through
November 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 Sydenham's Chorea, 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
Centers for Disease Control (CDC)
1600 Clifton Road, NE
Atlanta, GA 30333
(404) 329-4236
References
INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and
Co., Pp. 2160-2161.
THE MERCK MANUAL, Volume 2, 14th Ed.: Robert Berkow, M.D.; ed.-in-chief;
Merck Sharp & Dohme Laboratories., 1982. Pp. 227.
CARBAMAZEPINE: AN ALTERNATIVE DRUG FOR THE TREATMENT OF NONHEREDITARY
CHOREA. M. Roig, et al.; PEDIATRICS, (September 1988; 82(Pt.2)). Pp. 492-
495.
TETRABENAZINE THERAPY OF DYSTONIA, CHOREA, TICS AND OTHER DYSKINESIAS. J.
Jankovic et al.; NEUROLOGY, (March 1988, 38(3)). Pp. 391-394.