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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.
-
-