$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.