Sydenham's Chorea
Also called St. Vitus' dance, Sydenham's chorea is a disorder effecting children and
characterized by jerky, uncontrollable movements, either of the face or of the arms and
legs.
General Information about Sydenham's Chorea
Sydenham's chorea is a disorder that occurs in children and is associated with
rheumatic fever. Rheumatic fever is an acute infectious disease caused by certain types of
streptococci bacteria. It usually starts with strep throat or tonsillitis. These types of
streptococci are able to cause disease throughout the body. The most serious damage caused
by rheumatic fever is to the valves in the heart. At one time, rheumatic fever was the
most common cause of damaged heart valves, and it still is in most developing countries
around the world. Rheumatic fever and rheumatic heart disease are still present in the
industrialized countries, but the incidence has dropped substantially.
Rheumatic fever may appear in several different forms. Sydenham's chorea is one of five
"major criteria" for the diagnosis of rheumatic fever. There are also four
"minor criteria" and two types of laboratory tests associated with the disease.
The "Jones criteria" define the diagnosis. They require laboratory evidence of a
streptococcal infection plus two or more of the criteria. The laboratory evidence may be
identification of streptococci from a sore throat or antibodies to streptococcus in the
blood. The most common criteria are arthritis and heart disease, occurring in half to
three-quarters of the patients. Sydenham's chorea, characteristic nodules under the skin,
and a specific type of skin rash occur only 10% of the time.
Causes of Sydenham's Chorea
The cause is only certain types of streptococci, called "Lancefield Group A
beta-hemolytic." These particular germs seem to be able to create an immune response
that attacks the body's own tissues along with the germs. Those tissues are joints, heart
valves, skin, and brain.
Many patients suffer from strep throat, just before developing this new set of
symptoms. They may also have joint pains without swelling, a condition known as
arthralgia. Sydenham's chorea will appear as uncontrollable twitching or jerking of any
part of the body that is worse when trying to repress it but disappears with sleep.
Diagnosis of Sydenham's Chorea
Because rheumatic fever is such a damaging disease, a complete evaluation should be
done whenever it is suspected. This includes cultures for streptococci, blood tests, and
usually an electrocardiogram (heartbeat mapping to detect abnormalities).
Treatment of Sydenham's Chorea
Suspected streptococcus infections must be treated. All the other manifestations of
rheumatic fever, including Sydenham's chorea and excluding heart valve damage, remit with
the acute disease and do not require treatment. Sydenham's chorea generally lasts for
several months.
Prevention of Sydenham's Chorea
All strep throats should be treated with a full 10 days of antibiotics (penicillin or
erythromycin). Treatment may best be delayed a day or two to allow the body to build up
its own antibodies. In addition, for those who have had an episode of rheumatic fever or
have damaged heart valves from any other cause, prophylactic antibiotics should be
continued to prevent recurrence.
It is possible to eradicate dangerous streptococcus from a community by culturing
everyone's throat and treating everyone who tests positive. This is worth doing wherever a
case of rheumatic fever appears, but it is expensive and requires many resources. |
