Sydenham’s chorea is a rare neurological disease, caused by infection with a bacteria called group A b-hemolytic streptococci. Its main symptom is an alteration in movements. This disease was first described by an English doctor, Thomas Sydenham, in 1686.
In this article we explain what this disease consists of, relevant data and what its causes, symptoms and treatments are.
Sydenham chorea: definition and general characteristics
Sydenham’s chorea is a rare neurological disease. Other names it receives are: Minor Korea, Rheumatic Korea or San Vitus Dance. It is a movement disorder caused by the infection of some bacteria belonging to group A “Streptococcus”.
Regarding its characteristics, it is a degenerative, non-progressive, inflammatory disease of the central nervous system (CNS) ; Let us remember that the CNS is made up of the brain and the spinal cord.
Its onset is usually insidious and of limited duration. In relation to its evolution, this is variable, and is not easy to specify. Its disappearance is usually gradual. Symptoms can last approximately 3 months, although in some cases they last up to 6 and 12 months.
On the other hand, the movements that appear in Sydenham’s Korea have no apparent purpose, they are non-repetitive and involuntary. These movements end up disappearing and luckily they do not leave neurological consequences Depending on the case, the symptoms may be more severe or milder, and may even only consist of temporary psychological alterations, such as: emotional instability, restlessness and irritability.
This disorder appears more frequently in girls who have not yet reached puberty. However, Sydenham chorea can also appear in children.
A little history…
Sydenham Korea appeared more than 600 years ago, in the form of an epidemic (around 1418). However, it was not until 1686 that an English doctor, Thomas Sydenham, described the disease.
Later, Richard Bright, another English doctor, in 1831 was the one who discovered that Sydenham chorea was associated with rheumatic fever (RF) and that’s how he described it.
More data
When does Sydenham chorea usually appear? It all depends on the age at which the person becomes infected with the bacteria, logically, although said age ranges between six and 15 years. These patients usually have a rheumatic or cardiac history.
Its frequency is greater, as we mentioned, in the female sex. In fact, in adolescence, people affected by Sydenham Chorea are practically all women.
Causes
As we have already mentioned, the cause of Sydenham Korea is an infection of bacteria belonging to group A Streptococcus Streptococcus is a type of bacteria that causes other conditions such as rheumatic fever and strep throat. Specifically, the bacteria that causes Sydenham Chorea is called group A b-hemolytic streptococci.
How do this type of group A bacteria act? Interacting with a specific area of the brain: the basal ganglia (involved in movement, posture and speech). Thus, through a reaction of this brain structure, Sydenham Chorea originates.
On the other hand, Sydenham’s Korea It affects approximately 10% of people affected by rheumatic fever This association is related to the body’s autoimmune mechanisms.
In fact, Sydenham Chorea is actually a symptom (rather a sign) of another condition, caused by group A bacteria. Streptococcus; This condition is acute rheumatic fever (RF).
Two options can occur: either the person suffers from this fever, or they have suffered from it recently Some people with RF only show this sign, Sydenham Chorea, while others show a few more.
Regarding the symptoms that accompany this disease (which usually last 3 months, with cases of up to 6 and 12) we find the following four:
1. Movement disorders
We have said that the main alteration of Sydenham Chorea is a movement disorder. Thus, movements appear that have the following characteristics: they have no apparent purpose (that is, they are “meaningless” movements), they are not repetitive, they are involuntary, fast, poorly coordinated and abrupt. Its duration is limited and it does not leave neurological sequelae.
These movements disappear during sleep, and affect any muscle group (minus the ocular muscles of the eyes). Furthermore, it is necessary to specify that in mild cases of Sydenham’s Chorea, patients simply have difficulties dressing and eating, in addition to a clumsy appearance.
2. Psychic alterations
Another symptom of Sydenham’s Korea is psychic alterations, of a temporary nature. The most common are three: emotional instability, restlessness and irritability.
3. Rheumatic fever
As we have seen, rheumatic fever is another of the symptoms of Korea (or one of its signs), which It also appears as a consequence of infection by group A streptococcus It is an inflammatory disease that can cause conditions to the heart, skin, brain and joints.
On the other hand, a third of Korean cases appear associated with another symptom: rheumatic carditis. This consists of a condition that causes permanent damage to the heart valves
Treatment
In relation to the treatment of Sydenham Korea, currently There is no drug that is 100% effective in all cases in Korea
On the other hand, when the condition is severe, sometimes it is necessary to sedate the patient, in order to protect them from the self-harm that can be caused by chorea (for example, hitting the arms and legs).
Although there is no drug that is 100% effective in all cases in Korea, there are drugs that can alleviate the symptoms. Depending on their severity, four options are usually used:
1. Phenobarbital
Phenobarbital is a barbiturate, and it is the drug of choice to treat Sydenham’s Chorea. It is administered orally and Your prescription is maintained as long as necessary to eliminate abnormal movements Its efficiency is around 80%.
2. Diazepam
Diazepam is a benzodiazepine (anxiolytic), which is usually the second option. That is to say, It is administered in cases where phenobarbital has failed
3. Haloperidol
The third option to treat Sydenham Chorea is haloperidol, a type of antipsychotic. It is used when previous drugs have not worked. However, In children it is very toxic
4. Corticosteroids?
Corticosteroids have also been used in some cases, although it has not been 100% proven that these can improve the symptoms of Korea.
