Frequently Asked Questions …

How do you test for Sydenham’s Chorea?

There is no definitive test for Sydenham’s Chorea. The diagnosis is made by a doctor who needs to listen to the history of symptoms, observe the child and make a physical examination. Often, investigations are carried out to rule out any other condition that may explain the symptoms, and this can include blood samples. Blood tests and throat swabs looking for signs of infection with the Streptococcus bacteria that can trigger Sydenham’s Chorea can be helpful, but the diagnosis does not depend on these tests.

When the diagnosis of Sydenham’s Chorea is suspected, a child will be referred to a Paediatrician (a children’s specialist). The Paediatrician may look for help from a further specialist called a Neurologist. They will ask about the history of the symptoms, examine the child, organise any investigations required (which may sometimes include an MRI brain scan), and may ask other doctors to see the child before making a diagnosis, if appropriate.

Can childhood St Vitus dance / Sydenham’s Chorea cause heart disease in adulthood?

Children with Sydenham’s Chorea (often known as St Vitus dance or Chorea Minor) can have problems with their heart. Sydenham’s Chorea is a form of Rheumatic Fever, which can cause inflammation of the heart and lead to abnormalities with the heart valves. To protect against this, children are prescribed an anti-biotic. We know that some children can go on to experience heart disease in adulthood, however every case is different. Sometimes the heart valves are affected by Rheumatic Fever in a way that has only very minor impact on a child’s life as long as they continue to take antibiotics throughout their childhood.

How long does Sydenham’s Chorea last?

The movement disorder (chorea) almost always settles down either on its own, or with the help of medication. This can be quite rapid, but it can take months or longer, and varies from patient to patient. Occasionally a very mild form of chorea will persist. Symptoms can vary over time and the movement disorder is often more noticeable if the child is tired or unwell. We know that the movement disorder can come back later in childhood, however it is usually less severe and this will settle down over time. Some children go on to develop tics, which are a sign of a different kind of movement disorder. At first it can be hard to tell the difference between tics and chorea. It is thought that around one in five children will have at least one relapse over time, although the true number may be much higher. It is rare for relapses to occur in adulthood, however this may occur during pregnancy (chorea gravidorum, see below) or with the use of the oestrogen containing contraceptives, including the combined oral contraceptive pill.

Little is known about the long-term effect of Sydenham’s Chorea on behaviour and mood. In the majority of cases, the young person will make a full recovery after about two years. We know however, that some children may experience behavioural and emotional changes that require on-going support from the Child and Adolescent Mental Health Service.

What are the long-term effects of Sydenham’s Chorea?

Each child varies in the long-term effects of Sydenham’s Chorea. Many children go on to make a full recovery, whilst others may experience relapses in their symptoms, particularly the movement disorder. As with all serious illnesses, it is important to accept that recovery may take some time and a child may be fatigued or distressed as a consequence of their illness. Some children may go on to develop tics and / or emotional and behavioural problems, and in some cases these may last until they are adults. Some children may require support from the Mental Health services for a short while and in some cases there may be a need that continues over time.

We know that adults who had Sydenham’s Chorea as a child are at an increased risk of developing cardiac difficulties, particularly valve abnormalities and heart failure, and may require on-going support and medical treatment for this.

Is there a difference between boys and girls with Sydenham’s Chorea?

In one important way – if a girl has had SC in childhood, there is a chance it might come back in pregnancy (called Chorea Gravidarum). There is little research but it has been thought that around 20-40% of young women who have had SC may be affected in this way. Care must be taken with prescribing in pregnancy but treatment for Chorea Gravidarum will be similar to the usual treatment of Sydenham’s chorea and the chorea will settle when the baby is born. You should also be aware that pregnant women with rheumatic heart disease (which some people with SC get) can start to get symptoms, or worse symptoms, that need specialist care.  

In the same way, oral contraceptive pills that contain oestrogens (female hormones) can trigger a relapse of Sydenham’s Chorea (and it might be years after starting to take them).  The chorea usually settles down within a few months of stopping the pill.  There are also reports of vaginal creams and hormone replacement therapy (HRT) triggering a relapse of SC, for the same reason.  

What is the best way to treat Sydenham’s Chorea?

A child with Sydenham’s Chorea is first treated with antibiotics, usually Penicillin. This is to treat any Streptococcal infection that is present. The child will be required to remain on Penicillin until they are eighteen years old, and this is advised to protect against inflammation of the heart.

Children with chorea may benefit from advice from an Occupational Therapist or a Physiotherapist, as there are various aids and adaptations that can help to minimise the effects of the movement disorder, while it lasts.

If the movement disorder is problematic and impacting on the child’s day to day functioning, a Paediatric Neurologist, who will consider using medicines to help settle the chorea, may see the child. All medicines for movement disorder have side effects and there is no completely reliable treatment, but various drugs may be helpful. In severe cases of Sydenham’s Chorea, there may be consideration of treatment to reduce the activity of the immune system, but this is not a routine approach.

Problems arising in the months after the acute illness will need to be assessed and treated according to the assessment at this time.

Can Sydenham’s Chorea persist for decades?

Most children with Sydenham’s Chorea will be free of the condition within their childhood. This is a complex disorder and there are changes that arise as part of the condition which may continue in to adult life. Chorea usually settles but there are risks linked to the oral contraceptive pill and to pregnancy (Chorea Gravidarum). A small number of children have some persistent motor symptoms. These may be tics that are brought on by the chorea, rather than the initial type of movement disorder.

The emotional and behavioural effects of Sydenham’s Chorea can change a person’s life. Sometimes there are persistent changes in the brain that can be shown on a scan, but in other cases the process is more subtle. Not all change is a bad thing. Some people believe that Jane Avril, a famous dancer in Paris in the nineteenth century, became successful because of the impact of “St Vitus Dance” (or Sydenham’s Chorea) on her life.

Can Adults have Sydenham’s chorea?

It would be rare for a UK or US neurologist to see an adult with Sydenham’s chorea.  Adults very rarely get Sc for the first time although individual cases have been described. In adults new symptoms of the movement disorder described as ‘chorea’ can have a variety of causes and require medical investigation.

Sydenham’s chorea usually clears up after one or two episodes but some children may go on to have symptoms as adults. Some research describes the experience of adults with persistent or recurrent Sydenham’s chorea but it is not known how often the motor symptoms of chorea return or persist. It is not clear why some adults have symptoms although some women who had Sydenham’s chorea as children may have recurrent chorea in relation to hormonal changes in pregnancy or with oestrogen containing oral contraceptive pills (and potentially other oestrogen containing contraceptives, for example skin patches and vaginal rings). One study* has suggested that the disease mechanism behind Sydenham’s chorea relapses in adults may be different from the mechanism in childhood. For this reason some doctors might consider adult episodes not to be true relapses.

Sydenham’s chorea can also trigger problems such as tics or emotional difficulties. Tic disorders and emotional problems that started in childhood may also come and go in adult life. If there are no typical symptoms of chorea during episodes of these difficulties in adults it is not possible to say with any certainty that these are a form of relapse. Sometimes it can be hard to tell the difference between tics and chorea movements and this further complicates the picture.

*Reference: in 2004, Korn Lubetski et al, wrote:

“In a significant subgroup of patients, SC recurrence might not be a true relapse of rheumatic fever. It might represent either a primary underlying abnormality that renders patients susceptible to developing such a movement disorder or the outcome of permanent subclinical damage to the basal ganglia following the initial SC episode.”

Arch Neurol . 2004;61:1261-1264.

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