Sydenham’s Chorea (also known as St Vitus’ Dance or Chorea Minor) is a disease affecting the brain, particularly causing unusual movements, unsteadiness and poor coordination. 

It can turn a normal child into someone unable to speak, walk or feed themselves.

It happens after a common bacterial childhood infection, usually a sore throat, called Streptococcus pyogenes (also called Group A streptococcus). In certain individuals, for reasons which are unclear, their own immune system reacts in an unusual way to this infection, where even though the infection is cleared, parts of the child’s brain called the basal ganglia are also affected. 

The basal ganglia are collections of brain cells located deep within the brain which have a role in controlling movement and emotional responses.

Sydenham’s Chorea usually develops a couple of weeks (but up to six months) after a child has suffered a Streptococcal infection. You may not even remember or realize that your child had a Streptococcal infection.

Most people who develop Sydenham’s Chorea do so before the age of eighteen. It is more common in girls than boys.

Sydenham’s Chorea can occur as part of a wider inflammatory response called Rheumatic Fever, which involves other parts of the body including the skin, joints and heart. 

Sydenham’s Chorea can present in a number of ways. Often the child’s behaviour changes, before the abnormal movements start.

Over hours to days, uncoordinated jerking movements in the arms, hands, legs, feet and face. start to be seen. These movements are called chorea. Sometimes the chorea only involves one side of the body (this is called “hemichorea”).

Further neurological symptoms of Sydenham’s Chorea include:

• changes in mood and behaviour,
• fatigue,
• problems using hands to play or eat,
• floppiness (loss of muscle tone),
• speech difficulties,
• problems with walking,
• grimacing,
• headache,
• restlessness.

Children with Sydenham’s Chorea often have other kinds of odd movements such as tics (see below), which can include involuntary noises.

Other physical problems may be due to rheumatic fever (joint pain, for example).

See examples of chorea at our Youtube playlist.

The diagnosis of  Sydenham’s Chorea may be made as a result of a review of the story of the
child’s symptoms and a physical examination by a General Practitioner (GP), a
Paediatrician or at an Accident and Emergency department. There is no definite
test for Sydenham’s Chorea, but tests may be important to rule out other
conditions that might be suspected in some cases. The diagnosis may be
supported by a blood sample or throat swab looking for signs of Streptococcal
infection.

Once the diagnosis has been suspected, a child may be referred to a specialist
Paediatric doctor called a Neurologist who will further assess by asking
questions and physically examining them, before confirming a clinical diagnosis
of Sydenham’s Chorea. Sometimes a child may need to go for an MRI scan to rule
out other conditions, but this is not always necessary.

Depending on a child’s condition, the Neurologist may refer on to other professionals,
including:

• Cardiologist
• Community Paediatrician
• Educational Psychologist
• Liaison CAMHS, Neuro-Psychiatry
  Service
  Neuro-Psychologist
• Physiotherapist / Occupational
  Therapist / Speech & Language Therapist
• Rheumatologist
• School Nurse
• Specialist Nurse

Chorea is a particular kind of abnormal movement that is a key feature of Sydenham’s chorea, although it can be seen in other conditions.

The movements are involuntary, that is to say the child cannot help control them, and in this sense they are similar to tics.  

The child will often look as if they are unsteady, as if there legs are going away from under them, or as if they are staggering.  They will often find it difficult to do things with their hands, such as writing or playing, because their hands and arms will keep going the wrong way.

The chorea usually develops over a day or two.  Sometimes it only involves one side of the body (called “hemichorea”).

There are other abnormal movements that are seen in Sydenham’s Chorea, such as grimacing (odd facial expressions) and “piano playing” movements of the hands and fingers.  

Symptoms can vary over time, for example the chorea is often more noticeable if a child is tired or unwell. 

Your doctor may try to treat the movement disorder if it is having a major impact on the child’s day to day functioning. With or without medication, It almost always settles down although it usually takes 2 to 4 months, and in some cases it can take a year or more.  It just varies from patient to patient.

See our Youtube Playlist for more examples.

Tics

There are children who suddenly develop tics, where it isn’t clear whether the diagnosis is Sydenham’s chorea or something else, for example PANDAS.  Tics are quite common in childhood, more common in boys, they often come and go over the years before disappearing around or after puberty.  It can be hard to tell the difference between tics and chorea, which is why having an experienced doctor is important.  

Tics are sudden, jerky or flailing movements, typically of the eyes, face, neck, shoulders and arms.  You will see the same movement happening over and over (this is called “stereotyped”), although there can be long breaks in between.  Many different tics can be present at the same time, and one kind of tic can go away after a while, only to be replaced by another.  Some children also make involuntary noises, such as grunts and yelps, these are also a kind of tic (“vocal tic”).

Chorea tends to be a mixture of fast (jerky) and slower (writhing) movements, and tends to be more chaotic and unpredictable.   

So observing and describing all the different kind of movements is really important for getting to a diagnosis, as is looking for the other features of Sydenham’s chorea such as the grimacing and “piano playing” fingers mentioned above, plus features of streptococcal infection and evidence of rheumatic fever (such as heart damage).  

Treatment for Sydenham’s Chorea is based on the following steps:

• Most doctors will try to remove any Streptococcal infection from the child using a treatment course of Penicillin.  But the infection may have already cleared by the time the diagnosis is made
• In most cases, preventive, long term penicillin (or an alternative antibiotic, if you are allergic) is then recommended.  If your Sydenham’s chorea is part of rheumatic fever, then long term penicillin should definitely be given, to prevent heart damage, or worsening of any heart damage.
  • If you don’t have rheumatic fever, then it’s not clear whether long term penicillin prevents relapses of chorea, but it makes sense that it might.  There doesn’t appear to be a risk of heart damage if you have SC without other features of rheumatic fever.
  • See here for more on long term penicillin.
• Your doctor may offer medicines to treat the chorea if it is having a major impact on the child’s day to day functioning. There are not any large trials to help decide which medication is best, but doctors often use an epilepsy medicine called Sodium Valproate which can help reduce the chorea. Other medications which may be tried include Haloperidol, Pimozide, Carbamazepine and Clonidine.
• Your doctor may offer immune treatment.  There is certainly good evidence that steroid treatment reduces the severity and duration of the movement disorder (from an average of 119 days to just 54 days).  There is also good evidence that intravenous immunoglobulin (IVIG) treatment helps.  But there isn’t enough information from research yet to know about which children will benefit most from these treatments, which is better, or possible longer term benefits or harms. A brief scientific review is available here.
• Occupational therapy, physiotherapy and sometimes speech and language therapy is important for trying to help the child do as much for themselves as possible, and prevent loss of muscle strength.
• Mental health team input may be necessary where there are problems with mood or anxiety.

The movement disorder (chorea) almost always settles down either on its own or with the help of medication. This can take months or even longer and varies from patient to patient. Symptoms can vary over this time, for example the movement disorder is often more noticable if the child is tired or unwell.

The movement disorder can recur later in childhood (a relapse), but once again this will settle down over time. Older medical papers and textbooks suggest that one in five children with Sydenham’s Chorea will have at least one relapse during childhood. Many doctors now feel this may be an under-estimate. It is very rare for patients to have relapses during adulthood, although it is recognised to occur during pregnancy (Chorea Gravidarum) and in association with the oral contraceptive pill.

Unfortunately little is known about the long term effect of behaviour and mood in children who have had Sydenham’s Chorea. In the majority of cases, the young person will make a full recovery after about two years, with no long lasting symptoms, and will not require on-going treatment or follow-up. However, some children may experience behavioural and emotional changes that may start before and carry on after the movement disorder and may require on-going support from a Child & Adolescent Mental Health Service. These changes may come and go and in some cases there are underlying changes in the brain which can persist into adult life. The symptoms that affect a child’s mental health may need help and treatment over a longer period of time than the chorea.

How does Sydenham’s Chorea affect my life?

Sydenham’s Chorea should not stop your child living and enjoying life. At first, chorea movements may make it difficult for your child to get out and about like they normally do. This part of the condition should pass quite quickly as the movement disorder reduces. Some children complain about being tired and this may impact on their ability to enjoy school, hobbies, friendships and family time. This tiredness can be a problem even when there is no heart problem and after movements seem to have settled. It can help to look with your child at their activities through the week and reduce any that are not enjoyable or completely necessary, that could be potentially increasing their tiredness. This will help to focus energy and time on getting the best from their body, physically and mentally as the child builds up their strength gradually.

Feelings & Behaviour

Some children will develop emotional or behavioural difficulties. These might include depression, anxiety about separation from parents, personality changes, being overly emotional, obsessive-compulsive disorder (OCD) and attention deficit/hyperactivity disorder (ADHD). It is not known if these emotional effects are part of Sydenham’s Chorea or a result of it. Sometimes emotional outbursts happen just before involuntary movements begin. Some children are able to cover up symptoms in school but behave very differently at home. This is hard for parents to manage, but is a sign that the child feels safest at home. This may mean that a child can cope in school by letting problems out with the family. Changes in your child’s mental health can be very difficult for parents, grandparents, external family and siblings to adjust to. Some parents talk about seeing their child ‘changing before their eyes’.

How will Sydenham’s Chorea affect school?

You might have lots of hospital appointments to attend initially. Together with the disease this can affect your child’s attendance at school. It is not helpful for children to be worrying about getting behind with work. It is helpful for parents or carers to link closely with school to minimise the work a child is missing and to help them to keep up. It is useful to pick up school work for your child to complete at home and sometimes, teachers may visit your child to complete lessons at home. Starting back at school may be easier with a part-time timetable. It may be helpful to ask for a child to be referred to the school’s Educational Psychologist for additional support. If your child is attending school, you may want to inform the school about the condition, including the possible emotional and behavioural issues. Your child may notice that their concentration and attention is affected and this can make learning in the classroom harder on some days. Sydenham’s Chorea is not well known. A child may be more vulnerable to bullies if their peers do not understand the symptoms. It might be helpful to ask the school to complete a lesson on Sydenham’s Chorea to help educate the class on the condition. Some children find the chorea movements difficult to manage in school and health professionals can help you to think about how you manage this more successfully. For example, sometimes a change of position in the class can mean that movements are less troubling for the child and those nearby. Extra time in exams and tests may also be needed.