Sydenham’s Chorea

Also known as: St Vitus’ Dance; Chorea Minor

Background

Sydenham’s Chorea is a disease affecting the brain, particularly causing unusual movements, unsteadiness and poor coordination.

It can turn a healthy 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). The body’s own immune system attacks the infection but also parts of the child’s brain which have a role in controlling movement and emotional responses.

In most cases the child makes a full recovery, although it can take several months.

Credits

Medical text written November 2021 by Sydenham’s Chorea Association, UK.

Although great care has been taken in the compilation and preparation of all entries to ensure accuracy, we cannot accept responsibility for any errors or omissions. Any medical information provided is for education/information purposes and is not designed to replace medical advice by a qualified medical professional.

What are the symptoms

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

The movement disorder comes on over a period of hours to days with uncoordinated jerking movements in the arms, hands, legs, feet and face. These movements are called chorea. Sometimes the chorea only involves one side of the body.

Further neurological symptoms of Sydenham’s Chorea can be changes in mood and behaviour, fatigue, loss of fine and gross motor skills, loss of muscle tone, motor and speech difficulties, gait disturbance, facial grimacing, headache, slowed thinking and restlessness.

Some children with Sydenham’s Chorea go on to develop a different kind of movement problem with motor and/or vocal tics and habits.

Other physical symptoms may include inflammation of the heart and joint pain. This usually indicates that the underlying problem is rheumatic fever, although you can have Sydenham’s chorea without rheumatic fever too.

What are the causes

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 attacks not only the infection, but parts of the child’s own 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.

How is it diagnosed

The diagnosis of Sydenham’s Chorea may be made as a result of a review of the child’s symptoms and a physical examination by a doctor, ideally one who has seen it before.

There is no definite test for Sydenham’s Chorea, but blood tests and scans may be important to rule out other conditions that might be suspected in some cases. Sometimes a lumbar puncture is done.  The diagnosis may be supported by a blood sample or throat swab looking for signs of Streptococcal infection.

How is it treated

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. 
  • In most cases, preventive, long term penicillin (or an alternative antibiotic, if you are allergic) is then recommended. 
  • Your doctor may offer medicines to treat the chorea if it is having a major impact on the child’s day to day functioning. Doctors often use an epilepsy medicine called Sodium Valproate.
  • Your doctor may offer immune treatment.  There is certainly good evidence that steroid treatment and 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.
  • Occupational therapy, physiotherapy and sometimes speech and language therapy is important.

Inheritance patterns and prenatal diagnosis

Inheritance patterns

Not applicable

Prenatal diagnosis

Not applicable

Is there support?

Sydenham’s Chorea Association

Email: info@sydenhamschorea.org.uk
Website: sydenhamschorea.org.uk

The Association are a Registered Charity in Scotland no. SCO047005. They are a small group of parents of children affected by Sydenham’s chorea, and professionals previously or currently involved in the care of affected children.  This is a rare condition that many health care professionals have little awareness of, so many families have a frustrating experience of delayed diagnosis and lack of support.  They offer direct one to one family support but also seek to increase awareness and understanding of the condition, which in some cases causes problems through into adult life. As far as they are aware, they are the only support group for Sydenham’s chorea in the world. They have a website as well as a Facebook page.  They organise fund raising and educational events.

Group details added August 2021.

Back to A-Z Conditions