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Dancing Eye syndrome

Also known as: Kinsbourne syndrome, Myoclonic Encephalopathy, Opsoclonus Myoclonus syndrome, Opsoclonus-myoclonus-ataxia syndrome

Background

Dancing eye syndrome (DES) or Opsoclonus myoclonus syndrome (OMS) is a very rare neurological condition which usually develops in the second or third year of life. The syndrome gets its name from the very unusual and characteristic eye movements, which are almost always present.

Credits

Medical text written August 1996 by Dr J Wilson, Honorary Consultant Paediatric Neurologist, Great Ormond Street Hospital, London, UK. Last updated February 2017 by Dr M Pike, Honorary Consultant Paediatric Neurologist, Children's Hospital, Oxford, 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 is provided is for education/information purposes and is not designed to replace medical advice by a qualified medical professional.

What are the symptoms?

The main features of the syndrome are ataxia (unsteadiness), myoclonus (jerky movements of the trunk and limbs), opsoclonus (rapid involuntary eye movements in all directions) and usually irritability with sleep disturbance. As with many other conditions, there is considerable variation in severity.

Most children develop the condition in the second or third year of life, but rarely it may occur earlier or later in childhood. There is also an adult variant of the condition.

What are the causes?

In children, the condition may occur with no clear trigger, or it may follow a viral illness. In about 50% of cases, it may be associated with a tumour called a neuroblastoma. Whenever DES/OMS is diagnosed, it is therefore important that a detailed series of tests are undertaken to make sure that neuroblastoma is not present.

If neuroblastoma is present it is usually, but not always, a benign (non-cancerous) variant of the tumour. Treatment of the neuroblastoma, whilst important in its own right, does not appear to alter the outcome of DES/OMS.

Because of the association with preceding infection and with neuroblastoma and the frequent response of the condition to treatments which modify the immune system (immunomodulatory therapies), it is thought likely that DES/OMS is the result of an immune or allergic cross-reaction in the brain, though there is no definite proof of this to date.

How is it diagnosed?

The diagnosis is based on the four characteristic clinical features:

  • eye movements (which are sometimes fleeting and difficult to spot)
  • unsteadiness and jerkiness
  • irritability and sleep disturbance
  • neuroblastoma.

The criteria for making a diagnosis have been formulated as being at least three of these four features in a child. Because it is very rare, many doctors are unfamiliar with the condition and the diagnosis can sometimes be delayed.

How is it treated?

The treatments used for DES/OMS are those used to suppress the immune response. Steroids given by mouth or injection have been the main drugs used as they dampen down the effect of the immune system. Other treatments which modify the immune system may also be used; these include cyclophosphamide, rituximab, intravenous immunoglobulin and azathioprine. Large-scale research trials in Europe and the USA are aimed at improving the treatment options.

The longer term outcome of this condition is very variable. A very few children fully recover very quickly without treatment. Others respond to treatment partially or completely. More severely affected children often have longer-term problems with learning, coordination, behaviour and sleep which need careful assessment and treatment in their own right.

Inheritance patterns and prenatal diagnosis

Inheritance patterns
There is no indication that the condition runs in families although immune-related conditions, such as thyroid disease and rheumatoid arthritis, are more common in families with a child affected by DES/OMS.

Prenatal diagnosis
Not applicable.

Is there support?

Dancing Eye Syndrome Support Trust

Tel: 01793 493131 or 07434 304291
Email: support@dancingeyes.org.uk
www.dancingeyes.org.uk

The Trust is a Registered Charity in England and Wales No. 1060181, established in 1988. It offers information, telephone support and the opportunity to meet other families.

Group details last confirmed February 2017.

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