About Dancing Eye Syndrome

The Dancing Eye Syndrome (DES) is so called because of the very abnormal eye movements which are almost invariably present in this rare condition.

Unlike the oscillating movements of nystagmus, eye movements are chaotic and best described as dancing. They are jerky and correspond with jerky movements which develop in the limbs particularly the arms. These movements give the Dancing Eye Syndrome its other name of opsoclonus-myoclonus, referring respectively to the jerky eye and limb movements. DES was first described in the 1950s by Kinsbourne and Sandifer, Neurologists at Great Ormond Street Children’s Hospital, London. The condition mostly affects children though occasionally infants and adults are also affected.

In the United Kingdom it is probable that there are between 5 and 10 newly affected children recognised each year.

Typically, a previously well child will become irritable and unsteady and have jerky in coordination of the arms and legs. The condition worsens over a couple of weeks or so, the child becoming more miserable and ultimately unable to walk. The illness can be distinguished from the acute unsteadiness and nystagmus that complicates some viral illnesses such as chicken pox. In DES the children are extremely unhappy

What is the cause of the condition?

he precise reason why this happens is unknown but we now recognise this as a condition whereby the immune system is activated, termed an auto-immune disorder. Immune cells or antibodies that are triggered by the presence of a tumour or infection. Somehow,  the natural immune response to destroy a tumour or virus, becomes overactive and acts indiscriminately to start to attack the body.

Other auto-immune diseases include, for example, rheumatoid arthritis, and some cases of inflammation of nerves and muscles, the character of the illness depending upon what tissues are attacked.

In the case of DES, the immune system comprising of immune cells and antibodies, are directed against areas of the brain concerned with motor function, eye movement and coordination. Often the child also has their personality and learning severely affected by this immune attack. 

Approximately 50 per cent of cases the condition develops as a complication of neuroblastoma, a tumour of childhood which is, in the majority of cases, is easily treated. Despite exhaustive investigations, there is no evidence that neuroblastoma is present in the 50% of children with DES.