Dancing Eye Syndrome (DES) is so called because of 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 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.
Typically, a previously well child will become irritable and unsteady and have jerky coordination of the arms and legs. The condition worsens over a couple of weeks or so, the child can become unable to walk. The illness can be distinguished from the acute unsteadiness and nystagmus that complicates some viral illnesses such as chicken pox.
The cause of the condition is not known with certainty but it is thought to be an auto-immune condition in which antibodies which are normally directed to repelling infections and foreign material introduced into the body, start acting against the body itself. 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 it is believed that antibodies are directed against areas of the brain concerned with coordination and with personality and learning. In approximately 10 per cent of cases the condition develops as a complication of neuroblastoma, a tumour of childhood which is, in the majority of cases, self-curing.
It has been suggested that it is the immune-based process of rejection of the tumour which acts indiscriminately on parts of the brain which are structurally similar to the tumour that is being destroyed. Despite exhaustive studies, there is no evidence that neuroblastoma is present in the majority of children with DES but it is thought that an immune-based process is present in all. Occasionally, viral infections can trigger the illness.
A very small proportion of affected children recover spontaneously – without medical intervention. However, the majority require medication.
The most useful group of drugs which have been found to be effective are steroids and ACTH, a hormone which stimulates the adrenal glands to produce steroid. Using this medication, children improve comparatively quickly and abnormal movements are usually abolished within a month of starting treatment. The youngsters become happier and abnormal movements are abolished. Unfortunately the majority of children are left with some degree of learning and/or behavioral disability.
Research is now concentrating on exploring the mechanism of the disease, the identification of the immune process, and trial of alternative drugs which regulate immune disorders.
For further information visit www.dancingeyes.org.uk.
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