Dancing Eye Syndrome (DES; also known as Ospoclonus Myoclonus Syndrome, OMS) is a rare condition characterised by unusual eye movements, which are almost always present, along with unsteadiness, jerky movements of the trunk and limbs, irritability and sleep disturbance. The condition mostly affects children (in their second or third year of life), though occasionally infants and adults are affected. It was first described in the 1950s by Kinsbourne and Sandifer, neurologists at Great Ormond Street Children’s Hospital, London.
Typically, a previously-well child will become irritable and unsteady, with jerky coordination of the arms and legs. The condition worsens over a couple of weeks or so, in which time the child can become unable to walk. The individual may also develop speech problems. The illness can be distinguished from the acute unsteadiness and nystagmus that complicates some viral illnesses such as chicken pox.
This condition is accompanied by eye movements which are chaotic and best described as “dancing”. They are one of the later symptoms to develop. The jerky eye movements correspond with jerky movements which develop in the limbs, particularly the arms. These movements give Dancing Eye Syndrome its other name of ‘opsoclonus-myoclonus’.
The cause of dancing eye syndrome is not known with certainty but it is thought to be an auto-immune condition. In autoimmune conditions, antibodies which are normally directed to repelling infections and foreign material introduced into the body, start acting against the body itself causing harm. It is believed that antibodies are directed against the area of the brain known as the cerebellum, which is concerned with coordination, personality and learning. In some cases, the condition develops as a complication of neuroblastoma, a frequently benign tumour which develops from nerve cells which are immature (not fully developed). Occasionally, viral infections can trigger the illness. In other cases, no cause for the condition can be found.
A diagnosis of dancing eye syndrome is made when typical symptoms of the condition are present and the following diagnostic criteria are met (at least 3 out of 4 present).
1. Presence of neuroblastoma (type of cancer affecting immature nerve cells)
2. Uncontrolled eye movement (known as opsoclonus)
3. Movement disorder with sudden muscle contractions (myoclonus) and or lack of coordination (ataxia)
4. Behavioural or sleep disturbance
The individual may also have laboratory tests done to check for certain antibodies or abnormal white blood cells linked with the condition.
A very small proportion of affected children recover spontaneously – without medical intervention. However, most will require some form of treatment. Some individuals will experience a partial recovery, whilst others will fully recover. In a large percentage of cases, this condition will follow a relapsing disease course and will require further periods of treatment.
In cases where a neuroblastoma is present, the individual may undergo surgery, chemotherapy, radiation or some combination of these interventions.
There are different types of medications available to target the immune system. A group of medicines known as immunosuppressive agents can be used to dampen down the activity of the immune system. Examples include azathioprine, cyclophosphamide and rituximab. 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 behavioural disability.
General eye check-ups are important for people with Dancing Eye Syndrome, as these individuals may still be at risk of developing other kinds of eye problems that affect the general population, some of which may be treatable.
No matter what level of vision a person may have, it is important to look after the eyes. To find out more about what can be done to take care of the eyes on a daily basis, please visit our Tips for Good Eye Health.
Researchers are seeking improved treatment methods to target dancing eye syndrome. A multinational European clinical trial is now underway to examine a new treatment escalation process for this condition. This process will involve a steroid treatment used solely, before progression to a combination of a steroid and immunosuppressive agent treatment if no pre-planned improvements are observed.
Information about clinical trials can be found on the clinical trials website and can be searched by condition and trial location.
Receiving a diagnosis can be overwhelming for anyone, but this is not a journey that you have to make alone. There are many groups and resources available to provide support for people living with Dancing Eye Syndrome.
Fighting Blindness offers a free and confidential counselling service (Insight Counselling). For further information please contact insight@fightingblindness.ie or call 01 6746496.
A mindfulness group is also available on every Wednesday at the Fighting Blindness office at 11am.
For technology support and guidance, the Dublin-based Technology Exchange Club meets every Monday at the Fighting Blindness office at 11am. Another Technology Exchange Club, based in Cork, meet every Saturday in the Cork City Library, Grand Parade, Cork City at 11am. The Cork-based club do not meet on Bank Holiday weekends or on the second Saturday of the month. For further information please contact insight@fightingblindness.ie or call 01 6746496.
Féach provides support for parents of children living with sight loss in Ireland.
ChildVision is the national education centre for children with sight loss in Ireland.
NCBI (National Council for the Blind in Ireland) provides support and services for people living with sight loss in Ireland.
Irish Guide Dogs for the blind helps individuals and their families to achieve improved mobility and independence.
Dancing Eye Syndrome Support Trust provide families with information and support.