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Diseases

Genetic and Rare Diseases Information Center (GARD)

Opsoclonus-myoclonus syndrome


Other Names for this Disease
  • Polymyoclonus infantile
  • Infantile polymyoclonus
  • Opsoclonus myoclonus syndrome
  • OMS
  • Kinsbourne syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

I was diagnosed with opsoclonus myoclonus syndrome as a child. Can you provide me with information regarding symptoms, diagnosis, treatment, and prognosis?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is opsoclonus-myoclonus syndrome?

Opsoclonus-myoclonus syndrome (OMS), also known as dancing eyes-dancing feet syndrome, is a rare neurologic disorder characterized by opsoclonus (rapid, multi-directional eye movements), sudden muscle contractions (myoclonic jerks), uncoordinated movement (ataxia), irritability and sleep disturbance. The onset is usually abrupt, often severe, and can become chronic.[1] OMS typically occurs in association with tumors (neuroblastomas), or following a viral infection.[2][3][4] Treatment may include corticosteroids or ACTH (adrenocorticotropic hormone). When there is a tumor present, treatment may include chemotherapy, surgery, and/or radiation. When the underlying cause of OMS is treated, symptoms may improve.[2] Relapses are common and may occur without warning.[2][3]
Last updated: 1/27/2016

What are the signs and symptoms of opsoclonus-myoclonus syndrome?

Signs and symptoms of opsoclonus-myoclonus syndrome may include an unsteady, trembling gait; myoclonus (sudden, brief, shock-like muscle spasms); and opsoclonus (irregular, rapid eye movements).[2] Myoclonus occurs most when trying to move and worsens with agitation or stimulation, but can also be present at rest. An affected person may appear tremulous, or have jerking movements. The face, eyelids, limbs, fingers, head and trunk may be involved. During the peak of the illness, sitting or standing is difficult or impossible.[3] Other symptoms may include difficulty speaking; poorly articulated speech or an inability to speak; difficulty eating or sleeping; excessive drooling; incoordination; rage attacks; head tilt; a decrease in muscle tone; malaise; and/or other abnormalities.[2][3] Children may appear to be nervous, irritable or lethargic while adults may have mental clouding (encephalopathy).[3]
Last updated: 1/27/2016

What causes opsoclonus myoclonus syndrome?

Opsoclonus myoclonus syndrome is generally caused by the presence of a tumor (such as neuroblastoma) or a viral infection (such as influenza, Epstein-Barr, and Coxsackie B). Other causes of this condition are rare.[2][3]
Last updated: 1/27/2016

How is opsoclonus myoclonus syndrome diagnosed?

A diagnosis of opsoclonus myoclonus syndrome (OMS) is mostly based on the presence of the characteristic signs and symptoms of the condition (i.e. it is mainly a clinical diagnosis). In some cases, laboratory tests for certain antibodies and/or for abnormal white blood cells may also be performed.[3]

The diagnosis is clinical, based on the presence of 3 out of the 4 following criteria:

1) Neuroblastoma
2) Uncontrolled eye movement (opsoclonus)
3) A movement disorder with sudden muscle contractions (myoclonus) and/or incoordination (ataxia), and
4) Behavioural and/or sleep disturbance.

In adults with OMS, a blood exam may show Hu anti-neuronal nuclear antibodies (anti-Hu) but not in children. The most efficient methods for detecting a neuroblastoma (which is present in many affected people) are MRI with contrast, and/or helical (or spiral) CT scanning.[4]
Last updated: 1/27/2016

How might opsoclonus myoclonus syndrome be treated?

Treatment for opsoclonus myoclonus syndrome may include corticosteroids or ACTH (adrenocorticotropic hormone), human intravenous immunoglobulins (IVIG), or immunosuppressive agents.[2][3] In cases where there is a tumor present, treatment such as surgery for tumor removal, chemotherapy or radiation may be required.[2] In many cases, a combination of the above yields the best result.

Sleep problems and associated rage attacks may respond to trazodone. Some adults with paraneoplastic OMA do not have more neurologic symptoms when the removing or after treatment the neuroblastoma. Adults may be less likely to respond to immunological treatment compared with children.[5] 
Last updated: 1/27/2016

What is the prognosis for individuals with opsoclonus myoclonus syndrome?

Although individuals may fully recover from the underlying cause of the condition, many individuals retain some level of neurological impairment. Full recovery is more common in cases caused by infections than those due to neuroblastoma. Children with the mildest symptoms have the greatest chance of returning to normal after treatment. For those with moderate severity, myoclonus tends to lessen over time, but coordination may not fully return. Some learning and behavioral issues may also remain. Those with severe symptoms have the highest risk for permanent neurological problems.[3] 

Relapses of the neurological symptoms may occur with subsequent illness, fever, stress, medication, anesthesia, discontinuation of immunotherapy or after immunizations. Such relapses are common and may occur more than once. In some cases, many years may elapse between episodes. Relapses should be treated, preferably with the same type of treatment used initially to obtain better results.[3] 
Last updated: 1/27/2016

References
Other Names for this Disease
  • Polymyoclonus infantile
  • Infantile polymyoclonus
  • Opsoclonus myoclonus syndrome
  • OMS
  • Kinsbourne syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.