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Opsoclonus-myoclonus syndrome

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Other Names:
Opsoclonus myoclonus syndrome; OMS; Kinsbourne syndrome; Opsoclonus myoclonus syndrome; OMS; Kinsbourne syndrome; Ataxo-opso-myoclonus syndrome; OMA syndrome; Opsoclonus-myoclonus-ataxia syndrome; Dancing eye-dancing feet syndrome; Dancing eye syndrome; POMA syndrome; Paraneoplastic opsoclonus-myoclonus; Paraneoplastic opsoclonus-myoclonus-ataxia syndrome See More
Categories:
Opsoclonus-myoclonus syndrome (OMS) is a rare disorder that affects the nervous system. Symptoms include rapid, multi-directional eye movements (opsoclonus), quick, involuntary muscle jerks (myoclonus), uncoordinated movement (ataxia), irritability, and sleep disturbance. The onset of OMS is usually abrupt and often severe. The disease may become chronic.[1] OMS typically occurs in association with tumors (neuroblastomas), or following a viral or bacterial 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. In some cases, when the underlying cause of OMS is treated, symptoms improve.[1][2][3]
Last updated: 5/11/2017
Signs and symptoms of opsoclonus-myoclonus syndrome (OMS) may include:[2][3]
  • Unsteady, trembling gait (manner of walking)
  • Sudden, brief, shock-like muscle spasms (myoclonus). While it occurs most when trying to move and worsens with agitation or stimulation, it can also be present at rest. Myoclonus can make a person appear nervous or shaky, or have jerking movements. The face, eyelids, limbs, fingers, head and trunk may be involved. When the illness is at its worst, sitting or standing is difficult or impossible.
  • Irregular, rapid eye movements (opsoclonus)
Other symptoms may include difficulty speaking or inability to speak, difficulty eating or sleeping, excessive drooling, lack of coordination, rage attacks, head tilt, a decrease in muscle tone, and/or general feeling of discomfort or illness.[2][3]

Children may appear to be nervous, irritable, or lethargic while adults may have mental clouding (encephalopathy).[3]
Last updated: 5/11/2017
Opsoclonus-myoclonus syndrome is often associated with the presence of a tumor (such as neuroblastoma) and referred to as a paraneoplastic syndrome. In other cases, it is associated with a viral infection (such as influenza, Epstein-Barr, hepatitis C, HIV,or Coxsackie B) or a bacterial infection (such as as streptococcal, Lyme disease, or mycoplasma). In some cases, the cause is unknown.[2][3]
Last updated: 5/11/2017
Opsoclonus-myoclonus syndrome does not appear to be inherited. The disorder is sporadic and occurs in people with no family history of the condition.[4]
Last updated: 5/22/2017
A diagnosis of OMS is mostly based on the presence of the characteristic signs and symptoms. In some cases, laboratory tests for certain antibodies and/or for abnormal white blood cells may also be done.[3]

The diagnosis is based on the presence of any 3 out of the 4 following criteria:
  1. Rare type of cancer that affects the nerve tissue (neuroblastoma)
  2. Uncontrolled eye movement (opsoclonus)
  3. A movement disorder with sudden muscle contractions (myoclonus) and/or lack of coordination (ataxia)
  4. Behavioral 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 helical (or spiral) CT scanning.[4]
Last updated: 5/11/2017
There are no official treatment recommendations for OMS. Management may involve: 

  • Surgery, chemotherapy, and/or radiation: When there is a tumor present, treatment such as surgery for tumor removal, chemotherapy, or radiation may be required.[2] In children, the removal of the neuroblastoma does not always improve neurologic symptoms. Some adults with paraneoplastic OMA do not have more neurologic symptoms with the removal pf the neuroblastoma or after its treatment.
  • Corticosteroids or ACTH (adrenocorticotrophic hormone), human intravenous immunoglobulins (IVIG), or immunosuppressive agents such as immuran, cyclophosphamide, mycophenolate mofetil, and rituximab. In many cases, a combination of medications leads to the best result.[2][3][5] Adults may be less likely to respond to immunological treatment compared with children.[5]
  • Trazodone: Used to treat sleep problems and associated rage attacks.
Last updated: 5/11/2017
Although a person may fully recover from the underlying cause of OMS, many people continue to have some neurological impairment. Full recovery is more common in cases due to infection 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 neurological symptoms may occur with future illness, fever, stress, medication, anesthesia, discontinuation of immunotherapy, or after immunizations. These relapses are common and may occur more than once. In some cases, many years may pass between episodes. Relapses should be treated, preferably with the same type of treatment used initially.[3] 
Last updated: 1/27/2016

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Differential diagnosis includes acute inflammatory cerebellar ataxia that is differentiated from OMS by the type of eye movement (nystagmus), the absence of irritability, and the usually rapid recovery without treatment.
Visit the Orphanet disease page for more information.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Opsoclonus-myoclonus syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Patient Registry

  • The Autoimmune Registry supports research for Opsoclonus-myoclonus syndrome by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.
  • A registry supports research by collecting of information about patients that share something in common, such as being diagnosed with Opsoclonus-myoclonus syndrome. The type of data collected can vary from registry to registry and is based on the goals and purpose of that registry. Some registries collect contact information while others collect more detailed medical information. Learn more about registries.

    Registries for Opsoclonus-myoclonus syndrome:
    OMS Patient Registry
     

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Opsoclonus-myoclonus syndrome. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • I was diagnosed with this disorder, but I don't have a tumor. What else can cause this condition in adults? See answer

  • Is this disease one that runs in a family, and how can a person be tested for it? See answer

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


  1. Pranzatelli MR. Opsoclonus-Myoclonus Syndrome. National Organization for Rare Disorders (NORD). 2009; https://rarediseases.org/rare-diseases/opsoclonus-myoclonus-syndrome/.
  2. NINDS Opsoclonus Myoclonus Information Page. National Institute of Neurological Disorders and Stroke (NINDS). https://www.ninds.nih.gov/Disorders/All-Disorders/Opsoclonus-Myoclonus-Information-Page.
  3. Pranzatelli MR. What is the Opsoclonus-Myoclonus Syndrome?. Opsoclonus-Myoclonus U.S.A. And International web site. http://www.omsusa.org/pranzatelli-Brochure1.htm.
  4. Opsoclonus-myoclonus syndrome. Orphanet. November, 2014; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=EN&Expert=1183.
  5. Dalmau J and Rosenfeld MR. Opsoclonus myoclonus ataxia. UpToDate. May 3 2016; http://www.uptodate.com/contents/opsoclonus-myoclonus-ataxia.