Opsoclonus-myoclonus syndrome

What causes opsoclonus-myoclonus syndrome?

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.

Last updated on 05-01-20

How is opsoclonus-myoclonus syndrome diagnosed?

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.

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.

Last updated on 05-01-20

Is opsoclonus-myoclonus syndrome inherited?

Opsoclonus-myoclonus syndrome does not appear to be inherited. The disorder is sporadic and occurs in people with no family history of the condition.

Last updated on 05-01-20

What is opsoclonus-myoclonus syndrome?

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. OMS typically occurs in association with tumors (neuroblastomas), or following a viral or bacterial infection. 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.

Last updated on 05-01-20

Other than a tumor, what can cause opsoclonus myoclonus syndrome in an adult?

About 20-40% of adults with opsoclonus myoclonus syndrome (OPS) are found to have cancer somewhere in their body. In these cases, it is believed the cancer cells trigger (cause) an immune response which ends up mistakingly attacking other non-cancerous cells in the body (paraneoplastic syndrome).

Non-paraneoplastic (non-cancer related) adult onset OPS has been linked to various infections, including Lyme disease, enterovirus, West Nile virus, Epstein Barr virus, HIV, salmonella, cytomegalovirus, and after Rubella vaccination or a streptococcal infection. Similar to paraneoplastic cases, it is believed the virus triggers an abnormal immune response. Although in many cases of non-paraneoplastic OPS a cause can not be found (idiopathic), most medical researchers believe the likely cause is an undetected viral infection triggering the immune system.

Last updated on 05-01-20

What is the long-term outlook for people with opsoclonus-myoclonus syndrome?

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.

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.

Last updated on 05-01-20

How might opsoclonus-myoclonus syndrome be treated?

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. 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. Adults may be less likely to respond to immunological treatment compared with children.
  • Trazodone: Used to treat sleep problems and associated rage attacks.

Last updated on 05-01-20

Name: International Parkinson and Movement Disorder Society 555 East Wells Street, Suite 1100
Milwaukee, WI, 53202-3823, United States
Phone: +1-414-276-2145 Fax : +1-414-276-3349 Email: info@movementdisorders.org Url: https://www.movementdisorders.org/
Name: Dancing Eye Syndrome Support Trust 78 Quantock Road Worthing
West Sussex BN13 2HQ
United Kingdom
Phone: 01925 654654 Email: support@dancingeyes.org.uk Url: http://www.dancingeyes.org.uk
Name: The Opsoclonus Myoclonus Syndrome Support Network, Inc. 2116 Casa Linda Drive
West Covina, CA, 91791, United States
Phone: 626-315-8125 Email: sandragreenberg@hotmail.com
Name: OMSLife Foundation P.O. BOX 2899
Cypress, TX, 77410, United States
Email: Mike@Omslife.Org Url: http://www.omslifefoundation.org Contact form: http://www.omslifefoundation.org/?q=en/contacts

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