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Opsoclonus-myoclonus syndrome
Other Resources UpToDate PubMed

Opsoclonus-myoclonus syndrome

Contributors: Jennifer Vermilion MD, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed

Synopsis

Opsoclonus-myoclonus syndrome (OMS), also known as opsoclonus-myoclonus-ataxia syndrome, is a rare disorder characterized by opsoclonus (rapid, involuntary, chaotic, and conjugate eye movements or "dancing eyes"), myoclonic jerks, and ataxia. While it can be seen in any age group, it is more commonly described in the pediatric population. In children, onset is typically before 5 years of age. In addition to motor symptoms, children also often present with developmental regression, irritability, and sleep disturbances.

OMS may be autoimmune, postinfectious, postvaccination, or paraneoplastic in etiology. In adults, OMS can be associated with breast cancer or small cell lung carcinoma. In children, nearly half of those diagnosed with OMS are found to have an occult neuroblastoma. In children with OMS without an associated neuroblastoma, the cause is often not identified.

Disease onset tends to be subacute. In children, the course is often relapsing. The majority of affected children have long-term sequelae, including motor, behavioral, and cognitive deficits. Most adults achieve complete remission with therapy of the underlying cause.

Codes

ICD10CM:
H55.89 – Other irregular eye movements

SNOMEDCT:
230350000 – Opsoclonus-myoclonus syndrome

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

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Therapy

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References

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Last Reviewed:08/27/2018
Last Updated:04/22/2019
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Opsoclonus-myoclonus syndrome
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A medical illustration showing key findings of Opsoclonus-myoclonus syndrome : Nausea, Vomiting, Ataxia, Irritability, Malaise, Myoclonus
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