Abstract
Opsoclonus Myoclonus Syndrome (OMS) is a rare condition in children, that for expert consensus is defined to be met three of the four criteria: opsoclonus, ataxia, myoclonus, behavioral disorders or sleep and diagnosis of neuroblastoma. OMS frequently occurs in children associated with neural crest tumors, the most prevalent being neuroblastoma, followed by ganglioneuroblastoma, ganglioneuroma, and hepatoblastoma. The OMS association and neuroblastoma has a peak incidence between 6 and 36 months of age and the possibility that a child with neuroblastoma have a OMS is about 50%. While the OMS is not a common pediatric pathology, its association with neoplastic disease is very strong and ignore its existence and clinical presentation may lead to delays or underdiagnosis of the tumor with negative impact on the prognosis of the entity base and the future neurological patient. Here we present the case of a girl of 15 months with OMS associated with retroperitoneal ganglioneuroblastoma that improved after tumor resection and treatment with corticosteroids and immunoglobulin.
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