Opsoclonus-myoclonus-ataxia syndrome (OMAS) is the most common type of paraneoplastic neurologic syndrome (PNS) in children. The annual incidence of OMAS is about 0.02/105,and the majority of OMAS cases occurs in children under 4 years old, especially in children with neuroblastoma (NB). The pathogenesis of OMAS associated with NB has not yet been fully clarified. The key pathophysiology might be closely associated with inflammatory reaction and resultant damage to neuronal tissues mediated primarily by autoimmune mechanisms, rather than direct tumor infiltration or metastasis. The common clinical manifestations include gait disturbances, ataxia, and myoclonus. The mainstay of management for OMAS associated with NB is the treatment of the primary tumor. This present review focuses on recent advances in the pathogenesis, clinical manifestations, diagnosis and treatment of OMAS associated with NB.
