Opsoclonus-myoclonus syndrome in adults is a rare and heterogeneous disorder with the clinical features of opsoclonus, myoclonus, ataxia, and behavioral and sleep disturbances. The pathophysiology is thought to be immunological on the basis of paraneoplastic or infectious etiologies. Immunomodulatory therapies should be performed although the response may be incomplete. A number of autoantibodies have been identified against a variety of antigens, but no diagnostic immunological marker has yet been identified. This review focuses on underlying mechanisms of opsoclonus-myoclonus syndrome, including findings that have been identified recently, and provides an update on the clinical features and treatments of this condition.
Keywords: Antibodies; Autoimmune; Encephalitis; Opsoclonus–myoclonus syndrome; Paraneoplastic.