AbstractThis review examines some of the advances in understanding myoclonus over the last 25 years. The classification of myoclonus into cortical, brainstem, and spinal forms has been consolidated, each with distinctive clinical characteristics and physiological mechanisms. New genetic causes of myoclonus have been identified, and the molecular basis of several of these conditions has been discovered. It is increasingly apparent that disease of the cerebellum is particularly important in the genesis of cortical reflex myoclonus. However, the precise mechanism and origin of myoclonus in many situations remain uncertain. Effective treatment of myoclonus remains limited, and the challenge lies ahead to develop more therapeutic options. © 2011 Movement Disorder Society