Myoclonus, an involuntary movement disorder reveals itself with a wide variety of short muscle twitches or jerks, and may cause severe disability. From a clinical perspective, it is sometimes difficult to discriminate myoclonus from other central movement disorders. Moreover, myoclonus has a spectrum of causes including rare neurological syndromes and uncommon manifestations of systemic disease. Its pathogenesis is only partially understood. Neurophysiologic investigations suggest a close relationship between certain types of myoclonic jerking and epilepsy. The use of anticonvulsants for treatment of myoclonus has its basis in such observations and empirical evidence. Often high doses or a combination of drugs, or both are required, with, however, serious side effects.