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Myoclonus is defined as a sudden, brief, lightning-like involuntary movement that is caused by muscle contraction (positive myoclonus) or sudden inhibition of muscle contraction (negative myoclonus). It presents a challenging picture for several reasons. (1) There is a vast array of clinical presentations, which makes even the identification of myoclonus difficult. (2) Myoclonus is a clinical symptom, for which there are numerous causes. (3) There are multiple pathophysiologic mechanisms underlying myoclonus. For these reasons, there are several classification schemes and the differential diagnosis is broad. Further, treatment of myoclonus is hampered by a scarcity of large-scale randomized studies. As such, it is difficult to identify a unified and comprehensive approach to clinical evaluation and treatment of myoclonus. Although there is presently no consensus as to the ideal method of classifying or clinically approaching myoclonus, the various classification schemes each incorporate important aspects of myoclonus with the goal of devising an optimal treatment strategy.
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