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Keywords:

  • myoclonus-dystonia;
  • primary dystonia;
  • clinical neurology;
  • epsilon-sarcoglycan;
  • genetics;
  • pathophysiology

Abstract

Our knowledge of the clinical, neurophysiological, and genetic aspects of myoclonus-dystonia (M-D) has improved markedly in the recent years. Basic research has provided new insights into the complex dysfunctions involved in the pathogenesis of M-D. On the basis of a comprehensive literature search, this review summarizes current knowledge on M-D, with a focus on recent findings. We also propose modified diagnostic criteria and recommendationsfor clinical management. © 2008 Movement Disorder Society