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Keywords:

  • myoclonus;
  • cortex;
  • primary;
  • asymmetric;
  • aging

Abstract

Background:

Asymmetric cortical myoclonus is typically thought to be associated with either contralateral cortical structural lesions or degenerative disorders such as corticobasal degeneration when onset is in middle-aged or aged adults. This view has been challenged after a recent case series brought to light a syndrome of senile-onset, asymmetric cortical myoclonus not associated with any such identifiable disorders, thus, named “primary progressive myoclonus of aging.” This is rare and no other reports have been published; hence, further such cases need to be highlighted.

Case reports:

Here, we describe 3 patients with some similarities, namely, adult-onset, asymmetric myoclonus that is most likely to be cortical, with an unremarkable thorough diagnostic workup, but with younger age at onset and longer follow-up time.

Conclusions:

This report expands on previous phenotypical descriptions attempting to further develop and refine this possible diagnostic entity. © 2011 Movement Disorder Society