Late-onset asymmetric myoclonus: an emerging syndrome

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.

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.

This report expands on previous phenotypical descriptions attempting to further develop and refine this possible diagnostic entity.