Characterization of severe action myoclonus in sialidoses

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To asses the characteristics of severe action myoclonus in three patients with progressive myoclonus epilepsy (PME) due to sialidosis.We assessed EEG-EMG coherence, relative power (RP) and bandwidth (BW) of the EMG-peak associated with myoclonus; we also evaluated somatosensory evoked potentials and long-loop reflexes (LLRs). We compared the findings with those obtained in ten Unverricht-Lundborg (UL) patients.The presentation of sialidosis included macular cherry-red spot, skeletal malformation and polyneuropathy in the infantile form and optic atrophy in the juvenile form. From its onset in adolescence myoclonus rapidly worsened, quickly leading to severe disability. In sialidosis patients, the EMG-peak was characterised by higher RP (p

Original languageEnglish
Pages (from-to)86-93
Number of pages8
JournalEpilepsy Research
Issue number1-2
Publication statusPublished - Mar 2011


  • Cortical rhythmic myoclonus
  • EEG-EMG coherence
  • Progressive myoclonus epilepsy
  • Sialidosis
  • Spectral analysis
  • Unverricht-Lundborg disease

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology


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