Late-onset asymmetric myoclonus: An emerging syndrome

Authors

  • Petra Katschnig MD,

    1. Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, University College London, Queen Square, London, United Kingdom
    2. Department of Neurology, Division of Special Neurology, Medical University Graz, Graz, Austria
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  • João Massano MD,

    1. Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, University College London, Queen Square, London, United Kingdom
    2. Department of Neurology, Hospital de S. João, and Faculdade de Medicina da Universidade do Porto, Porto, Portugal
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  • Mark J. Edwards PhD,

    1. Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, University College London, Queen Square, London, United Kingdom
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  • Petra Schwingenschuh MD,

    1. Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, University College London, Queen Square, London, United Kingdom
    2. Department of Neurology, Division of Special Neurology, Medical University Graz, Graz, Austria
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  • Carla Cordivari MD,

    1. Department of Clinical Neurophysiology, National Hospital for Neurology and Neurosurgery, University College London, Queen Square, London, United Kingdom
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  • Kailash P. Bhatia MD

    Corresponding author
    1. Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, University College London, Queen Square, London, United Kingdom
    • Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London, WC1N 3BG UK, Box 13
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  • Relevant conflicts of interest/financial disclosures: Nothing to report.

    This research was supported by National Institutes of Health Grants R01 NS39422, P30 ES09089, P42 ES10349, and R01 CA102484.

    Full financial disclosures and author roles may be found in the online version of this article.

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

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