Get access

Electrophysiological evidence of cerebral dysfunction in childhood opsoclonus-myoclonus syndrome

Authors

  • Hiroshi Sakuma MD,

    Corresponding author
    1. Department of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry, Tokyo, Japan
    • Department of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry, 4-1-1, Ogawa-Higashicho, Kodaira, Tokyo 187 8551, Japan
    Search for more papers by this author
  • Yuko Shimizu MD,

    1. Department of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry, Tokyo, Japan
    Search for more papers by this author
  • Yoshiaki Saito MD,

    1. Department of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry, Tokyo, Japan
    Search for more papers by this author
  • Kenji Sugai MD,

    1. Department of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry, Tokyo, Japan
    Search for more papers by this author
  • Masumi Inagaki MD,

    1. Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry, Tokyo, Japan
    Search for more papers by this author
  • Makiko Kaga MD,

    1. Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry, Tokyo, Japan
    Search for more papers by this author
  • Masayuki Sasaki MD

    1. Department of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry, Tokyo, Japan
    Search for more papers by this author

  • Potential conflict of interest: None reported.

Abstract

To address cerebral involvement in childhood opsoclonus-myoclonus syndrome (OMS), electrophysiological investigations including electroencephalograms and evoked potentials were performed in three children affected by nonparaneoplastic OMS. Most patients displayed abnormalities in visual- and somatosensory-evoked potentials, consisting of delayed latency or disorganization of cortical components. Symptoms of OMS have been attributed primarily to an abnormality in the cerebellum and its associated pathway. This theory was consistent with cerebellar lesions detected by neuroimaging. Electrophysiological evidence of cerebral dysfunction observed in this study, however, implies additional involvement of the cerebral cortex, which may contribute to the accompanying psychiatric and cognitive disorders. © 2010 Movement Disorder Society

Ancillary