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Cortical myoclonus in children.

Katsuhiko Oguro1, Keiko Oya, Chieko Natori

  • 1Division of Child Neurology, Shizuoka Children's Hospital, Urushiyama 860, Shizuoka-shi 420-8660, Japan. ogurok@mx1.s-cnet.ne.jp

Brain & Development
|April 12, 2003
PubMed
Summary

Cortical myoclonus in children presents diverse etiologies and physiological features. Imaging and electrophysiological studies, including EEG backaveraging and TMS, are crucial for diagnosis, especially when routine EEG is inconclusive.

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Area of Science:

  • Pediatric Neurology
  • Clinical Neurophysiology
  • Neuroimaging

Background:

  • Cortical myoclonus is a distinct electrophysiological condition affecting children and adults.
  • Stimulus-sensitive jerks and giant somatosensory-evoked potentials (SEPs) are characteristic, though imaging abnormalities are often subtle.
  • Limited reports on childhood cortical myoclonus exist in Japan, possibly due to unfamiliarity with specific diagnostic techniques.

Purpose of the Study:

  • To describe the clinical and physiological characteristics of cortical myoclonus in ten pediatric patients.
  • To highlight the utility of advanced electrophysiological techniques in diagnosing cortical myoclonus.
  • To investigate the relationship between lesion location and myoclonus generation.

Main Methods:

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  • Routine EEG, EEG backaveraging, SEP measurement, CT/MRI, and transcranial magnetic stimulation (TMS).
  • Analysis of clinical presentation, electrophysiological findings, and neuroimaging results.
  • Correlation of lesion sites with sensorimotor cortex involvement.

    • Main Results:

    • All ten patients showed clear evidence of cortical myoclonus.
    • EEG backaveraging was essential in six patients lacking spikes on routine EEG.
    • TMS supported a cortical origin in four patients; diverse etiologies were noted, with cerebrovascular disease most common.
    • Lesions adjacent to the sensorimotor cortex were found in nine patients, suggesting nearby cortical neurons contribute to myoclonus.

    Conclusions:

    • Cortical myoclonus in children has varied causes and presentations.
    • EEG backaveraging and TMS are valuable tools for diagnosing cortical myoclonus, particularly when routine EEG is normal.
    • Lesions near the sensorimotor cortex, rather than within it, are implicated in the generation of focal myoclonus.