High-frequency rhythmic cortical myoclonus in a long-surviving patient with nonketotic hypergylcemia

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Abstract

An 11-year-old girl with nonketotic hyperglycinemia who typically presented with a picture of early myoclonic encephalopathy in the neonatal period is presented in this article. Treated early with sodium benzoate and dextromethorphan, she became seizure-free, while myoclonus persisted. During examination, multifocal rhythmic myoclonic jerks in gamma frequency enhanced by motor activity were noted. Coherence analysis of the electroencephalography-electromyography relationship indicated a cortical origin of the myoclonic jerks. Observation of this case suggests that rhythmic cortical myoclonus may represent a late evolution of this rare disorder.

Original languageEnglish
Pages (from-to)321-324
Number of pages4
JournalJournal of Child Neurology
Volume23
Issue number3
DOIs
Publication statusPublished - Mar 2008

Keywords

  • Electroencephalography
  • Epilepsies
  • Metabolic
  • Myoclonic
  • Myoclonus

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health

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