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

Massimo Mastrangelo, Laura Canafoglia, Silvana Franceschetti, C. Oppezzo Chiara, Fabio Mosca, Francesca Menni, Rossella Parini, Claudia Ciano, Vidmer Scaioli, Ferruccio Panzica

Research output: Contribution to journalArticlepeer-review


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
Issue number3
Publication statusPublished - Mar 2008


  • Electroencephalography
  • Epilepsies
  • Metabolic
  • Myoclonic
  • Myoclonus

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health


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