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

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.

• Authors: Massimo Mastrangelo, Laura Canafoglia, Silvana Franceschetti, Chiara Oppezzo, Fabio Mosca, Francesca Menni, Rossella Parini, Claudia Ciano, Vidmer Scaioli, Ferruccio Panzica
• Journal: Journal of child neurology (J Child Neurol) Vol. 23 Issue 3 Pg. 321-4 (Mar 2008) ISSN: 0883-0738 [Print] United States
• PMID: 18182648 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Anticonvulsants
• Dextromethorphan
• Sodium Benzoate

Topics

• Anticonvulsants (therapeutic use)
• Cerebral Cortex (physiopathology)
• Child
• Child, Preschool
• Dextromethorphan (therapeutic use)
• Disease Progression
• Electroencephalography
• Epilepsies, Myoclonic (complications, drug therapy)
• Female
• Humans
• Hyperglycinemia, Nonketotic (complications, drug therapy)
• Infant
• Infant, Newborn
• Magnetic Resonance Imaging
• Movement Disorders (complications, drug therapy)
• Myoclonus (complications, drug therapy)
• Sodium Benzoate (therapeutic use)
• Survivors