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Journal of Child Neurology
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Article

High-Frequency Rhythmic Cortical Myoclonus in a Long-Surviving Patient With Nonketotic Hypergylcemia

Massimo Mastrangelo1, Laura Canafoglia2*, Silvana Franceschetti2, Chiara Oppezzo1, Fabio Mosca3, Francesca Menni4, Rossella Parini5, Claudia Ciano2, Vidmer Scaioli2, and Ferruccio Panzica2

1 V. Buzzi Hospital, A.O. ICP, Milan, Italy
2 IRCCS Foundation, Carlo Besta Neurological Institute, Milan, Italy
3 IRCCS Foundation Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena Unive
4 IRCCS Foundation Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena University of Milan, Italy
5 S. Gerardo Hospital, Monza, Italy

* To whom correspondence should be addressed. E-mail: canafoglia{at}istituto-besta.it.


  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.

First published on January 8, 2008, doi:10.1177/0883073807308699

Journal of Child Neurology 2008;23:321.

A more recent version of this article appeared on March 1, 2008

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