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This version was published on March 1, 2008
Journal of Child Neurology, Vol. 23, No. 3, 321-324 (2008)
DOI: 10.1177/0883073807308699
© 2008 SAGE Publications

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

Massimo Mastrangelo

Pediatric Neurology Unit, V. Buzzi Hospital, A.O. ICP, Milan, Italy

Laura Canafoglia

Department of Neurophysiology and Epileptology, IRCCS Foundation, Carlo Besta Neurological Institute, Milan, Italy, canafoglia@ istituto-besta.it

Silvana Franceschetti

Department of Neurophysiology and Epileptology, IRCCS Foundation, Carlo Besta Neurological Institute, Milan, Italy

Chiara Oppezzo

Pediatric Neurology Unit, V. Buzzi Hospital, A.O. ICP, Milan, Italy

Fabio Mosca

NICU, Institute of Paediatrics and Neonatology, University of Milan

Francesca Menni

Paediatric Clinic, IRCCS Foundation Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena University of Milan, Italy

Rossella Parini

Metabolic Disease Center Fondazione Mariani, Department of Pediatrics, S. Gerardo Hospital, Monza, Italy

Claudia Ciano

Department of Neurophysiology and Epileptology, IRCCS Foundation, Carlo Besta Neurological Institute, Milan, Italy

Vidmer Scaioli

Department of Neurophysiology and Epileptology, IRCCS Foundation, Carlo Besta Neurological Institute, Milan, Italy

Ferruccio Panzica

Department of Neurophysiology and Epileptology, IRCCS Foundation, Carlo Besta Neurological Institute, Milan, Italy

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.

Key Words: myoclonus • epilepsies • myoclonic • metabolic • electroencephalography


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