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

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