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Byline: Hamit. Ozyurek, Guzide. Turanli, Didem. Aliefendioglu, Turgay. Coskun
Early myoclonic encephalopathy (EME) is a rare malignant epileptic syndrome. The erratic myoclonus with or without focal motor seizures, time of onset before 3 months of age, and suppression-burst (SB) pattern in EEG are accepted as the diagnostic criteria for EME. We report a 40-day-old infant with the diagnosis of non-ketotic hyperglycinemia (NKHG). The infant developed myoclonic and focal tonic seizures on the first day of life. His first sleep EEG recorded after onset of seizure was normal. Because of the diagnosis of NKHG and early developed myoclonic seizure, we thought the infant might be EME, and repeated sleep EEG on admission in which asymmetrical SB pattern was seen. We concluded that the absence of SB pattern in the first EEG recording does not exclude the diagnosis of EME, but repetition of EEG is necessary to demonstrate the presence of SB pattern to meet the diagnostic criteria for EME.
Early epileptic encephalopathy with suppression burst (SB) is a malignant epilepsy syndrome seen during the neonatal and early infantile periods. Early infantile epileptic encephalopathy (EIEE or Ohtahara syndrome) and early myoclonic encephalopathy (EME) are included in this epileptic group.
According to the International Classification of Epilepsies and Epileptic Syndromes (ILAE, 1989), EME is categorized as age-related, generalized symptomatic epilepsies of non-specific etiology. EME is clinically characterized by the onset of erratic or fragmentary myoclonus. Erratic, partial myoclonus usually appears as the first seizure, even as early as a few hours after birth. The myoclonus usually involves the face or extremities. Neurological abnormalities are constant: very severe delay in …