Early myoclonic encephalopathy (EME) is a rare malignant epileptic syndrome. The erratic myoclonus with or without focal motor seizures, onset before 3 months of age, and persistent suppression-burst pattern in electroencephalograph (EEG) are accepted as the diagnostic criteria for EME. We report an 11 month old infant with EME which was secondary to non-ketotic hyperglycinemia.

Effect of ephedrine in ketotic hypoglycaemia. The effect of oral ephedrine administration on a child with ketotic hypoglycaemia who had not responded to dietary measures alone was studied. After ephedrine administration, the hypoglycaemia developing after ketogenic stress was less profound and was substantially delayed. The impaired glycaemic response to glucagon during hypoglycaemia, character...