Early myoclonic encephalopathy is an epileptic encephalopathy syndrome with onset either in the neonatal period or within the first 3 months of life, characterized by erratic, fragmentary, or massive myoclonus, focal seizures, and late tonic spasms. The prognosis is severe. Early myoclonic encephalopathy and Ohtahara syndrome together are recognized as 2 of the epileptic encephalopathies seen in early infancy and characterized by suppression burst on electroencephalogram.
In this updated article, Dr. Jules Beal of Weill Cornell Medicine and New York-Presbyterian Queens Hospital and Dr. Solomon Moshé of Albert Einstein College of Medicine review the clinical and neurophysiological data, management, and etiologic factors. Early myoclonic encephalopathy is believed to have various prenatal etiologies that often remain unknown; inborn errors of metabolism and genetic disorders are sometimes found.
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