Epileptic encephalopathies in early infancy with suppression-burst.

摘要: Early infantile epileptic encephalopathy with suppression-burst, or Ohtahara syndrome (OS), and early myoclonic (EME) are encephalopathies onset of frequent seizures in the neonatal infancy period a characteristic EEG pattern, namely, which higher-voltage bursts slow waves mixed multifocal spikes alternate isoelectric suppression phase. Their nosologic independence is now widely accepted, although some controversy initially occurred because their common characteristics such as age onset, features, seizure intractability, poor prognosis. Major differences between two syndromes include (1) tonic spasms OS versus partial erratic myoclonias EME, (2) continuous suppression-burst pattern both waking sleeping states this almost limited to sleep (3) static structural brain damage genetic metabolic disorders EME. The most important differentiating point evolutional age, may reflect pathophysiologic difference. evolves West further Lennox-Gastaut but EME demonstrates no unique evolution; it continues for long time changes into epilepsy severe multiple independent spike foci.