Early myoclonic encephalopathy, or neonatal myoclonic encephalopathy, is a seizure disorder that begins in the neonatal period.67 Seizures consist of:
The EEG demonstrates bursts of spikes, sharp waves, and slow waves separated by suppression of the background activity.
Early myoclonic encephalopathy is associated with a variety of different etiologies, including nonketotic hyperglycinemia.
The seizures are very difficult to treat and affected infants are usually severely impaired neurologically. More than half of them die before 1 year of age.
Reviewed and revised January 2004 by Gregory L. Holmes, MD, Dartmouth Medical School
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