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Definition

A severe form of epilepsy characterized by frequent tonic seizures or spasms beginning in infancy with a specific EEG finding of suppression-burst patterns, characterized by high-voltage bursts alternating with almost flat suppression phases. Affected individuals have neonatal or infantile onset of seizures, profound mental retardation, and MRI evidence of brain hypomyelination.

Acronym

DEE4

Synonyms

Early myoclonic encephalopathy
EIEE4
EME
Epileptic encephalopathy, early infantile, 4
Neonatal epilepsy with suppression-burst pattern

Keywords

Epilepsy
Mental retardation

 
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