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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. DEE3 is characterized by a very early onset, erratic and fragmentary myoclonus, massive myoclonus, partial motor seizures and late tonic spasms. The prognosis is poor, with no effective treatment, and children with the condition either die within 1 to 2 years after birth or survive in a persistent vegetative state.

Acronym

DEE3

Synonyms

Early myoclonic encephalopathy
EIEE3
EME
Epileptic encephalopathy, early infantile, 3
Neonatal epilepsy with suppression-burst pattern

Keywords

Epilepsy

 
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