Early Infantile Developmental and Epileptic Encephalopathy syndrome (EIDEE)
Posted on
August 16, 2024
Let's learn all the facts about a brain pattern called
Early Infantile Developmental and Epileptic Encephalopathy syndrome (EIDEE)
!
Early Infantile Developmental and Epileptic Encephalopathy syndrome (EIDEE)
Both sexes are affected equally. Abnormalities of tone (most frequently central hypotonia) andposture are present. Movement disorders are common and can be present at seizure onset or appear with age. Most children will have moderate to profound developmental impairment.
Cortical visual impairment, orthopedic problems and feeding difficulties are also seen. Seizures are usually drug-resistant unless a treatable structural, genetic or metabolic cause is found. Children with this syndrome may evolve to have infantile epileptic spasms syndrome or Lennox- Gastaut syndrome.
Common causes include structural brain abnormality (most common), genetic etiologies, metabolic etiologies. A cause can be found in ~80% of cases. The background EEG is abnormal in all states.
Either burst suppression or multifocal epileptiform discharges with diffuse slowing aremandatory. A burst-suppression pattern composes high voltage bursts (150-300uV) of mixed spikes and sharp-waves, lasting 1-5 sec, with an inter-burst interval (IBI) of 3-10 sec when there is marked suppression (<5uV) of the EEG. The ictal EEG varies with the seizure type occurring.