MALIGNANT EPILEPTIC ENCEPHALOPATHIES IN INFANCY AND EARLY CHILDHOOD

B. Rešić, M. Tomasović, I. Valić, M. Meštrović, R. Kuzmanić Šamija, I. Olujić

Specific epileptic syndrome are reviewed in infants and early childhood with common feature. These seizures are age dependente and aetiologicaly heterogenic. Their characteristic are minor generalisation, high frequency and intractability. EEG findings show continous and severe epileptic discharges. Mental defect frequently associated. In these group are included: syndrome West (WS); early infantile epileptic encephalopathy (EIEE); early (neonatal) mioclonic epilepsy (EME); and Lennox-Gastaut syndrome (LGS). WS consist of infantile spasms, mental retardation and hypsarrhythmia. In patients with EIEE seizures occure in early infancy, spasms are mainly tonic and intractable. EEG pattern are "suppresion burst"activity. Severe psychomotor retardation is present. EME caracterize early beginning in the first days of life with eratic fragmentary myoclonus and severe course with death in first year. EEG patterns are "suppresion burst", aetiology is heterogenous. LGS-consist of multiple seizure types including tonic seizures, atypical absences and episodes of tonic and non convulsive status epilepticus with slow spike-waves, spikes and bursts of 10-20 Hz spikes during sleep.