Therapeutic strategy in severe encephalopathies

Introduction. The severe epileptic syndromes of infancy are dependent on age, symptomatic or cryptogenic aetiology and are drug-resistant so that overall prognosis -control of epilepsy and adequate cognition- is poor so that some authors consider it to be catastrophic. All may be identified and differentiated on electroclinical criteria. Development. We study the neonatal myoclonic encephalopathies in the two clinical forms described by Aicardi and Ohtahara, West 's syndrome (infantile spasms),severe myoclonic epilepsy of Dravet-Dalla Bernardina and the Lennox-Gastaut syndrome, briefly describing their electroclinical semiology and therapeutic strategies using the range of available drugs, together with other medical and surgical therapeutic options. Conclusions. Treatment of severe epileptic encephalopathies of childhood is useless/not viable in neonatal myoclonic encephalopathies and severe myoclonic epilepsy of childhood, whilst in the West and Lennox-Gastaut syndromes the results depend on the aetiology, and are better in the cryptogenic types with control of 20% and 30% of the cases but with normal intelligence in only 5% and 9%.