EPILESSIA MIOCLONICA SEVERA DELL’INFANZIA: STUDIO DI FOLLOW-UP DI UN CAMPIONE DI SOGGETTI DALLA PRIMA INFANZIA ALL’ETÀ ADULTA.

Severe Myoclonic Epilepsy of Infancy (SMEI) or Dravet syndrome is a rare epileptic encephalopathy with onset within the first year of life manifesting in 1:30.000 subjects. Epileptic manifestations are associated to progressive neurocognitive deterioration and verbal speech impairment. The epileptic history and semiology, the electroencephalographic pattern, the genotype and pharmacological treatment of a group of six Italian pazients with SMEI were retrospectuvely analyzed.