[Early myoclonic epileptic encephalopathy (EMEE) (author's transl)]

The authors describe the electroclinical and evolutive aspects of 4 cases (including 2 brothers) of myoclonic epileptic encephalopathy beginning between 2 days and 10 weeks of life. From the onset of myoclonic jerks, polymorphous fits (partial seizures, tonic seizures) and multifocal electrical abnormalities are associated. Repeated spasms and 'suppression-burst' patterns appear later. The neurological status deteriorates progressively, leading within a few months to decerebration posture with opisthotonus. In spite of thorough neuroradiological, biochemical, cytological to metabolic investigations, etiology remains unknown. However, the electroclinical and evolutive patterns are similar to that of metabolic diseases, especially non-ketotic hyperglycemia. The authors discuss the relations between their observations and those in the literature and the nosological problems of this particular epileptic encephalopathy of infancy.