Abstract
In a neonate who had seizures in the second day of life, the EEG showed - during waking and sleep alike - periodic bursts of asymmetrical high voltage on a low amplitude background tracing. From the third week head jerks occurred, followed by repetitive spasms and then adversive tonic seizures. A severe psychomotor retardation was noted. A CT examination demonstrated a right hemispheric hypertrophy. The pathological examination revealed astrocytic gliosis of the cortex associated with a decrease in the oxidative enzyme activity of neurons, and characterized electron microscopically, by the presence of spheroid bodies in the neuropile. This case was different from those published by Aicardi and Goutières (encéphalopathie myoclonique néo-natale) because there was no erratic myoclonus. It is more similar to the 'early infantile epileptic encephalopathy with suppression bursts' described by Ohtahara. This electro-clinical condition should be characterized as the earliest type of secondary generalized epilepsy.
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