[Single photon emission tomography (SPECT) in severe infantile myoclonic epilepsy (EMS)].

Ten children, three boys and seven girls, with an average age of 7.5 years diagnosed as having severe myoclonic epilepsy (SME) underwent single photon emission computerised tomography (SPECT) with HMPAO. All had CT, nuclear MR and various EEG studies, these findings made at the same time as SPECT. CT and nuclear MR produced no relevant data. EEG, although without paroxystic abnormalities in two cases, showed generalised discharge in the remaining eight, predominantly in the right hemisphere in five. SPECT was normal in two cases (of 20 and 30 months) and showed areas of hypoperfusion localised in one hemisphere in five cases, three in the left and two in the right, and in both hemispheres in three cases. Areas of hypoperfusion were located in frontal and/or temporal and/or parietal regions. In two cases there was concordance with the prevalence of EEG paroxystic activity and in two other cases there was discordance. Our findings, with SPECT abnormalities after two years of age, suggest SME could be considered as a multifocal epilepsy brought on by secondary structural irregularities.