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[Single photon emission tomography (SPECT) in severe infantile myoclonic epilepsy (EMS)]
1Unidad de Neuropediatria, Hospital Universitario Virgen del Rocio, Sevilla.
Insights
Severe myoclonic epilepsy (SME) in children may stem from multifocal origins. SPECT scans reveal hypoperfusion in affected brain regions, suggesting secondary structural irregularities contribute to this epilepsy type.
Area of Science:
- Neurology
- Pediatric Neurology
- Neuroimaging
Background:
- Severe myoclonic epilepsy (SME) is a significant pediatric neurological disorder.
- Understanding the underlying pathophysiology of SME is crucial for effective treatment strategies.
Purpose of the Study:
- To investigate the utility of single photon emission computerised tomography (SPECT) with HMPAO in diagnosing severe myoclonic epilepsy (SME) in children.
- To correlate SPECT findings with electroencephalogram (EEG) and neuroimaging (CT, MRI) data in pediatric SME cases.
Main Methods:
- Ten children diagnosed with SME underwent SPECT (HMPAO), CT, nuclear MRI, and EEG.
- SPECT findings were analyzed for perfusion abnormalities and compared with EEG and other imaging results.
Main Results:
- CT and nuclear MRI provided no relevant diagnostic data.
- EEG showed generalized discharges in eight out of ten patients, predominantly in the right hemisphere in five.
- SPECT revealed hypoperfusion in localized areas (unilateral or bilateral frontal, temporal, and/or parietal regions) in eight out of ten children, particularly those over two years of age.
Conclusions:
- SPECT abnormalities, especially in children over two years old, suggest that SME may be a multifocal epilepsy.
- Secondary structural irregularities appear to play a role in the development of SME.
- SPECT imaging holds potential for identifying regional cerebral blood flow deficits in pediatric SME.
Abstract:
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.