18FDG-PET in epilepsies of infantile onset with pharmacoresistant generalised tonic-clonic seizures.

AIMS: To investigate the pathophysiology of pharmacoresistant epilepsies with cryptogenic generalised tonic-clonic seizures (GTCS) from infancy.

METHODS: 18F-Deoxy-Glucose-Positron Emission Tomography 18FDG-PET) with statistical parametric mapping (SPM). Inclusion criteria were: pharmacoresistant chronic epilepsy with GTCS commencing in infancy, no focal seizures except alternating hemiconvulsions and no focal epileptic discharges in the EEG during the first year of the disease, no focal changes upon routine neuroradiological investigations, no indication of brain damage according to history and clinical examination.

RESULTS: 15 boys and 15 girls with a mean age of 6.4 years (range l-14 years) were included. All still suffered from seizures despite past treatment with a mean of five drugs. Nearly all were mentally retarded, 19 to a severe and 10 to a minor degree. Fifteen were ataxic and 11 hypotonic. The EEG in 23 showed irregular generalised spike-wave discharges. PET SPM analysis revealed bioccipital hypometabolism related to sedation. Pathological monofocal hypometabolic areas were found in three, multifocal hypometabolic areas in 22 and diffuse bilateral hypometabolism in three patients. Frontal hypometabolism correlated to the degree of mental retardation, hypotonia, and ataxia. Temporomesial hypometabolism correlated to the occurrence of obtunded states and prominent delta rhythms in the EEG. Central and parietal changes were associated with the occurrence of myoclonic seizures and spike-wave discharges.

CONCLUSIONS: 18FDG-PET in many of these children with cryptogenic generalised epilepsies showed multifocal hypometabolic areas of unknown aetiology. Primary cortical microdysgenesis and secondary changes due to the severe and long-standing epilepsy must be considered. Only a minority of patients showed restricted focal hypometabolism as a possible indication for surgical treatment.