LAUSR

OBJECTIVE Atypical benign rolandic epilepsy (BRE) is an underrecognized and poorly understood manifestation of a common epileptic syndrome. Most consider it a focal epileptic encephalopathy in which frequent, interictal, centrotemporal spikes lead to negative motor seizures and interfere with motor and sometimes speech and cognitive abilities. We observed focal cortical hypermetabolism on PET in three children with atypical BRE and investigated the spatial and temporal relationship with their centrotemporal spikes. METHODS EEG, MRI and PET were performed clinically in three children with atypical BRE. The frequency and source localization of centrotemporal spikes was determined and compared with the location of maximal metabolic activity on PET. RESULTS Cortical hypermetabolism on thresholded PET t-maps and current density reconstructions of centrotemporal spikes overlapped in each child, in the central sulcus region, the distances between the "centers of maxima" being 2 cm or less. Hypermetabolism was not due to recent seizures or frequent centrotemporal spikes at the time of FDG uptake. SIGNIFICANCE The findings suggest that localized, increased cortical activity, in the region of the EEG focus, underlies the negative clinical manifestations of atypical BRE. Similar findings are reported in the broader group of epileptic encephalopathies associated with electrical status epilepticus in sleep.