BENIGN EPILEPSY OF CHILDHOOD WITH CENTROTEMPORAL SPIKES AND UNILATERAL DEVELOPMENTAL OPERCULAR DYSPLASIA

The case of a 15-year-old right-handed girl with developmental delay, mild retardation, astereognosis, and tactile discrimination impairment in the left arm, is reported. During sleep, right partial simple moto r seizures with sialorrhea and saliva pooling were present, sometimes followed by secondary generalization. Absences and drop attacks were a lso observed. Electroencephalography revealed left centrotemporal spik e waves spreading to the homologous contralateral region, generalized 3-Hz spike-and-wave complexes enhanced by hyperventilation, and genera lized polyspike-and-wave discharges during drowsiness. Magnetic resona nce imaging showed right perisylvian (opercular) malformation. Benign epilepsy of childhood with centrotemporal spikes (BECCTS) is a focal i diopathic epilepsy presumed to be of genetic origin. Although brain da mage is not expected, structural lesions including opercular macrogyri a have been reported. This coexistence has been considered mainly casu al and only exceptionally causal. The Foix-Chavany-Marie syndrome or o perculum syndrome is the result of bilateral opercular damage. In our child the right structural and the left functional lesions appeared mu tually activated, yielding a transient opercular syndrome. The concomi tance of BECCTS and developmental opercular dysplasia is suggestive of a common genetic substratum.