Opercular epileptic syndrome:: An unusual form of benign partial epilepsy in childhood

Introduction. Functional opercular syndrome in childhood is an exceptional form of presentation of benign partial epilepsy with centro-temporal rolandic spikes (BECRS). Clinical cases. We studied the evolution of four patients, three of them followed for more than 15 years. Two were siblings, and their;rather suffered from BECRS with permanent language problems (verbal dyspraxia) and difficulty of protunding his tongue in adulthood A third patient suffered benign familial neonatal convulsions (BFNC). In all four patients the actual illness begun as a BECRS with opercular troubles as an ictal phenomena. At about four years of age, the opercular disfunction became evident, with severe drooling, facial hypomobility and speech disturbance which waxed and vanished along weeks, months or years, apparently not ictal. Antiepileptic drugs not only were unable to control this situation but also, some of them, like carbamazepine, even worsened the opercular disfunction, increased the number of seizures and enhanced the neuropsychologic disfunction. Only clobazam could achieve the control on opercular disfunction. After 16 years, no further treatment was needed for all patients. There were some permanent sequelae, as speech and orolingual dyspraxia and different neuropsychologic problems. Conclusion. Of noteworth the best performance was attained by the patient treated with clobazam on monotherapy.