Two children, aged 2 and 4 years, with a characteristic onset of benign childhood epilepsy with centrotemporal spikes (BECTS), developed intractable seizures and cognitive decline, as reported from the Cleveland Clinic Foundation, OH. Seizures were repetitive clonic in one and hemifacial clonic in the second patient, and occurred more than 100 times daily. Some nocturnal generalized tonic-clonic seizures also occurred. Ictal EEGs showed focal sharp waves in central midline or centrotemporal regions, with or without evolution to generalized discharges. Interictal epileptiform, focal sharp wave, discharges were activated during drowsiness and sleep. MRIs were normal. Despite trials with five different antiepileptic drugs, including valproic acid (120 mcg/ml), carbamazepine, phenobarbital, phenytoin, ethosuximide, with prednisone and acetazolamide, seizures were uncontrolled. The IQ declined from 139 at 6 years, to 103 at 8 years, when surgery was considered but deferred in one. Seizures resolved spontaneously after 9 years of age in one, and they improved with the ketogenic diet after age 5 years in another. Cognition improved after seizures were controlled, or resolved spontaneously, in both patients. [1]

COMMENT. BECTS is usually a benign disorder, characterized by infrequent seizures, with or without therapy, and spontaneous remission in later childhood. Rarely, seizures may be repetitive and frequent, refractory to medication, and associated with intellectual regression. The authors stress the need for a conservative approach to management, since spontaneous remission can be expected. The role of the antiepileptic medication in the cognitive decline of one of these patients is certainly suspect.