Benign childhood epilepsy with centrotemporal spikes (BCECTS) is among the most frequent benign focal epilepsies in childhood and accounts for about 15%–25% of all epileptic syndromes in children with ages between 4 and 12 years.1 The annual incidence ranges between 7.1 and 21 per 100,000 in children under the age of 15 years2 with a slight male predominance.3 Absence of neurological and intellectual deficits are part of the definition, even though BCECTS has also been reported in patients with neuroradiologically documented cerebral lesions.4,5 Genetic factors play an important etiological role, as corroborated by the higher incidence of positive family history for epilepsy and focal electroencephalographic (EEG) abnormalities in BCECTS patients.6 Linkage to chromosome 15q14 was pointed out,7 but not confirmed in several studies.8
Age at onset ranges between 4 and 10 years in 90% of patients, with a peak around 7 years. Seizures occur during sleep in 80%–90% of patients, and only while awake in less than 10%. Seizure frequency is usually low with 10% of patients experiencing one seizure only.1 Duration of seizures commonly ranges from 30 seconds up to no more than 2–3 minutes. Major clinical findings include: (i) orofacial motor signs, specially tonic or clonic contractions of one side of the face with a predilection for the labial commissure (contralateral to centrotemporal spikes); (ii) speech arrest; (iii) sialorrhea, a characteristic ictal symptom which may not be related to increased salivation, swallowing disturbance, or both; (iv) somatosensory symptoms, represented by unilateral numbness or paresthesias of the tongue, lips, gums, and inner cheek. Less-frequent ictal manifestations include: (i) generalized seizures which are not infrequently observed in younger children and probably related to rapid generalization of focal seizures and (ii) postictal paresis.9
The benign character of Rolandic spikes was recognized in the 1950s.10,11 Several long-term follow-up studies confirmed the good prognosis,12,13,14,15 and over 90% of cases will achieve remission by age 12 years.16 The prognosis is favorable even for patients whose seizures are difficult to control, and seizures almost always remit spontaneously in adolescence. No differences in seizure frequency, seizure relapse rate, duration of active period of epilepsy, and social adjustments have been found between BCECTS patients receiving an antiepileptic drug (AED) and untreated patients.17 The only predictor of short-term prognosis is the age at BETCS onset: the earlier the onset of seizures, the longer the active period. The occurrence of GTCS seizures after recovery from BCECTS is a rare event, involving approximately 2% of subjects, similar to the incidence in partial seizures.18
The cornerstone of BCECTS diagnosis is the characteristic interictal EEG pattern. Background EEG activity is normal both during wakefulness and sleep. Interictal epileptic discharges and location of spikes include typical sharp/spike-waves ...