The frequency of atypical features among 196 patients (118 male and 78 female; mean age at diagnosis 7.64 years, range 1.5-14) with benign childhood epilepsy with centrotemporal spikes (BCECTS) was determined by a retrospective chart study in four pediatric neurology outpatient clinics in Israel between the years 1991 and 2008. Mean duration of follow-up was 4.43 years (range 2-11). Comorbidities or presentations included electrical status epilepticus in slow waves sleep (ESES) in 9 (4.6%) patients, Landau-Kleffner syndrome in 4 (2%), BCECTS with frequent refractory seizures in 3 (1.5%), BCECTS with falls in 2 (1%), atypical variant in 1 (0.5%), and oromotor dysfunction in 1 (0.5%). Sixty-one (31%) had ADHD, 43 (21.9%) had specific cognitive deficits, and 23 (11.7%) had behavioral disorders, including aggressiveness, anxiety, depression, and pervasive developmental disorder. [1]

COMMENT. BCECTS is the most prevalent of benign focal epilepsies of childhood, accounting for 15-25% of epilepsy syndromes in children <15 years of age. Age at onset ranges from 3-13 years, with peak incidence at 7-8 years. The male-to-female ratio is 3:2. Hemifacial motor seizures are preceded by drooling and oromotor apraxia (Lombroso, 1967; Roulet, Deonna et al, 1989, 1993), and frequently followed by ipsilateral hand and leg involvement, usually nocturnal. Apart from ESES, an EEG diagnosis manifesting S/W activity for 85% of slow-wave sleep time, and found in 5% of the above series, atypical forms of BCECTS were uncommon. In contrast, comorbidities were frequent, and especially, ADHD, specific cognitive deficits, and behavior disorders.