There is currently increasing interest in identifying and classifying pediatric benign
epilepsy syndromes and recently several new syndromes have been recognized. Benign
epilepsy syndromes, by definition, occur in children with normal developmental history, respond well to
therapy, and remit without sequelae. The large majority of children with benign
epilepsy syndromes follow a truly benign course. The concept of benign
epilepsy syndromes has, however, been challenged by the minority of patients who continue to have
seizures despite
therapy, develop new
seizures after initial remission, or exhibit neuropsychological abnormalities. Without long-term follow-up, benignity can not be truly ascertained a priori. Thus it may be preferable to use the terms possible and probable before the name of a specific syndrome until such time that the diagnosis of a definite benign syndrome is confirmed on long-term follow-up. In this review of the pediatric benign
localization-related epilepsy syndromes, we address the concept of benignity and the process of diagnosis of a benign
epilepsy syndrome. In addition we review the epidemiology, clinical manifestations, EEG findings, work-up, diagnostic criteria, differential diagnosis, genetics, management and prognosis of
benign infantile familial convulsions, benign
partial epilepsy in infancy with
complex partial seizures, benign
partial epilepsy in infancy with secondarily
generalized seizures, benign infantile convulsions associated with mild
gastroenteritis, and benign infantile
focal epilepsy with midline spikes and waves during sleep.