Idiopathic focal epilepsies

Abstract

In this chapter we include a series of epilepsies with onset in pediatric age characterized by focal seizures, idiopathic etiology, normal psychomotor development, and a benign course related to the spontaneous remission of seizures without sequelae. These entities are age-dependent and seizures tend to disappear spontaneously. For these reasons often the drug treatment is not necessary. On the basis of genetic assessment idiopathic focal epilepsies can be divided into two groups: nonautosomal dominant and autosomal dominant. In the group of nonautosomal entities we include benign epilepsy with centro-temporal spikes, Panayiotopoulos syndrome, idiopathic childhood occipital epilepsy described by Gastaut, and benign idiopathic midline spikes epilepsy. Seizures are rare, sometimes prolonged, as autonomic status in Panayiotopoulos syndrome. A common feature is the presence of peculiar EEG interictal paroxysmal abnormalities. In the group with an autosomal dominant mode of inheritance we include benign familial infantile seizures and benign familial neonatal-infantile seizures. These entities are characterized by partial seizures in cluster, self-limited in a brief period during the first months of life. There are no typical interictal EEG abnormalities. In some families a mutation in SCN2A, the gene coding for the 2α subunit of the voltage-gated sodium channel, has been described.