Benign childhood focal epilepsies

Abstract

Summary The idiopathic focal epilepsies comprise a group of syndromes characterized by focal‐onset seizures for which there is no detectable structural brain abnormality and for which there is a proposed functional mechanism for the epilepsy and electroencephalography (EEG) abnormalities. This group includes benign rolandic epilepsy (BRE), benign epilepsy with occipital paroxysms (both early onset and late‐onset types), idiopathic photosensitive occipital lobe epilepsy, and some less well‐defined syndromes. The limits of the early onset idiopathic occipital epilepsy syndrome are not clear, and perhaps this entity represents part of a larger syndrome group of “autonomic” age‐related epilepsies. The term “idiopathic” implies absence of a structural brain lesion and a genetic propensity to seizures. The term “benign” implies that the epileptic seizures are easily treated or require no treatment, show remission without sequelae with ultimate and definitive remission before adulthood, do not have severe or exceedingly disturbing seizures, and have no associated serious intellectual or behavioral disturbances. It may be that a syndrome is benign only when it can be recognized early with reasonable certainty, thereby avoiding unnecessary investigations, overtreatment, and lifestyle restrictions. Although BRE has such characteristic clinical and EEG features to make early recognition possible, this is less constantly so in the other focal idiopathic epilepsy syndromes, where the term “benign” may be inappropriate. Mild and selective neuropsychological impairment may occur even in those with typical syndromes but it is unclear whether such selective deficits outlast the active phase of epilepsy. Sometimes the clinical course may be complicated by obvious cognitive and language impairments. In such cases, the term benign is obviously inappropriate, even when seizures are rare. In most patients with the typical focal idiopathic epilepsy syndromes, medication is not necessary.

Mechanisms Responsible for Cognitive Impairment in Epilepsy

“…Children with Landau -Kleffner syndrome and continuous spike-wave in slow wave sleep (CSWS) have frequent epileptic discharges during sleep and have significant cognitive (especially language) impairments (Van 2013). However, children with benign rolandic epilepsy also have frequent discharges in sleep but their cognitive outcomes are close to normal (Guerrini and Pellacani 2012). Therefore, magnitude of the negative impact of discharges could be small.…”

Altered attention networks in benign childhood epilepsy with centrotemporal spikes (BECTS): A resting-state fMRI study

“…Benign childhood epilepsy with centrotemporal spikes (BECTS) or rolandic epilepsy is the most common childhood epilepsy syndrome, representing 15-25% of all childhood epilepsy cases [1]. The semiology is characterized by brief, simple, partial orofacial motor or sensory seizures during sleep or upon awakening with or without secondary generalization [1], with a typical electroencephalogram (EEG) that shows centrotemporal spikes [2].…”

“…The semiology is characterized by brief, simple, partial orofacial motor or sensory seizures during sleep or upon awakening with or without secondary generalization [1], with a typical electroencephalogram (EEG) that shows centrotemporal spikes [2].…”

Rare variants in γ‐aminobutyric acid type A receptor genes in rolandic epilepsy and related syndromes

“…14,15 The c-aminobutyric acid type A receptor (GABA A -R) is a ligand-gated chloride channel mediating fast inhibitory synaptic transmission in the mammalian brain. It consists of a pentameric assembly of different subunits (a [1][2][3][4][5][6] , b 1-3 , c 1-3 , d, e, p, h, q 1-3 ). The most common synaptic GABA A -R subtype is composed of 2 a 1-3 subunits, 2 b 2-3 subunits, and 1 c 2 subunit encoded by the genes GABRA1-3, GABRB2-3, and GABRG2, respectively.…”