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Epilepsia. 2012 Sep;53 Suppl 4:9-18. doi: 10.1111/j.1528-1167.2012.03609.x.

Benign childhood focal epilepsies.

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1
Pediatric Neurology Unit and Laboratories, Children's Hospital A. Meyer-University of Florence, Florence, Italy. r.guerrini@meyer.it

Abstract

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.

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