THE BENIGN FOCAL EPILEPSIES OF CHILDHOOD. HOW MANY SYNDROMES AND THEIR CLINICAL SIGNIFICANCE?

Objective: According to the 'Proposed diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE task force on classification and terminology', Epilepsia 2001;42:796–803, the key term benign has the following definition: benign epilepsy syndrome is a syndrome characterized by epileptic seizures that are easily treated, or require no treatment, and remit without sequelae. However, most of the benign focal epilepsies of childhood are not really benign, and thus, these should not be prognostically neglected.

Methods and Results: After reviewing recent literature, the following 'benign' focal epilepsies will be discussed:

Benign familial neonatal seizures (also non-familial); Benign familial infantile seizures (also non-familial); Benign familial neonatal-infantile seizures; Early-onset benign childhood occipital epilepsy (Panayiotopoulos syndrome); Late-onset childhood occipital epilepsy (Gastaut syndrome); Rolandic epilepsy; Autosomal dominant nocturnal frontal lobe epilepsy.

Conclusion: Knowledge about the mentioned syndromes is of importance in the differential diagnosis from other epilepsies in corresponding age groups. It is of importance to know that cognitive dysfunction may occur in most of these syndromes. Febrile seizures and epilepsy may appear years after the onset of the neonatal seizure syndrome. Some of the syndromes are genetically mapped, many of them representing channelopathies. This is of importance for improved genetic councelling, and for further investigation and understanding of other familial/non-familial epilepsies.

Keywords: Children, focal epilepsies, genetics