PEDIATRIC EPILEPSY SYNDROMES - AN UPDATE AND CRITICAL-REVIEW

Epilepsy syndromes occupy an important position in the current nosolog y of the epilepsies, describing and classifying seizure disorders with shared clinical and EEG features. Increasingly, this schema is being refined as new information becomes available and our understanding of etiology and presentation of each syndrome widens. Advances in neuroim aging and neurogenetics have been particularly important and are likel y to fundamentally change our concepts of syndrome classification. At present, the International League Against Epilepsy classification of e pilepsy syndromes according to presumed localization (partial, general ized, undetermined) and etiology (idiopathic, cryptogenic, symptomatic ). In clinical practice, it is often useful to conceptualize epilepsy syndromes according to their usual age at presentation, which greatly facilitates syndrome identification in new patients and recognizes the age-related expression of many childhood epilepsies. Definitional pro blems exist for many pediatric epilepsy syndromes, particularly the ep ileptic encephalopathies of early infancy, the benign epilepsies of in fancy and childhood, the myoclonic epilepsies of infancy and early chi ldhood, and the idiopathic generalized epilepsies of childhood and ado lescence. It is likely that further input from the fields of molecular genetics and neuroimaging will enable the classification of epilepsie s to become more etiologically oriented and disease specific.