Epigraph Vol. 10 Issue 1, Winter 2007-2008

Report from the Commission on Classification and Terminology

Anne Berg
Anne Berg

The early formal efforts in classification resulted in tremendously useful systems for identifying specific diagnostic entities. Two ILAE reports provided separate classifications; the one from 1981 was specifically for seizures (1) whereas the 1989 report addressed types of epilepsy and syndromes (2). Both of these systems have become comfortable old friends. Yet, when we consider the advances that have been made in the years since these reports were issued, it is clear that these older schemes do not reflect much of the new information that is being generated and our expanded understanding of the epilepsies. Many of the concepts used in the structure of the 1989 classification of the epilepsies require revision. For example, “idiopathic” is often taken to mean “genetic” and is often accompanied by the presumption of “benign.” Few would be happy classifying Dravet syndrome along with Childhood Absence Epilepsy. The generalized-focal dichotomy is being relaxed as the roles of specific networks in producing “generalized” seizures are recognized. Perhaps most frustrating from the point of view of genetics, is the assumption that all focal epilepsies that are not part of already identified idiopathic syndromes are presumed “symptomatic.” In fact, all epilepsy is symptomatic of something. Since that report was issued, several new entities have been recognized within the focal epilepsies which have a suspected or demonstrated genetic basis, either because of patterns of familial aggregation or because of demonstrated genetic associations.

The Commission is currently tackling three separate but interrelated projects. The first concerns developing a new computer-based approach for organizing syndromes into clinically and scientifically justifiable groups along multiple relevant dimensions. Careful selection of terminology that is precise, meaningful, and unencumbered by past history is a key component of this activity. Instead of the quasi-taxonomic classification of 1989, the new system will reflect current and well-founded concepts and, as much as possible, eschew presumptions that have not been adequately demonstrated. The second task is to develop a rigorous, objective set of techniques and research standards for identifying potential new syndromes and testing those new syndromes. This will replace the highly descriptive and expert opinion-driven process of the past. Finally, the third task involves the development of a diagnostic manual designed to help physicians apply this information to individual patients.

Classification of epilepsies and seizures has been an area that has engendered great passion. So it is with much caution that the Commission on Classification and Terminology of the ILAE will recommend or institute changes. The intention is to seek comment from the epilepsy community, particularly on the organizational framework, once a prototype is developed. Input from colleagues throughout the community, both formal and informal, will be encouraged and welcome for all three projects.

Commission on Classification and Terminology Members and Liaisons:
Anne Berg (Chair), Jeffrey Buchhalter, Jerome Engel, Tracy Glauser, Douglas Nordli, Perrine Plouin, Philippe Ryvlin, Ingrid Scheffer, Walter von Emde Boas, Samuel Berkovic, Peter Wolf, Solomon Moshé and Martin Brodie.

1. Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised clinical and electrographic classification of epileptic seizures.
Epilepsia 1981;22:489-501.
2. Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes.

Anne Berg
E-mail: atberg@niu.edu

Anne T. Berg, PhD, is Research Professor in the Department of Biological Sciences at Northern Illinois University in Dekalb, Illinois, USA. She is also the Chair of the ILAE Commission on Classification and Terminology.