ILAE EPILEPSY CLASSIFICATION AND TERMINOLOGY

A PROPOSED DIAGNOSTIC SCHEME FOR PEOPLE WITH EPILEPTIC SEIZURES AND WITH EPILEPSY: REPORT OF THE ILAE TASK FORCE ON CLASSIFICATION AND TERMINOLOGY A major contribution of the International League against Epilepsy was the establishment of standardized classifications and terminology for epileptic seizures and syndromes. This provided a universal vocabulary that not only facilitated communication among clinicians, but also established a taxonomic foundation for the performance of quantitative clinical and basic research on epilepsy. Much, however, has changed since the currently used Classification of Epileptic Seizures was accepted in 1981 (1), and the currently used Classification of Epilepsies and Epileptic Syndromes was accepted in 1989 (2). Consequently, the Executive Committee of the ILAE which took office in July 1997, agreed that review and revision of the current classification system would be a priority for this Executive term. A Task Force on Classification and Terminology was appointed, which divided itself into four working groups concerned with Descriptive Terminology for Ictal Events; Seizures; Syndromes and Diseases; and Impairment (Table 1). During the course of several meetings, and vigorous email discussions, the Task Force agreed that it would not be possible to replace the current international classifications with similar revised and updated classifications that would be universally accepted and meet all the clinical and research needs such a formal organizational system would be expected to provide. Rather, the Task Force is proposing a diagnostic scheme which makes use of standardized terminology and concepts, to describe individual patients (Table 2). Within this diagnostic scheme, a variety of approaches to classification are possible, and some are presented here by way of example only. The Task Force views the development of specific classifications as a continuing work in progress. Flexible and dynamic classifications will be revised periodically based not only on rapidly emerging new information, but also based on the resolution of problems that will inevitably be identified through usage. At this point, the proposal does include several definitive changes in concepts and terminology (Table 3), and classifications are presented as examples of what could be devised in the future. Rationale for the proposal Despite the fact that each new ILAE classification has represented considerable effort on the part of acknowledged experts from many different countries, they have always met with a certain degree of resistance from the international epileptology community. This is due in part to the fact that a rigid classification shapes the manner in which future generations of clinical and basic neuroscientists think about epilepsy and epileptic phenomena, thereby influencing (perhaps unduly) clinical practice and research. For instance, in the current Classification of Epileptic Seizures, the division of partial seizures into "simple" and "complex" inappropriately created the impression that impairment of consciousness had certain mechanistic implications related to limbic system involvement. Confusion, and at times vociferous objections, resulted in part from the fact that the 1970 International Classification of Epileptic Seizures had used the term "complex partial seizures" synonymously with "temporal lobe seizures" (3). Over the past two decades, detailed investigations of the anatomical substrates of ictal semiology, based largely on work carried out in epilepsy surgery centers, has strongly suggested that fundamental mechanisms of certain limbic seizures are different from those of neocortical seizures, and that both can be associated with impairment of consciousness or not. Consequently, the designation of partial seizures as "simple," or "complex," has in the process lost meaningful precision. Indeed, the 1981 Classification of Epileptic Seizures was purposely based purely on ictal
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phenomenology and associated EEG findings rather than anatomical substrates and pathophysiological mechanisms, because insufficient information was available at the time to permit the authors to do otherwise. It is the belief of the Task Force that adequate evidence does now exist to permit creation of a list of seizure types that represent diagnostic entities, as opposed to phenomenological descriptions, based on known or presumed common anatomy and pathophysiology. Such diagnostic entities would, like syndromes, have etiological, therapeutic, and prognostic implications, and could be used to supplement syndromic diagnoses, or stand alone when syndromic diagnoses cannot be made. The 1981 Classification of Epileptic Seizures has also been criticized because it is not purely semiological; post hoc etiological information and EEG data are often required in order to use it properly, and the dichotomy of "partial" vs. "generalized" belies a need to avoid anatomical implications. The Task Force believes that a purely descriptive phenomenological approach to defining ictal semiology has definite clinical value, and the new diagnostic scheme proposed here includes a modification of a previously proposed classification of ictal phenomenology (4), as an option that can be used in detail where appropriate. Similarly, the previous dichotomous classifications based on concepts of "partial" or "localization related" vs. "generalized" abnormalities created the false impression that epileptic seizures, or epilepsy syndromes, were either due to localized disturbances in one hemisphere or disturbances involving the entire brain. In fact, there are a variety of conditons between focal and generalized epileptogenic dysfunctions that include diffuse hemispheric abnormalities, multifocal abnormalities, and bilaterally symmetrical localized abnormalities. Although concepts of partial and generalized epileptogenicity have value and can usually be applied, perhaps more with respect to ictal events than syndromes, it may not be appropriate or useful to attempt to contain all seizures and syndromes within one or the other of these categorizations. The term "partial" itself has come under criticism because it implies part of a seizure, or part of a syndrome, rather than a seizure or syndrome that begins in part of one hemisphere. For this reason, the 1989 Classification of Epilepsies and Epileptic Syndromes replaced the term "partial" with "localization-related." This latter terminology has been cumbersome and is not consistently used. The Task Force is now proposing that the terms "partial" and "localization-related" be replaced with the older term "focal," which remains in common usage. It must be strongly emphasized, however, that the term "focal" does not mean the epileptogenic region is a small, well-delineated focus of neuronal pathology; focal seizures, as well as focal syndromes, are almost always due to diffuse, and at times widespread, areas of cerebral dysfunction. Another change in terminology evident in this document is the omission of the words "convulsion" and "convulsive" in the list of epileptic seizure types, and epilepsy syndromes. The Task Force felt that these are lay terms that are nonspecific, and at times improperly used. Consequently, it was agreed to be consistent, not only in descriptive ictal terminology, but also in naming seizure types and syndromes, to avoid these terms. For instance, the Task Force is proposing that the term "Febrile convulsions" be replaced by "Febrile seizures." There has also been dissatisfaction with the terms "idiopathic" and "cryptogenic." Problems with the former have resulted from misunderstanding of the correct definition of "idiopathic," which means a disorder unto itself, sui generis, and not etiology unknown. Problems with the latter have been due to an imprecision in definition; "cryptogenic" is usually used to designate conditions that are not idiopathic, or are presumed to be symptomatic, when the etiology has not been determined, but it is also used by some for conditions in which it is not known whether they are idiopathic or symptomatic. The Task Force has been unable to find an acceptable alternative

to the term "idiopathic," which, when used correctly, confers a useful taxonomic concept. The terms "benign" and "genetic" were discarded because not all idiopathic epilepsies are necessarily benign, and not all genetic epileptic conditions (e.g., the progressive myoclonus epilepsies) are idiopathic. Although the term "essential" is also used in medicine to convey the same meaning, the Task Force believes that most epileptologists have now learned to use the term "idiopathic" correctly, and that there is value in maintaining continuity. Consequently, it is recommended that the terms "idiopathic" and "symptomatic" be retained, but that the term "cryptogenic," although still acceptable, be replaced by the more precise term "probably symptomatic." Most epilepsy syndromes can be classified as either idiopathic or symptomatic; however, a dichotomous classification system that attempts to categorize all syndromes in this manner has been avoided. Another important criticism of previous rigid syndromic classifications has been a failure to recognize the fact that some syndromes are well accepted, whereas others are controversial, or lack sufficient data. Formally recognizing a syndrome by including it in an official international classification may give it inappropriate legitimacy, while failing to recognize a syndrome in the official classification can discourage studies that are necessary to lead to its acceptance. Any official ILAE-sanctioned list of epilepsy syndromes must differentiate between universally accepted syndromes and those in development, and must also be sufficiently flexible to permit additions and deletions of syndromes as new information becomes available. The rapidly moving field of genetics has contributed greatly in recent years to our understanding of many diseases, including some epileptic disorders, but the relationship between genetic disturbances and phenotypic expression remains complicated and poorly understood. Because a single, relatively well-defined, idiopathic epilepsy syndrome can be due to more than one genetic abnormality, and different members of a family sharing a common genetic abnormality can present with different epilepsy syndromes, it was considered premature to attempt to classify epilepsy syndromes, to any great extent, on the basis of specific genetic etiologies. There is no doubt, however, that in the near future genetic classifications of certain epilepsy syndromes will become possible, and that these classifications will have considerable clinical value. It will be necessary for such classifications to include syndromes based on families, in addition to syndromes of individuals, and indeed the Task Force has included three such conditions in the current recommended list of epilepsy syndromes (c.f. Table 5): Generalized epilepsy with febrile seizures plus, Familial focal epilepsy with variable foci, and Idiopathic generalized epilepsies with variable phenotypes. The first two of these are considered to be syndromes in development, and diagnosis would not be possible without evidence of multiple-affected family members. The third is a new concept which remains under discussion. Description of the proposal The Task Force is asking the General Assembly to approve a diagnostic scheme, rather than a fixed classification, when it next meets in Buenos Aires in May 2001. This diagnostic scheme is intended to provide the basis for a standardized description of individual patients, and consists of five levels, or Axes (Table 2). The Axes are organized to facilitate a logical clinical approach to the development of hypotheses necessary to determine the diagnostic studies that need to be performed, and the therapeutic strategies to be undertaken. The diagnostic scheme described here will be made up of flexible and dynamic modules within which the Task Force will make periodic changes and updates as needed, with the approval of the Executive Committee. The Task Force is proposing that this diagnostic scheme include the development of flexible, rather than rigid, classifications, eliminating the need for the General Assembly, which meets only once every two years, to agree on every revision. Acceptance of

this diagnostic scheme, therefore, does not exclude the creation of various classification systems for seizures and syndromes, or the continued use of some aspects of the current classification. The Task Force will be concerned with the construction of classification systems during the next Executive term, but it is anticipated that seizures and syndromes will not be organized into fixed dichotomous classifications, but rather categorized in various ways for various purposes. Axis 1 consists of a description of the ictal semiology, using a standardized Glossary of Descriptive Terminology. The description of the ictal event, without reference to etiology, anatomy, or mechanisms, can be very brief or extremely detailed, as required for clinical or research purposes. Although detailed descriptions of the onset and evolution of localized ictal phenomena are often not necessary, they can be useful; for instance, in patients who are candidates for surgical treatment, or for research designed to elucidate the anatomical substrates or pathophysiological mechanisms underlying specific clinical behaviors. Communication among clinicians, and among researchers, will be greatly enhanced by the establishment of standardized terminology for describing ictal semiology. Axis 2 is the epileptic seizure type, or types, experienced by the patient, derived from a list of accepted seizure types which represent diagnostic entities with etiologic, therapeutic, and/or prognostic implications. Localization within the brain should be specified when this is appropriate, and in the case of reflex seizures, the specific stimulus will also be specified here. The Task Force has constructed a list of accepted epileptic seizure types, including forms of status epilepticus, and precipitating factors for reflex seizures (Table 4). Seizure types have been divided into self-limited seizures and continuous seizures, and further divided into generalized seizures and focal seizures, but it is anticipated that other approaches to organization, categorization, and classification of seizure types will be devised for specific purposes. Axis 3 is the syndromic diagnosis derived from a list of accepted epilepsy syndromes (Table 5), although it is understood that a syndromic diagnosis may not always be possible. The recommended list distinguishes between epilepsy syndromes and conditions with epileptic seizures that do not require a diagnosis of epilepsy, and also identifies which syndromes are still in development. It is important to stress that the list shown in Table 5 contains syndromes that are still under discussion, such as the new concept of Idiopathic generalized epilepsies with variable phenotypes, and the Reflex epilepsies, and that the Task Force will continue to revise this list based on the results of further deliberations, input from the membership, and new information. As with epileptic seizures, it is anticipated that different approaches to organization, categorization, and classification of epilepsy syndromes will be created for specific purposes. One example of an approach to classification of epilepsy syndromes is shown in Table 6. Whereas this classification system may be easy for epileptologists to understand, a more simplified version will likely be constructed for teaching purposes, and used by primary care physicians, while more detailed, or completely different, classification systems might be necessary for epidemiological studies, clinical drug trials, presurgical evaluation, basic research, and genetic characterizations. Axis 4 will specify etiology when this is known. The etiology could consist of a specific disease derived from a classification of diseases frequently associated with epileptic seizures or syndromes (Table 7), a genetic defect, or a specific pathological substrate, for instance for the symptomatic focal epilepsies. The classification of diseases frequently associated with epileptic seizures shown in Table 7 is preliminary, and will require considerable effort over the course of the next Executive Term to be made as comprehensive as possible.

Axis 5 is an optional designation of the degree of impairment caused by the epileptic condition. Classification of impairment will be derived from the World Health Organization ICIDH-2 International Classification of Functioning and Disability (5), which is currently in preparation. Modification may be necessary for application to seizure disorders. The most recent draft of the Glossary of Descriptive Terminology for Ictal Semiology (Axis 1), detailed descriptions of epileptic seizure types (Axis 2), and epileptic syndromes (Axis 3), and the current draft of the classification of the WHO ICIDH-2 (Axis 5) can be viewed on the ILAE classification website http://www.epilepsy.org/ctf-1. Although the proposal to be put to the ILAE General Assembly in May merely requests approval of the overall diagnostic scheme, with permission to continue to revise and update the details within each Axis in an ongoing, flexible manner, input from our membership now on these details, as well as on the overall scheme, will be most welcome. The Task Force would particularly like to invite comments on some of the more important remaining problems, including terms for describing ictal impairment of consciousness, the acceptance of Idiopathic generalized epilepsies with variable phenotypes as a single syndrome, the inclusion of a category of Epileptic encephalopathies, and the proposed categorization of Reflex seizures and syndromes. Comments for the Task Force can be directed to the chair by e-mail (engel@ucla.edu), mail, or fax (1-310-206-8461). Table 2 PROPOSED DIAGNOSTIC SCHEME FOR PEOPLE WITH EPILEPTIC SEIZURES AND WITH EPILEPSY Epileptic seizures and epilepsy syndromes are to be described and categorized according to a system that utilizes standardized terminology, and that is sufficiently flexible to take into account the following practical and dynamic aspects of epilepsy diagnosis: 1. Some patients cannot be given a recognized syndromic diagnosis. 2. Seizure types and syndromes change as new information is obtained. 3. Complete and detailed descriptions of ictal phenomenology are not always necessary. 4. Multiple classification schemes can, and should, be designed for specific purposes (e.g. communication and teaching; therapeutic trials; epidemiological investigations; selection of surgical candidates; basic research; genetic characterizations). This diagnostic scheme is divided into five parts, or Axes, organized to facilitate a logical clinical approach to the development of hypotheses necessary to determine the diagnostic studies and therapeutic strategies to be undertaken in individual patients: Axis 1: Ictal phenomenology - from the Glossary of Descriptive Ictal Terminology can be used to describe ictal events with any degree of detail needed. Axis 2: Seizure type - from the List of Epileptic Seizures. Localization within the brain and precipitating stimuli for reflex seizures should be specified when appropriate. Axis 3: Syndrome - from the List of Epilepsy Syndromes, with the understanding that a syndromic diagnosis may not always be possible. Axis 4: Etiology - from a Classification of Diseases Frequently Associated with Epileptic Seizures or epilepsy syndromes when possible, genetic defects, or specific pathological substrates for symptomatic focal epilepsies. Axis 5: Impairment - this optional, but often useful, additional diagnostic parameter can be derived from an impairment classification adapted from the WHO ICIDH-2.

Table 3 DEFINTIONS OF KEY TERMS Epileptic Seizure Type: An ictal event believed to represent a unique pathophysiological mechanism and anatomical substrate. This is a diagnostic entity with etiological, therapeutic, and prognostic implications. (new concept) Epilepsy Syndrome: A complex of signs and symptoms that define a unique epilepsy condition with different etiologies. This must involve more than just the seizure type; thus frontal lobe seizures per se, for instance, do not constitute a syndrome. (changed concept) Epilepsy Disease: A pathological condition with a single specific, well-defined etiology. Thus Progressive myoclonus epilepsy is a syndrome, but Unverricht-Lundborg is a disease. (new concept) Epileptic encephalopathy: A condition in which the epileptiform abnormalities themselves are believed to contribute to the progressive disturbance in cerebral function. (new concept) Benign epilepsy syndrome: A syndrome characterized by epileptic seizures that are easily treated, or require no treatment, and remit without sequelae. (clarified concept) Reflex epilepsy syndrome: A syndrome in which all epileptic seizures are precipitated by sensory stimuli. Reflex seizures that occur in focal and generalized epilepsy syndromes that are also associated with spontaneous seizures, are listed as seizure types. Isolated reflex seizures can also occur in situations that do not necessarily require diagnosis of epilepsy. Seizures precipitated by other special circumstances, such as fever or alcohol withdrawal, are not reflex seizures. (changed concept) Focal seizures and syndromes: Replaces the terms partial seizures and localization-related syndromes. (changed concept) Simple and complex partial epileptic seizures: These terms are no longer recommended, nor will they be replaced. Ictal impairment of consciousness will be described when appropriate for the individual seizures, but will not be used to classify specific seizure types. (new concept) Idiopathic epilepsy syndrome: A syndrome that is only epilepsy, with no underlying structural brain lesion or other neurological signs or symptoms. These are presumed to be genetic and are usually age-dependent. (unchanged term) Symptomatic epilepsy syndrome: A syndrome in which the epileptic seizures are the result of one or more identifiable structural lesions of the brain. (unchanged term) Probably symptomatic epilepsy syndrome: Synonymous with, but preferred to, the term cryptogenic, used to define syndromes that are believed to be symptomatic, but no etiology has been identified. (new term) Table 4 EPILEPTIC SEIZURE TYPES AND PRECIPITATING STIMULI FOR REFLEX SEIZURES Self-limited seizure types Generalized seizures Tonic-clonic seizures (includes variations beginning with a clonic or myoclonic phase) Clonic seizures o Without tonic features o With tonic features Typical absence seizures Atypical absence seizures

Myoclonic absence seizures Tonic seizures Spasms Myoclonic seizures Eyelid myoclonia o Without absences o With absences Myoclonic atonic seizures Negative myoclonus Atonic seizures Reflex seizures in generalized epilepsy syndromes Focal seizures Focal sensory seizures o With elementary sensory symptoms (e.g., occipital and parietal lobe seizures) o With experiential sensory symptoms (e.g., temporo parieto occipital junction seizures) Focal motor seizures o With elementary clonic motor signs o With asymmetrical tonic motor seizures (e.g., supplementary motor seizures) o With typical (temporal lobe) automatisms (e.g., mesial temporal lobe seizures) o With hyperkinetic automatisms o With focal negative myoclonus o With inhibitory motor seizures Gelastic seizures Hemiclonic seizures Secondarily generalized seizures Reflex seizures in focal epilepsy syndromes Continuous seizure types Generalized status epilepticus Generalized tonic-clonic status epilepticus Clonic status epilepticus Absence status epilepticus Tonic status epilepticus Myoclonic status epilepticus Focal status epilepticus Epilepsia partialis continua of Kojevnikov Aura continua Limbic status epilepticus (psychomotor status) Hemiconvulsive status with hemiparesis Precipitating stimuli for reflex seizures Visual stimuli o Flickering light -color to be specified when possible o Patterns o Other visual stimuli

Thinking Music Eating Praxis Somatosensory Proprioceptive Reading Hot water Startle Table 5 EPILEPSY SYNDROMES AND RELATED CONDITIONS Benign familial neonatal seizures Early myoclonic encephalopathy Ohtahara syndrome * Migrating partial seizures of infancy West syndrome Benign myoclonic epilepsy in infancy Benign familial infantile seizures Benign infantile seizures (non-familial) Dravet's syndrome HH syndrome * Myoclonic status in nonprogressive encephalopathies Benign childhood epilepsy with centrotemporal spikes Early onset benign childhood occipital epilepsy (Panayiotopoulos type) Late onset childhood occipital epilepsy (Gastaut type) Epilepsy with myoclonic absences Epilepsy with myoclonic-astatic seizures Lennox-Gastaut syndrome Landau-Kleffner syndrome Epilepsy with continuous spike-and-waves during slow-wave sleep (other than LKS) Childhood absence epilepsy Progressive myoclonus epilepsies Idiopathic generalized epilepsies with variable phenotypes o Juvenile absence epilepsy o Juvenile myoclonic epilepsy o Epilepsy with generalized tonic-clonic seizures only Reflex epilepsies o Idiopathic photosensitive occipital lobe epilepsy o Other visual sensitive epilepsies o Primary reading epilepsy o Startle epilepsy Autosomal dominant nocturnal frontal lobe epilepsy Familial temporal lobe epilepsies * Generalized epilepsies with febrile seizures plus * Familial focal epilepsy with variable foci Symptomatic (or probably symptomatic) focal epilepsies o Limbic epilepsies

Mesial temporal lobe epilepsy with hippocampal sclerosis Mesial temporal lobe epilepsy defined by specific etiologies Other types defined by location and etiology o Neocortical epilepsies Rasmussen syndrome Other types defined by location and etiology * Syndromes in development CONDITIONS WITH EPILEPTIC SEIZURES THAT DO NOT REQUIRE A DIAGNOSIS OF EPILEPSY Benign neonatal seizures Febrile seizures Reflex seizures Alcohol withdrawal seizures Drug or other chemically-induced seizures Immediate and early post traumatic seizures Single seizures or isolated clusters of seizures Rarely repeated seizures (oligo-epilepsy) Table 6 AN EXAMPLE OF A CLASSIFICATION OF EPILEPSY SYNDROMES Groups of Syndromes Specific Syndromes Idiopathic Focal Epilepsies of Infancy and Benign Infantile Seizures (Non-Familial Childhood Benign Childhood Epilepsy with Centrotemporal Spikes Early Onset Benign Childhood Occipital Epilepsy (Panayiotopoulos type) Late Onset Childhood Occipital Epilepsy (Gastaut type) Familial (Autosomal Dominant) Focal Benign Familial Neonatal Seizures Epilepsies Benign Familial Infantile Seizures Autosomal Dominant Nocturnal Frontal Lobe Epilepsy Familial Temporal Lobe Epilepsy Familial Focal Epilepsy with Variable Foci* Symptomatic (or Probably Symptomatic) Focal Limbic Epilepsies Epilepsies Mesial Temporal Lobe Epilepsy with Hippocampal Sclerosis Mesial Temporal Lobe Epilepsy Defined by Specific Etiologies Other Types Defined by Location and Etiology Neocortical Epilepsies Rasmussen Syndrome Hemiconvulsion - Hemiplegia Syndrome
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Other Types Defined by Location and Etiology Migrating Partial Seizures of Early Infancy * Idiopathic Generalized Epilepsies Benign Myoclonic Epilepsy in Infancy Epilepsy with Myoclonic Astatic Seizures Childhood Absence Epilepsy Epilepsy with Myoclonic Absences Idiopathic Generalized Epilepsies with Variable Phenotypes Juvenile Absence Epilepsy Juvenile Myoclonic Epilepsy Epilepsy with Generalized Tonic-Clonic Seizures Only Generalized Epilepsies with Febrile Seizures Plus* Reflex Epilepsies Idiopathic Photosensitive Occipital Lobe Epilepsy Other Visual Sensitive Epilepsies Primary Reading Epilepsy Startle Epilepsy Epileptic Encephalopathies (in which the Early Myoclonic Encephalopathy epileptiform abnormalities may contribute to Ohtahara Syndrome progressive dysfunction) West Syndrome Dravet Syndrome (Previously Known as Severe Myoclonic Epilepsy in Infancy) Myoclonic Status in Non-Progressive Encephalopathies Lennox-Gastaut Syndrome Landau-Kleffner Syndrome Epilepsy with Continuous Spike-Waves during Slow Wave Sleep Progressive Myoclonus Epilepsies See specific diseases Seizures Not Necessarily Requiring a Diagnosis Benign Neonatal Seizures Febrile Seizures of Epilepsy Reflex Seizures Alcohol Withdrawal Seizures Drug or Other Chemically-Induced Seizures Immediate and early Post Traumatic Seizures Single Seizures or Isolated Clusters of Seizures Rarely Repeated Seizures (Oligo-Epilepsy) * Syndromes in Development Table 7 AN EXAMPLE OF A CLASSIFICATION OF DISEASES FREQUESNTLY ASSOCIATED WITH EPILEPTIC SEIZURES OR EPILEPSY SYNDROMES Groups of Diseases Specific Diseases
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Progressive Myoclonic Epilepsies

Neurocutaneous Disorders

Malformations Due to Abnormal Cortical Developments

Other Cerebral Malformations

Tumors

Chromosomal Abnormalities

Monogenic Mendelian Diseases with complex Pathogenic Mechanisms

Ceroid Lipofuscinosis Sialidosis Lafora Disease Unverricht-Lundborg Disease Neuroaxonal Dystrophy MERRF Dentatorubropallidoluysian Atrophy Other Tuberous Sclerosis Complex Neurofibromatosis Hypomelanosis of Ito Epidermal Nevus Syndrome Sturge - Weber Syndrome Isolated Lissencephaly Sequence Miller-Dieker Syndrome X-Linked Lissencephaly Subcortical Band Heterotopia Periventricular Nodular Heterotopia Focal Heterotopia Hemimegalencephaly Bilateral Perisylvian Syndrome Unilateral Polymicrogyria Schizencephalies Focal or Multifocal Cortical Dysplasia Microdysgenesis Aicardi Syndrome PEHO Syndrome Acrocallosal Syndrome Other DNET Gangliocytoma Ganglioglioma Cavernous Angiomas Astrocytomas Hypothalamic Hamartoma (with Gelastic Seizures) Other Partial Monosomy 4P or Wolf-Hirschorn Syndrome Trisomy 12p Inversion Duplication 15 Syndrome Ring 20 Chromosome Other Fragile X Syndrome Angelman Syndrome Rett Syndrome
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Inherited Metabolic Disorders

Prenatal or Perinatal Ischemic or Anoxic Lesions or Cerebral Infections Causing Nonprogressive Encephalopathies

Postnatal Infections

Other Postnatal Factors

Miscellaneous

Other Nonketotic Hyperglycinemia D-Glyceric Acidemia Propionic Acidemia Sulphite-Oxidase Deficiency Fructose 1-6 Diphosphatase Deficiency Other Organic Acidurias Pyridoxine - Dependency Aminoacidopathies (Maple Syrup Urine Disease, Phenylketonuria, Other) Urea Cycle Disorders Disorders of Carbohydrate Metabolism Disorders of Biotin Metabolism Disorders of Folic Acid and B12 Metabolism Glucose Transport Protein Deficiency Menkes Disease Glycogen Storage Disorders Krabbe Disease Fumarase Deficiency Peroxisomal Disorders Sanfilippo Syndrome Mitochondrial Diseases (Pyruvate Dehydrogenase Deficiency, Respiratory Chain Defects, MELAS) Porencephaly Periventricular Leukomalasia Microcephaly Cerebral Calcifications and other Lesions due to Toxoplasmosis, CVI, HIV, etc. Cysticercosis Herpes Encephalitis Bacterial Meningitis Other Head Injury Alcohol and Drugs Abuse Stroke Other Celiac Disease (Epilepsy with occipital calcifications and Celiac Disease) Coffin-Lowry Syndrome Alzheimer's Disease Huntington Disease Alpers' Disease

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REFERENCES 1. Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised clinical and electroencephalographic classification of epileptic seizures. Epilepsia 22:489-501, 1981. 2. Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia 30:389-399, 1989. 3. Gastaut H. Clinical and electroencephalographical classification of epileptic seizures. Epilepsia 11:102-113, 1970. 4. Lders H, Acharya J, Baumgartner C, Benbadis S, Bleasel A, Burgess R, Dinner DS, Ebner A, Foldvary N, Geller E, Hamer H, Holthausen H, Kotagal P, Morris H, Meencke HJ, Noachtar S, Rosenow F, Sakamoto A, Steinhoff BJ, Tuxhorn I, Wyllie E. Semiological seizure classification. Epilepsia 39:1006-1013, 1998. 5. International Classification of Functioning and Disability, Beta-2 Draft, Full Version. Geneva: World Health Organization, July 1999. Jerome Engel, Jr. UCLA School of Medicine Los Angeles, CA For the Task Force

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GLOSSARY INTRODUCTION This glossary intends to provide a standard terminology for health care workers to communicate what is observed and what a patient reports during a seizure. As this terminology is descriptive and phenomenological, its use would not imply or require knowledge of ictal pathophysiology, any pathological substrate or etiology. Many terms are adjectives modifying "seizure" which itself is defined under "general terms". This pertains to seizures with single or multiple components. Terms in this glossary, e.g. "seizure", "ictus", which have widespread applicability in other fields of clinical neuroscience, are herein defined according to their references to epilepsy. Some terms of this glossary are "fundamental" i.e. they encompass other more precise words. These can be used as the sole descriptor when data to more precisely characterise a phenomenon are not available. Such include aura, automatism, experiential, motor and sensory. A seizure will often consist of two or more phenomena occurring simultaneously or sequentially and would be described accordingly. Quantitative terms, such as duration of motor events, are not intended as immutable confines, but as clarifying guides to describe clinically observed events. Scientific progress dictates an evolution of terms to retain their relevance. However, needs of communication in everyday life require that changes be gradual and evolutionary than abrupt and revolutionary. The use of synonyms in this glossary reflects incidences where gradual changes are likely. Terminology in some areas remains unresolved. Therefore, we view this glossary as a dynamic process for which feedback will be welcomed. PRINCIPLES FOR TERMS AND DEFINITIONS In developing the "lexique" of this report we adopted and applied the following principles. Terms and definitions should: 1. Contain features which distinguish or modify seizure entities. 2. Be descriptive of the phenomena involved. 3. Comply with terminology of clinical neuroscience. 4. Employ current terminology and definitions wherever possible. 5. Contain new terms only if necessary. 6. Be easily translatable to other languages. 7. Be readily understood and employed by potential users.

DATA SOURCES Adams RD, Victor M. Principles of Neurology. McGraw Hill Book, New York, 1993, 5th edition. Aicardi, J. Epilepsy in Children. International Review of Child Neurology Series. Raven Press, New York, 1986, 1994. Benson, D.F. The Neurology of Thinking. Oxford University Press, New York, 1994, pp 224225. Blume, WT, Berkovic S, Dulac O. (1997) Search for a better classification of the epilepsies. In: Epilepsy: A Comprehensive Textbook, Vol. 1. Edited by J. Engel Jr. and T.A. Pedley, Lippincott-Raven Publishers, Philadelphia, Chapter 69, pp 779-789.

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Delgado-Escueta, A.V., Serratosa, J.M., Medina, M.T. Myoclonic seizures, and progressive myoclonus epilepsy syndromes. In: The Treatment of Epilepsy: Principles and Practice, 2nd ed. Edited by E. Wyllie, Williams & Wilkins, Baltimore, 1996, pp 467-483. Engel, J.Jr. Seizures and Epilepsy. F.A. Davis Company, Philadelphia, 1989. Engel, J.Jr. and Pedley, T.A. Epilepsy: A Comprehensive Textbook, Volumes 1-3, LippincottRaven Publishers, Philadelphia, 1997. Gastaut, H. and Broughton, R. Epileptic Seizures. Clinical and Electrographic Features, Diagnosis and Treatment. Charles C. Thomas, Springfield, Ill, 1972. Gloor, P. Consciousness as a neurological concept in epileptology: a critical review. Epilepsia 1986;27 (Suppl 2):S14-S26. Gloor, P. The Temporal Lobe and Limbic System. Oxford University Press, 1997. Hopkins AP, Michael WF. Spinal myoclonus. J Neurol Neurosurg Psychiatry 1974;37:11121115. Luders H, et al. Semiological seizure classification. Epilepsia 1998;39:1006-1013. Moscovitch, M. Information processing and the cerebral hemispheres. In: Gazzaniga MS, ed. Handbook of Behavioral Neurobiology: Neuropsychology. Vol 2. New York, NY: Plenum Publishing Corp; 1979:379-446. Penfield, W. and Jasper, H.H. Epilepsy and Functional Anatomy of the Human Brain. Little, Brown and Company, 1954. Pryse-Phillips, W. Companion to Clinical Neurology, Little, Brown and Company, 1995. Rowland, L.P. Merritt's Textbook of Neurology, 8th Edition. Lea & Febiger, Philadelphia, 1989. So, N. Epileptic auras. In: The Treatment of Epilepsy: Principles and Practice, 2nd ed. Edited by E. Wyllie, Williams & Wilkins, Baltimore, 1996, pp 376-384. Wolf, P. Epileptic Seizures and Syndromes. John Libbey & Company Ltd., 1994. Young, G.B. Coma and Impaired Consciousness: A Clinical Perspective. McGraw-Hill, New York, 1998. Young, G.B. and Pigott, S.E. Neurobiological basis of consciousness. Archives of Neurology 1999;56:153-157. Some non-medical texts: The Concise Oxford Dictionary. 7th edition. Edited by J.B. Sykes. Clarendon Press; Oxford, England, 1982. Dictionnaire Encyclopedique. Petit Larousse Illustre. Librairie Larousse, Paris, 1973. Dictionary of Canadian English. The Senior Dictionary. Edited by W.S. Avis, P.D. Drysdale, R.J. Gregg, M.H. Scargill. W.J. Gage Limited; Toronto, Canada, 1967. I. GENERAL TERMS 1.0 SEMEIOLOGY (SEMIOLOGY) That branch of linguistics concerned with signs and symptoms. 2.0 EPILEPTIC SEIZURE Manifestation(s) of epileptic (excessive and/or hypersynchronous), usually self-limited activity of neurons in the brain. 3.0 ICTUS A sudden neurological occurrence such as a stroke or an epileptic seizure. 4.0 EPILEPSY a) Epileptic Disorder: A chronic neurological condition characterised by recurrent epileptic seizures.

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b) Epilepsies: Those conditions involving chronic recurrent epileptic seizures that can be considered epileptic disorders. 5.0 FOCAL (syn. partial) A seizure whose initial semiology indicates, or is consistent with, initial activation of only part of one cerebral hemisphere. 6.0 GENERALISED (syn. bilateral) A seizure whose initial semiology indicates, or is consistent with, more than minimal involvement of both cerebral hemispheres. 7.0 CONVULSION Primarily a lay term. Episodes of excessive, abnormal muscle contractions, usually bilateral, which may be sustained or interrupted. II. TERMS DESCRIBING EPILEPTIC SEIZURE SEMEIOLOGY These are descriptors of seizures unless specified otherwise. 1.0 MOTOR Involves musculature in any form. The motor event could consist of an increase (positive) or decrease (negative) in muscle contraction to produce a movement. Unless noted, the following terms are adjectives modifying "motor seizure" or "seizure" e.g. "tonic motor seizure or dystonic seizure", and whose definitions can usually be understood as prefaced by: "refers to ...". 1.1 ELEMENTARY MOTOR A single type of contraction of a muscle or group of muscles that is usually stereotyped and not decomposable into phases. (However, see Tonic-Clonic, an elementary motor sequence). 1.1.1 TONIC A sustained increase in muscle contraction lasting a few seconds to minutes. 1.1.1.1 EPILEPTIC SPASM (Formerly Infantile Spasm) Noun: A sudden flexion, extension or mixed extension-flexion of predominantly proximal and truncal muscles which is usually more sustained than a myoclonic movement but not as sustained as a tonic seizure i.e. about 1 sec. Limited forms may occur: grimacing, head nodding.. Epileptic spasms frequently occur in clusters. 1.1.1.2 POSTURAL Adoption of a posture which may be bilaterally symmetrical or asymmetrical (as in a "fencing posture"). 1.1.1.2.1 VERSIVE A sustained, forced conjugate ocular, cephalic and/or truncal rotation or lateral deviation from the midline. 1.1.1.2.2 DYSTONIC Sustained contractions of both agonist and antagonist muscles producing athetoid or twisting movements which when prolonged may produce abnormal postures. 1.1.2 MYOCLONIC (adjective); MYOCLONUS (noun) Sudden, brief (< 100 msec) involuntary single or multiple contraction(s) of muscles(s) or muscle groups of variable topography (axial, proximal limb, distal). 1.1.2.1 NEGATIVE MYOCLONIC Interruption of tonic muscular activity for < 500 msec without evidence of antecedent myoclonia. 1.1.2.2 CLONIC Myoclonus which is regularly repetitive, involves the same muscle groups, at a frequency of about 2-3 c/sec, and is prolonged. Synonym: rhythmic myoclonus.

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1.1.2.2.1 JACKSONIAN MARCH Noun: Traditional term indicating spread of clonic movements through contiguous body parts unilaterally. 1.1.3 TONIC-CLONIC A sequence consisting of a tonic followed by a clonic phase. Variants such as clonic-tonic-clonic may be seen. 1.1.3.1 GENERALISED TONIC-CLONIC SEIZURE (syn. bilateral tonic-clonic seizure (Formerly "Grand Mal" Seizure)) Noun: Bilateral symmetrical tonic contraction then bilateral clonic contractions of somatic muscles usually associated with autonomic phenomena. 1.1.4 ATONIC Sudden loss or diminution of muscle tone without apparent preceding myoclonic or tonic event lasting one to two seconds or more, involving head, trunk, jaw or limb musculature. 1.1.5 ASTATIC Loss of erect posture that results from an atonic, myoclonic or tonic mechanism. Synonym: drop attack. 1.1.6 SYNCHRONOUS (Asynchronous) Motor events occurring (not) at the same time or at the same rate in sets of body parts. 1.2 AUTOMATISM Noun: A more or less coordinated, repetitive, motor activity usually occurring when cognition is impaired and for which the subject is usually amnesic afterwards. This often resembles a voluntary movement, and may consist of inappropriate continuation of ongoing preictal motor activity. The following adjectives are usually employed to modify "automatism". 1.2.1 OROALIMENTARY Lip smacking, lip pursing, chewing, licking, tooth grinding or swallowing. 1.2.2 MIMETIC Facial expression suggesting an emotional state, often fear. 1.2.3 MANUAL OR PEDAL 1. Indicates principally distal components, bilateral or unilateral. 2. Fumbling, tapping, manipulating movements. 1.2.4 GESTURAL Often unilateral. 1. Fumbling or exploratory movements with the hand directed toward self or environment. 2. Movements resembling those intended to lend further emotional tone to speech. 1.2.5 HYPERKINETIC 1. Involves predominantly proximal limb or axial muscles producing irregular sequential ballistic movements, such as pedalling, pelvic thrashing, rocking movements. 2. Increase in rate of ongoing movements or inappropriately rapid performance of a movement. 1.2.6 HYPOKINETIC A decrease in amplitude and/or rate or arrest of ongoing motor activity. 1.2.7 DYSPHASIC Impaired communication involving language without dysfunction of relevant primary motor or sensory pathways, manifested as impaired comprehension, anomia, paraphasic errors or a combination of these.

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1.2.8 DYSPRAXIC Inability to perform learned movements spontaneously or on command or imitation despite intact relevant motor and sensory systems and adequate comprehension and cooperation. 1.2.9 GELASTIC Bursts of laughter or giggling, usually without an appropriate affective tone. 1.2.10 DACRYSTIC Bursts of crying. 1.2.11 VOCAL Single or repetitive utterances consisting of sounds such as grunts or shrieks. 1.2.12 VERBAL Single or repetitive utterances consisting of words, phrases or brief sentences. 1.2.13 SPONTANEOUS Stereotyped, involve only self, and are virtually independent of environmental influences. 1.2.14 INTERACTIVE Not stereotyped, involve more than self, and are environmentally influenced. 2.0 NON-MOTOR 2.1 AURA Noun: A subjective ictal phenomenon that, in a given patient, may precede an observable seizure; if alone, constitutes a sensory seizure. 2.2 SENSORY A perceptual experience not caused by appropriate stimuli in the external world. Modifies "seizure or "aura". 2.2.1 ELEMENTARY A single, unformed phenomenon involving one primary sensory modality, e.g. somatosensory, visual, auditory, olfactory, gustatory, epigastric, or cephalic. 2.2.1.1 SOMATOSENSORY Tingling, numbness, electric shock sensation, pain, sense of movement, or desire to move. 2.2.1.2 VISUAL Flashing or flickering lights, spots, simple patterns, scotomata, or amaurosis. 2.2.1.3 AUDITORY Buzzing, drumming sounds or single tones. 2.2.1.4 OLFACTORY Odour, usually disagreeable. 2.2.1.5 GUSTATORY Taste sensations including acidic, bitter, salty , sweet or metalic. 2.2.1.6 EPIGASTRIC Abdominal discomfort including nausea, emptiness, tightness, churning, butterflies, malaise, pain, and hunger; sensation may rise to chest or throat. Some phenomena may reflect ictal autonomic dysfunction. 2.2.1.7 CEPHALIC Sensation in the head such as light headedness, tingling or headache. 2.2.1.8 AUTONOMIC A sensation consistent with involvement of the autonomic nervous system, including cardiovascular, gastrointestinal, sudomotor, vasomotor and thermoregulatory functions. (Thus, "autonomic aura"; cf. "autonomic seizure" 3.0).

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2.2.2 EXPERIENTIAL Affective, mnemonic or composite perceptual phenomena including illusory or composite hallucinatory events; these may appear alone or in combination. Included are feelings of depersonalisation. These phenomena have subjective qualities similar to those experienced in life but are recognised by the subject as occurring outside of actual context. 2.2.2.1 AFFECTIVE Components include: fear, depression, joy and (rarely) anger. 2.2.2.2 MNEMONIC Components which reflect ictal dysmnesia such as: feelings as familiarity (dj-vu) and unfamiliarity (jamais-vu). 2.2.2.3 HALLUCINATORY A creation of composite perceptions without corresponding external stimuli involving visual, auditory, somatosensory, olfactory and/or gustatory phenomena. Example: "hearing" and "seeing" people talking. 2.2.2.4 ILLUSORY An alteration of actual percepts involving the visual, auditory, somatosensory, olfactory or gustatory systems. 2.3 DYSCOGNITIVE The term describes events in which: 1) disturbance of cognition is the prominent or most apparent feature, and 2a) two or more of the following components are involved, or 2b) contributions of such components remain undetermined. Otherwise, use more specific term e.g. mnemonic experimental seziure or "hallucinatory experimental seizure." Components of cognition: perception: symbolic conception of sensory information attention: appropriate selection of a principal perception or task emotion: appropriate affective significance of a perception memory: ability to store and retrieve percepts or concepts executive function: anticipation, selection, monitoring of consequences, initiation of motor activity including praxis, speech 3.0 AUTONOMIC EVENTS 3.1 AUTONOMIC AURA A sensation consistent with involvement of the autonomic nervous system, including cardiovascular, gastrointestinal, sudomotor, vasomotor and thermoregulatory functions. (see 2.3.1.8). 3.2 AUTONOMIC SEIZURE An objectively documented and distinct alteration of autonomic nervous system function involving cardiovascular, pupillary, gastrointestinal, sudomotor, vasomotor and thermoregularity functions. 4.0 SOMATOTOPIC MODIFIERS 4.1 LATERALITY 4.1.1 UNILATERAL Exclusive or virtually exclusive involvement of one side as a motor, sensory or autonomic phenomenon. 4.1.1.1 HEMIA prefix to other descriptors e.g. hemiclonic.

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4.1.2 GENERALISED (syn. "bilateral") More than minimal involvement of each side as a motor, elementary sensory or autonomic phenomenon. Motor component: further modified as: 4.1.2.1 ASYMMETRICAL Clear distinction in quantity and/or distribution of behaviour on the two sides. 4.1.2.2 SYMMETRICAL Virtual bilateral equality in these respects. 4.2 BODY PART Refers to area involved i.e. arm, leg, face, trunk and other. 4.3 CENTRICITY Modifier describes proximity to the body axis. 4.3.1 AXIAL Involves trunk, including neck. 4.3.2 PROXIMAL LIMB Signifies involvement from shoulders to wrist, hip to ankle. 4.3.3 DISTAL LIMB Indicates involvement of fingers, hands, toes, and/or feet. 5.0 MODIFIERS AND DESCRIPTORS OF SEIZURE TIMING The following terms are listed in the form (adjective, noun, verb) according to principal usage; as adjective unless specified. 5.1 INCIDENCE Noun: Refers to the number of epileptic seizures within a time period or the number of seizure days per unit of time. 5.1.1 REGULAR, IRREGULAR Consistent (inconsistent) or predictable (unpredictable, chaotic) intervals between such events. 5.1.2 CLUSTER 1) Noun: Incidence of seizures within a given period (usually one or a few days) which exceeds the average of incidence over a longer period for the patient. 2) Verb: To vary in incidence as above. 5.1.3 PROVOCATIVE FACTOR Noun: Transient and sporadic endogenous or exogenous element capable of augmenting seizure incidence in persons with chronic epilepsy and evoking seizures in susceptible non-epileptic individuals. 5.1.3.1 REACTIVE Occurring in association with transient systemic perturbation such as intercurrent illness, sleep loss or emotional stress. 5.1.3.2 REFLEX Objectively and consistently demonstrated to be evoked by a specific afferent stimulus or by activity of the patient. Afferent stimuli can be: elementary, i.e. unstructured (light flashes, startle, a monotone) or elaborate i.e. structured, (a symphony). Activity may be elementary, e.g. motor (a movement); or elaborate, e.g. cognitive function (reading, chess playing), or both (reading aloud). 5.2 STATE DEPENDENT Occurring exclusively or primarily in the various stages of drowsiness, sleep, or arousal.

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5.3 CATAMENIAL Seizures occurring principally or exclusively in any one phase of the menstrual cycle. 6.0 DURATION Time between the beginning of initial seizure manifestations, such as the aura, to the cessation of experienced or observed seizure activity. Does not include non-specific seizure premonitions or postictal states. 6.1 STATUS EPILEPTICUS A seizure which shows no clinical signs of arresting after a duration encompassing the great majority of seizures of that type in most patients or recurrent seizures without resumption of baseline central nervous system function interictally. 7.0 SEVERITY A multicomponent assessment of a seizure by observers and the patient. Components primarily of observer assessment include: duration, extent of motor involvement, impairment of cognitive interaction with environment intraictally, maximum number of seizures per unit of time. Components primarily of patient assessment: extent of injury; emotional, social and vocational consequences of the attack. 8.0 PRODROME A preictal phenomenon. A subjective or objective clinical alteration, e.g. ill-localised sensation or agitation, that heralds the onset of an epileptic seizure but does not form part of it. 9.0 POSTICTAL PHENOMENON A transient clinical abnormality of central nervous system function that appears or becomes accentuated when clinical signs of the ictus have ended. 9.1 LATERALISING (TODD'S (OR BRAVAIS')) PHENOMENON Any unilateral postictal dysfunction relating to motor, speech, somatosensory and/or integrative functions including visual, auditory or somatosensory neglect phenomena. 9.2 NON-LATERALISING PHENOMENA Impaired cognition, amnesia, psychosis. 9.2.1 IMPAIRED COGNITION Decreased cognitive performance involving one or more of: perception, attention, emotion, memory, execution, praxis, speech (cf Dyscognitive, 2.3). 9.2.2 ANTEROGRADE AMNESIA Impaired ability to remember new material. 9.2.3 RETROGRADE AMNESIA Impaired ability to recall previously remembered material. 9.2.4 PSYCHOSIS Misinterpretation of external world in an awake, alert person; involves thought disorder of emotion and socialisation.

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