ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology
2017/03/08 by Ingrid E. Scheffer, Samuel Berkovic, Samuel F. Berkovic +18 · 92 citations
Medicine · Neuroscience · #Epilepsy research and treatment #Pharmacological Effects and Toxicity Studies #Neuroscience and Neuropharmacology Research
paper · pdf · doi:10.1111/epi.13709
Abstract
The International League Against Epilepsy (ILAE) Classification of the Epilepsies has been updated to reflect our gain in understanding of the epilepsies and their underlying mechanisms following the major scientific advances that have taken place since the last ratified classification in 1989. As a critical tool for the practicing clinician, epilepsy classification must be relevant and dynamic to changes in thinking, yet robust and translatable to all areas of the globe. Its primary purpose is for diagnosis of patients, but it is also critical for epilepsy research, development of antiepileptic therapies, and communication around the world. The new classification originates from a draft document submitted for public comments in 2013, which was revised to incorporate extensive feedback from the international epilepsy community over several rounds of consultation. It presents three levels, starting with seizure type, where it assumes that the patient is having epileptic seizures as defined by the new 2017 ILAE Seizure Classification. After diagnosis of the seizure type, the next step is diagnosis of epilepsy type, including focal epilepsy, generalized epilepsy, combined generalized, and focal epilepsy, and also an unknown epilepsy group. The third level is that of epilepsy syndrome, where a specific syndromic diagnosis can be made. The new classification incorporates etiology along each stage, emphasizing the need to consider etiology at each step of diagnosis, as it often carries significant treatment implications. Etiology is broken into six subgroups, selected because of their potential therapeutic consequences. New terminology is introduced such as developmental and epileptic encephalopathy. The term benign is replaced by the terms self-limited and pharmacoresponsive, to be used where appropriate. It is hoped that this new framework will assist in improving epilepsy care and research in the 21st century.
Citations
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- The novel sodium channel modulator GS‐458967 (GS967) is an effective treatment in a mouse model of SCN8A encephalopathy
- The power of ECG in multimodal patient‐specific seizure monitoring: Added value to an EEG‐based detector using limited channels
- The novel persistent sodium current inhibitor PRAX‐562 has potent anticonvulsant activity with improved protective index relative to standard of care sodium channel blockers
- Epidemiology of Developmental and Epileptic Encephalopathy and of Intellectual Disability and Epilepsy in Children
- The neuroprotective effects of N-acetylcysteine in psychiatric and neurodegenerative disorders: From modulation of glutamatergic transmission to restoration of synaptic plasticity
- Association of seizure control during pregnancy with adverse offspring outcomes in women with epilepsy
- Developmental and epileptic encephalopathies: from genetic heterogeneity to phenotypic continuum
- Mechanisms linking neurological disorders with reproductive endocrine dysfunction: Insights from epilepsy research
- PERSONALIZED BIOMARKERS OF MULTISCALE FUNCTIONAL ALTERATIONS IN TEMPORAL LOBE EPILEPSY
- The Human Phenotype Ontology in 2021
- The ClinGen Epilepsy Gene Curation Expert Panel—Bridging the divide between clinical domain knowledge and formal gene curation criteria
- P2Y12 Receptor Function Governs Microglial Surveillance and Cell–Cell Interactions in the Cerebral Cortex
- Temporal Lobe Epilepsy Perturbs the Brain‐Wide Excitation‐Inhibition Balance: Associations with Microcircuit Organization, Clinical Parameters, and Cognitive Dysfunction
- Implementation of a Novel Seizure Assessment Tool for Unified Seizure Evaluation Improves Nurse Response
- Neuroinflammation and Proinflammatory Cytokines in Epileptogenesis
- Neuroinflammatory mediators in acquired epilepsy: an update
- The neurodevelopmental spectrum of CASK-related disorder
- Clinical and genetic landscape of epilepsies with absence seizures and single‐gene etiology
- Modeling AP2M1 developmental and epileptic encephalopathy in Drosophila
- Development and validation of clinico‐imaging machine learning and deep learning models to predict responses to initial antiseizure medications in epilepsy
- De novo SRCAP variants cause developmental and epileptic encephalopathy and the phenotypic spectrum
- Intestinal microbiome alterations in pediatric epilepsy: Implications for seizures and therapeutic approaches
- Genetic complexity in pediatric onset epilepsy‐movement disorder syndromes: Insights from a cohort of 97 subjects
- Genetic risk factor identification for common epilepsies guided by integrative omics data analysis
- Autoimmune and paraneoplastic encephalitis–associated neuronal autoantibodies with serial EEG in adults with unexplained new‐onset seizures or worsening epilepsy
- Syndromic and etiological classification predicts seizure freedom in childhood and youth onset epilepsy: A population‐based study from the Norwegian Mother, Father, and Child Cohort Study
- The role of neuropsychology in stereo‐ EEG : A narrative review
- Neurocognitive, behavioral, and treatment burden as key predictors of parental stress in pediatric epilepsy
- Real‐world use of cenobamate in pediatric focal epilepsies and developmental epileptic encephalopathies: A multicenter retrospective series
- Frontiers in EEG as a tool for the management of pediatric epilepsy: Past, present, and future
- Chronic behavioral and seizure outcomes following experimental traumatic brain injury and comorbid Klebsiella pneumoniae lung infection in mice
- Transition to Adult Care in Childhood‐Onset Epilepsy: A Retrospective Cohort Study at a Japanese Children's Hospital
- Phase 1 study of ABI ‐009 ( nab ‐rapamycin) for surgically refractory epilepsy ( RaSuRE )
- Drosophila melanogaster as a rapid in vivo assay system for preclinical anti‐seizure medication testing
- Associations between the gut microbiota, immune cells, and different subtypes of epilepsy: A Mendelian randomization study
- GABRA2‐related encephalopathy: Identification of two phenotypes with distinctive electroclinical features
- Relationship between the time course of Burden of Amplitudes and Epileptiform Discharges scores and relapse in children with infantile epileptic spasms syndrome
- Epilepsy syndromes classification
- Psychopathology in children before and after epilepsy surgery: a prospective controlled study
- The epilepsy–autism phenotype associated with developmental and epileptic encephalopathies: New mechanism‐based therapeutic options
- The impact of radiofrequency thermocoagulation on brain connectivity in drug‐resistant epilepsy: Insights from stereo‐electroencephalography and cortico‐cortical evoked potentials
- Pharmacological Correction of Aberrant DSCAML1 Localization by 4‐Phenylbutyrate Ameliorates Epileptic Phenotypes in a Mouse Model Harboring the Patient‐Specific A2105T Mutation
- Behavioral Adverse Effects During Initial Six Weeks of Levetiracetam Therapy in Children with Epilepsy: A Prospective Cohort Study
- Bexicaserin for the treatment of seizures in developmental and epileptic encephalopathies: A phase 1b/2a trial ( PACIFIC )
- Hypophosphatemia in suspected seizures evaluated in first seizure clinics and neurology consults
- Spanish translation, adaptation, and validation of the Epilepsy Surgery Satisfaction Questionnaire‐19
- Motives and predictors of modified Atkins diet discontinuation as treatment of adults with drug‐resistant epilepsy
- Haploinsufficiency of brain‐specific kinase BRSK1 causes epilepsy and neurodevelopmental disorders
- Prenatal betamethasone–postnatal N‐methyl‐D‐aspartic acid model of spasms: Update on mechanisms and treatments
- Association study of ADORA2A gene polymorphisms with adverse drug reactions to valproic acid and oxcarbazepine in the treatment of children with epilepsy
- Climate change and hyponatremia‐related hospital admissions in people with focal epilepsy exposed to carbamazepine or its derivatives
- Ultra-rare biallelic THAP12 variants cause loss of function and underlie severe epileptic encephalopathy
- EEG features in late-onset epilepsy: possible correlation with cognitive impairment
- Areas of research priorities in epilepsy: A position paper of the European Reference Network for Rare and Complex Epilepsies, EpiCARE
- Distinct spike‐and‐wave EEG profiles reveal susceptibility to fleeting/almost loss of consciousness (so‐called blips) in generalized epilepsy
- The impact of revised definitions on the epidemiology of status epilepticus: A population‐based study
- Epilepsy expands the phenotype of L‐arginine:glycine amidinotransferase deficiency
- Long‐term outcomes of corpus callosotomy in adult patients with drug‐resistant epilepsy: Results from a single neurosurgical center in Mexico
- Extracting epilepsy‐related information from unstructured clinic letters using large language models
- Tumor‐related epilepsy in glioma: A multidisciplinary overview
- Chronic thalamic recordings for idiopathic generalized epilepsy and Lennox–Gastaut syndrome: Ictal and interictal electrophysiological findings
- Broadening the phenotype associated with pathogenic variants in the FGF12 gene: From developmental and epileptic encephalopathy to drug‐responsive epilepsy with favorable cognitive outcome
- The UMPIRE study: A first‐in‐human multicenter trial of bilateral subscalp monitoring for epileptic seizure detection
- The 2022 International League Against Epilepsy Classification and Definition of Childhood Epilepsy Syndromes: An Update for Pediatricians
- Knowledge, attitudes, and practices of epilepsy patients regarding the ketogenic diet therapy: A cross‐sectional study
- Effects of perampanel on bone health in adult patients with epilepsy
- Epilepsy [wikipedia]
- Epilepsy biomarkers - Toward etiology and pathology specificity. [europepmc]
- Genome-wide mega-analysis identifies 16 loci and highlights diverse biological mechanisms in the common epilepsies. [europepmc]
- Postconvulsive central apnea as a biomarker for sudden unexpected death in epilepsy (SUDEP). [europepmc]
- Autoimmune seizures and epilepsy. [europepmc]
- Global, regional, and national burden of epilepsy, 1990-2016: a systematic analysis for the Global Burden of Disease Study 2016. [europepmc]
- Evaluation of seizure treatment in anti-LGI1, anti-NMDAR, and anti-GABA B R encephalitis. [europepmc]
- The gut microbiome and epilepsy. [europepmc]
- AMPA receptor GluA2 subunit defects are a cause of neurodevelopmental disorders. [europepmc]
- Incidence and phenotypes of childhood-onset genetic epilepsies: a prospective population-based national cohort. [europepmc]
- Ultra-Rare Genetic Variation in the Epilepsies: A Whole-Exome Sequencing Study of 17,606 Individuals. [europepmc]
- Clinical risk factors in SUDEP: A nationwide population-based case-control study. [europepmc]
- Scn8a Antisense Oligonucleotide Is Protective in Mouse Models of SCN8A Encephalopathy and Dravet Syndrome. [europepmc]
- Dose-Ranging Effect of Adjunctive Oral Cannabidiol vs Placebo on Convulsive Seizure Frequency in Dravet Syndrome: A Randomized Clinical Trial. [europepmc]
- Cenobamate (YKP3089) as adjunctive treatment for uncontrolled focal seizures in a large, phase 3, multicenter, open-label safety study. [europepmc]
- Randomized phase 2 study of adjunctive cenobamate in patients with uncontrolled focal seizures. [europepmc]
- Clinical and experimental insight into pathophysiology, comorbidity and therapy of absence seizures. [europepmc]
- White matter abnormalities across different epilepsy syndromes in adults: an ENIGMA-Epilepsy study. [europepmc]
- Clinical sequencing yield in epilepsy, autism spectrum disorder, and intellectual disability: A systematic review and meta-analysis. [europepmc]
- The Human Phenotype Ontology in 2021. [europepmc]
- Seizure Cycles in Focal Epilepsy. [europepmc]
- The SANAD II study of the effectiveness and cost-effectiveness of levetiracetam, zonisamide, or lamotrigine for newly diagnosed focal epilepsy: an open-label, non-inferiority, multicentre, phase 4, randomised controlled trial. [europepmc]
- The Pharmacology and Clinical Efficacy of Antiseizure Medications: From Bromide Salts to Cenobamate and Beyond. [europepmc]
- Neurobehavioural comorbidities of epilepsy: towards a network-based precision taxonomy. [europepmc]
- GWAS meta-analysis of over 29,000 people with epilepsy identifies 26 risk loci and subtype-specific genetic architecture. [europepmc]
- Interleukin 6: at the interface of human health and disease. [europepmc]
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