ILAE (International League Against Epilepsy)
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Questions and Answers

What is the term for the type of epilepsy that has a presumed hereditary predisposition?

  • Structural Epilepsy
  • Genetic Epilepsy
  • Idiopathic Generalized Epilepsy (correct)
  • Focal Epilepsy

    • What is the difference between Genetic and Idiopathic Epilepsy?

  • Genetic epilepsy is a more severe type of epilepsy
  • Genetic epilepsy has a known mutation, while idiopathic does not
  • Genetic epilepsy is a type of idiopathic epilepsy (correct)
  • Genetic epilepsy is inherited, while idiopathic is not

    • What is the term for epilepsy that has a known structural cause?

  • Genetic Epilepsy
  • Structural Epilepsy (correct)
  • Focal Epilepsy
  • Metabolic Epilepsy

    • What is the importance of de novo mutations in epilepsy?

    <p>They can occur in both mild and severe epilepsies</p> Signup and view all the answers

    What is the difference between Focal and Generalized Epilepsy?

    <p>Focal epilepsy affects one part of the brain, while generalized epilepsy affects the whole brain</p> Signup and view all the answers

    What is the term for epilepsy that has a presumed hereditary predisposition, but the mutation is not known?

    <p>Idiopathic Generalized Epilepsy</p> Signup and view all the answers

    What is the term for epilepsy that is diagnosed on clinical research?

    <p>Idiopathic Generalized Epilepsy</p> Signup and view all the answers

    What is a critical problem in some parts of the world regarding epilepsy?

    <p>Stigma associated with epilepsy</p> Signup and view all the answers

    Which of the following seizure types originates at some point within and rapidly engages bilaterally distributed networks?

    <p>Generalized seizures</p> Signup and view all the answers

    What is the term for seizures that are impaired language, other cognitive domains, and positive features such as déjà vu?

    <p>Cognitive seizures</p> Signup and view all the answers

    Which of the following is NOT a type of epilepsy?

    <p>Dyscognitive epilepsy</p> Signup and view all the answers

    What is the primary purpose of the classification of epilepsies?

    <p>For clinical diagnosis</p> Signup and view all the answers

    What is the term for seizures that are characterized by anxiety, fear, joy, etc?

    <p>Emotional seizures</p> Signup and view all the answers

    What is the main difference between focal and generalized onset epilepsies?

    <p>The location of the seizure onset</p> Signup and view all the answers

    When a seizure type begins with 'focal, generalized, or absence', what can be presumed?

    <p>The word 'onset'</p> Signup and view all the answers

    In what cases may classification according to seizure type be the maximum level of diagnosis possible?

    <p>In cases with limited information</p> Signup and view all the answers

    What is the term for seizures that are characterized by impaired awareness and are discretely localized or more widely distributed?

    <p>Focal seizures</p> Signup and view all the answers

    What is the role of language in the classification of epilepsies?

    <p>To use words that mean what they say</p> Signup and view all the answers

    What is the term for epilepsy that cannot be classified into a specific syndrome or etiology?

    <p>Unknown epilepsy</p> Signup and view all the answers

    What is the relationship between epilepsy syndromes and seizure types?

    <p>Epilepsy syndromes are classified based on seizure types</p> Signup and view all the answers

    Which of the following is an example of combined focal and generalized epilepsy?

    <p>Dravet syndrome</p> Signup and view all the answers

    What is the significance of co-morbidities in the classification of epilepsies?

    <p>Co-morbidities are an important factor in classification</p> Signup and view all the answers

    What is the primary difference between unknown onset and generalized onset epilepsies?

    <p>The level of information available</p> Signup and view all the answers

    What is the significance of etiology in the classification of epilepsies?

    <p>Etiology is one of the factors considered in classification</p> Signup and view all the answers

    What is the term that replaced 'Malignant' and 'Catastrophic' in epilepsy syndromes?

    <p>Self-limited</p> Signup and view all the answers

    What is the primary cause of severe cognitive and behavioral impairment in Developmental and/or Epileptic Encephalopathy?

    <p>Epileptic activity itself</p> Signup and view all the answers

    What is the characteristic of epilepsy syndromes that is no longer approved by ILAE?

    <p>Epilepsy syndromes</p> Signup and view all the answers

    Which of the following is a characteristic of Benign epilepsy syndromes?

    <p>No learning concerns</p> Signup and view all the answers

    What is the primary difference between Developmental and/or Epileptic Encephalopathy and epilepsy syndromes?

    <p>Developmental component</p> Signup and view all the answers

    What is the term used to describe epilepsy that is not severe or debilitating?

    <p>Benign</p> Signup and view all the answers

    What is the primary cause of co-morbidities in epilepsy?

    <p>Underlying pathology</p> Signup and view all the answers

    What is the term used to describe the impact of epilepsy on daily life?

    <p>Psychosocial impact</p> Signup and view all the answers

    What is a key characteristic of Developmental Encephalopathy?

    <p>It may begin in utero or post birth</p> Signup and view all the answers

    What is the significance of Epileptic Encephalopathy?

    <p>It can occur at any age and may have a remediable component</p> Signup and view all the answers

    What is the term for the group of disorders that include KCNQ2 and STXBP1 encephalopathies?

    <p>Developmental and/or Epileptic Encephalopathies</p> Signup and view all the answers

    What is the purpose of the ILAE Classification of the Epilepsies?

    <p>To change the approach to diagnosis in the clinic</p> Signup and view all the answers

    What is the significance of the term 'Symptomatic Generalized Epilepsies'?

    <p>It is an outdated term used for two different groups of disorders</p> Signup and view all the answers

    What is the impact of the new classification framework on clinical care and practice?

    <p>It will change the approach to diagnosis in the clinic</p> Signup and view all the answers

    What is the focus of the ILAE Classification Task Force?

    <p>To update the terminology used in epilepsy research</p> Signup and view all the answers

    What is an example of a Genetic Generalized Epilepsy?

    <p>CAE, JAE, JME, GTCA</p> Signup and view all the answers

    Which type of epilepsy has a focal onset?

    <p>Hemispheric epilepsies</p> Signup and view all the answers

    What is the significance of the term 'Idiopathic Generalized Epilepsies'?

    <p>It implies a presumed hereditary predisposition</p> Signup and view all the answers

    What is the primary difference between Genetic and Idiopathic Epilepsies?

    <p>Genetic epilepsies have a known mutation, while idiopathic epilepsies do not</p> Signup and view all the answers

    Which type of epilepsy is characterized by a combination of focal and generalized seizures?

    <p>Combined focal and generalized epilepsy</p> Signup and view all the answers

    What is the significance of de novo mutations in epilepsy?

    <p>They are found in both mild and severe epilepsies</p> Signup and view all the answers

    What is the primary problem with the term 'Genetic' in epilepsy?

    <p>It is often misinterpreted to mean that gene testing is necessary</p> Signup and view all the answers

    Which of the following is NOT a type of epilepsy classification?

    <p>Epilepsy with a known psychological cause</p> Signup and view all the answers

    What is the significance of the term 'Unknown onset' in epilepsy?

    <p>It implies that the epilepsy cannot be classified into a specific type</p> Signup and view all the answers

    What is the primary purpose of using transparent language in the classification of epilepsies?

    <p>To facilitate communication between healthcare professionals</p> Signup and view all the answers

    In which cases may classification according to seizure type be the maximum level of diagnosis possible?

    <p>In some settings, where it is the maximum level of diagnosis possible</p> Signup and view all the answers

    What is the significance of etiology in the classification of epilepsies?

    <p>It is used to identify the underlying cause of the epilepsy</p> Signup and view all the answers

    What is the primary difference between focal and generalized onset epilepsies?

    <p>The location of the seizure onset</p> Signup and view all the answers

    What is the significance of co-morbidities in the classification of epilepsies?

    <p>They are used to identify other conditions that may be associated with the epilepsy</p> Signup and view all the answers

    What is the primary purpose of the classification of epilepsies?

    <p>To improve clinical diagnosis and treatment</p> Signup and view all the answers

    What is the significance of seizure types in the classification of epilepsies?

    <p>They are used to classify the epilepsy into different categories</p> Signup and view all the answers

    What is the relationship between epilepsy syndromes and seizure types?

    <p>Seizure types are a characteristic of epilepsy syndromes</p> Signup and view all the answers

    What is the primary characteristic of seizures that originate within networks limited to one hemisphere?

    <p>They may be discretely localized or more widely distributed</p> Signup and view all the answers

    What is the term for epilepsy that cannot be classified into a specific syndrome or etiology due to lack of information?

    <p>Unknown Epilepsy</p> Signup and view all the answers

    Which of the following seizure types may include cortical and subcortical structures but not necessarily the entire cortex?

    <p>Generalized seizures</p> Signup and view all the answers

    What is the purpose of clarifying features of seizures?

    <p>To encourage free text descriptors</p> Signup and view all the answers

    What is the term for epilepsy that combines both focal and generalized epilepsy?

    <p>Combined Epilepsy</p> Signup and view all the answers

    What is the characteristic of cognitive seizures?

    <p>Impaired language and other cognitive domains</p> Signup and view all the answers

    When can classification according to seizure type be the maximum level of diagnosis possible?

    <p>When a patient has a single event</p> Signup and view all the answers

    What is the term for seizures that are characterized by anxiety, fear, joy, etc?

    <p>Emotional seizures</p> Signup and view all the answers

    What is the primary distinction between epilepsy syndromes and Developmental and/or Epileptic Encephalopathy?

    <p>Epilepsy syndromes do not have a developmental component, while Developmental and/or Epileptic Encephalopathy does.</p> Signup and view all the answers

    What is the significance of the term 'Self-limited' in epilepsy syndromes?

    <p>It is a term used to describe epilepsy that is not severe or debilitating.</p> Signup and view all the answers

    What is the purpose of the ILAE Classification of the Epilepsies?

    <p>To provide a framework for understanding and organizing epilepsy disorders.</p> Signup and view all the answers

    What is the characteristic of epilepsy syndromes that is no longer approved by ILAE?

    <p>There are no approved ILAE epilepsy syndromes.</p> Signup and view all the answers

    What is the significance of co-morbidities in the classification of epilepsies?

    <p>Co-morbidities are an important factor in the classification of epilepsies.</p> Signup and view all the answers

    What is the primary difference between JME and CAE pair?

    <p>JME is a pair of epilepsy syndromes, while CAE is a single epilepsy syndrome.</p> Signup and view all the answers

    What is the significance of etiology in the classification of epilepsies?

    <p>Etiology is one of the factors considered in the classification of epilepsies.</p> Signup and view all the answers

    What is the characteristic of Benign epilepsy syndromes?

    <p>They are not severe or debilitating.</p> Signup and view all the answers

    What is the main difference between Developmental and Epileptic Encephalopathy?

    <p>One begins in utero, while the other can occur at any age</p> Signup and view all the answers

    Which term is no longer used to describe a group of epilepsy disorders?

    <p>Symptomatic Generalized Epilepsies</p> Signup and view all the answers

    What is the significance of the ILAE Classification of the Epilepsies?

    <p>It updates the terminology to reflect current scientific advances</p> Signup and view all the answers

    What is the primary impact of the new classification framework on clinical care and practice?

    <p>It will change the approach to diagnosis in the clinic</p> Signup and view all the answers

    What is the focus of the ILAE Classification Task Force?

    <p>To update the classification of the epilepsies</p> Signup and view all the answers

    Which of the following is an example of Developmental and/or Epileptic Encephalopathy?

    <p>All of the above</p> Signup and view all the answers

    What is the significance of Etiology in the classification of the epilepsies?

    <p>It is considered at all stages of the diagnosis process</p> Signup and view all the answers

    Which of the following is a characteristic of Genetic Generalized Epilepsies?

    <p>They include epilepsy syndromes such as CAE, JAE, and JME</p> Signup and view all the answers

    What is the primary purpose of the ILAE Classification of the Epilepsies?

    <p>To provide a framework for diagnosis and research</p> Signup and view all the answers

    What is the significance of the ILAE Classification of the Epilepsies being dynamic and translatable?

    <p>It allows for adaptation to changes in understanding of the epilepsies and their underlying mechanisms</p> Signup and view all the answers

    What is the primary challenge in creating a comprehensive classification of the epilepsies?

    <p>Creating a framework that is both relevant and robust</p> Signup and view all the answers

    What is the significance of the updated ILAE Classification of the Epilepsies in the context of global health?

    <p>It makes the classification more relevant and accessible worldwide</p> Signup and view all the answers

    What is the primary goal of the ILAE Commission for Classification and Terminology?

    <p>To create a comprehensive and dynamic classification of the epilepsies</p> Signup and view all the answers

    What is the significance of the ILAE Classification of the Epilepsies in the context of research?

    <p>It facilitates the advancement of epilepsy research and understanding</p> Signup and view all the answers

    What is the primary benefit of a comprehensive and dynamic classification of the epilepsies?

    <p>It improves patient care and outcomes</p> Signup and view all the answers

    What is the significance of the ILAE Classification of the Epilepsies in the context of clinical practice?

    <p>It facilitates accurate diagnosis and treatment of patients</p> Signup and view all the answers

    What is a key feature of epileptic encephalopathy?

    <p>Epileptiform activity interferes with development resulting in cognitive slowing and often regression</p> Signup and view all the answers

    What is the significance of amelioration of epileptiform activity in epileptic encephalopathy?

    <p>It may improve developmental consequences of the disorder</p> Signup and view all the answers

    What is a common feature of many epilepsies?

    <p>They are all associated with comorbidities such as learning and behavioral problems</p> Signup and view all the answers

    Why may the cause of epilepsy not be known in some patients?

    <p>Because the patient has not undergone sufficient evaluation</p> Signup and view all the answers

    What is the significance of a single gene in some epileptic encephalopathies?

    <p>It can cause an epileptic encephalopathy in some individuals and a self-limited epilepsy in others</p> Signup and view all the answers

    What is the impact of epileptiform activity on development in some individuals?

    <p>It can cause regression in individuals with normal development or preexisting developmental delay</p> Signup and view all the answers

    What is the significance of comorbidities in epilepsies?

    <p>They are often associated with learning, psychological, and behavioral problems</p> Signup and view all the answers

    What is the current limitation in diagnosing epilepsy in some healthcare settings?

    <p>Limited extent of evaluation available to patients</p> Signup and view all the answers

    According to the 2010 publication, what was the emphasis on in terminology?

    <p>Using transparent terminology where words mean what they say</p> Signup and view all the answers

    What is the category of epilepsy that includes multiple types of seizures?

    <p>Combined Generalized and Focal Epilepsy</p> Signup and view all the answers

    What is the primary purpose of the classification of the epilepsies?

    <p>To assist in improving epilepsy care and research in the 21st century</p> Signup and view all the answers

    What is the typical characteristic of an individual with Generalized Epilepsy?

    <p>Showing generalized spike-wave activity on EEG</p> Signup and view all the answers

    What is the significance of the classification of the epilepsies beyond the clinical domain?

    <p>It assists in improving clinical care and research in the 21st century</p> Signup and view all the answers

    What is the purpose of the revised Classification of the Epilepsies?

    <p>To provide a framework for classification of the epilepsies</p> Signup and view all the answers

    What is the importance of the classification of the epilepsies in understanding the patient's condition?

    <p>It helps in identifying the potential triggers for their seizures</p> Signup and view all the answers

    What is the category of epilepsy that includes epilepsy syndromes that cannot be classified into a specific syndrome or etiology?

    <p>Unknown Epilepsy</p> Signup and view all the answers

    What is the relationship between the classification of the epilepsies and the development of novel therapies?

    <p>The classification of the epilepsies assists in the development of novel therapies</p> Signup and view all the answers

    What is the significance of the 2014 definition of epilepsy?

    <p>It assumes that the patient has a diagnosis of epilepsy based on the definition</p> Signup and view all the answers

    What is the significance of the ILAE Commission on Classification and Terminology?

    <p>It is responsible for producing a revised Classification of the Epilepsies</p> Signup and view all the answers

    What is the significance of the International League Against Epilepsy (ILAE) in the classification of the epilepsies?

    <p>The ILAE provides a framework for the classification of the epilepsies</p> Signup and view all the answers

    What is the outcome of the revised Classification of the Epilepsies?

    <p>A framework for classification of the epilepsies</p> Signup and view all the answers

    What is the significance of the International Classification of Diseases in the classification of the epilepsies?

    <p>The International Classification of Diseases is used in conjunction with the classification of the epilepsies</p> Signup and view all the answers

    What is the primary goal of the classification of the epilepsies?

    <p>To assist in improving epilepsy care and research in the 21st century</p> Signup and view all the answers

    What is the significance of the classification of the epilepsies in improving epilepsy care?

    <p>It assists in improving epilepsy care and research in the 21st century</p> Signup and view all the answers

    What is the primary purpose of the classification of epilepsies?

    <p>To provide a framework for understanding and comparing epilepsy syndromes</p> Signup and view all the answers

    What is the significance of the ILAE Classification of the Epilepsies?

    <p>It has improved clinical care and practice for epilepsy patients</p> Signup and view all the answers

    What is the purpose of the ILAE Classification Task Force?

    <p>To provide a framework for understanding and comparing epilepsy syndromes</p> Signup and view all the answers

    What is the primary purpose of the revised operational classification of seizure types presented by the International League Against Epilepsy?

    <p>To recognize seizure types that can have either a focal or generalized onset</p> Signup and view all the answers

    What is the significance of the term 'focal' in the revised operational classification of seizure types?

    <p>It is used to describe seizure types that originate at a specific point in the brain</p> Signup and view all the answers

    What is the significance of the elimination of the terms 'dyscognitive', 'simple partial', 'complex partial', 'psychic', and 'secondarily generalized' in the revised classification?

    <p>It simplifies the language used in the classification system</p> Signup and view all the answers

    What is the relationship between the 2017 Classification and the 1981 Classification?

    <p>The 2017 Classification is an extension of the 1981 Classification</p> Signup and view all the answers

    What is the primary difference between the 2017 Classification and the 1981 Classification?

    <p>The 2017 Classification adopts more transparent names for seizure types</p> Signup and view all the answers

    What is the significance of the term 'awareness' in the revised operational classification of seizure types?

    <p>It is used to classify focal seizures</p> Signup and view all the answers

    What is the primary goal of the revised operational classification of seizure types?

    <p>To provide a practical and usable classification system</p> Signup and view all the answers

    What is the significance of the revised operational classification of seizure types for clinical practice?

    <p>It provides a more accurate and precise way to diagnose and classify seizure types</p> Signup and view all the answers

    What is the primary reason for the revised operational classification of seizure types?

    <p>To improve the clarity of nomenclature and classification</p> Signup and view all the answers

    What is the clinician's first task when encountering a seizure event?

    <p>To determine that the event has the characteristics of a seizure</p> Signup and view all the answers

    What is the key characteristic of a seizure?

    <p>A transient occurrence of signs and symptoms</p> Signup and view all the answers

    What is the main difference between the revised operational classification and the 1981 classification?

    <p>The revised classification is more focused on clarity of nomenclature and classification</p> Signup and view all the answers

    What is the purpose of the operational classification of seizure types?

    <p>To provide a framework for clinicians to classify seizures</p> Signup and view all the answers

    How are seizures classified according to the revised operational classification?

    <p>Into focal, generalized, and unknown onset seizures</p> Signup and view all the answers

    What is the significance of the revised operational classification of seizure types?

    <p>It improves the clarity of nomenclature and classification</p> Signup and view all the answers

    What is the primary reason why retrospective seizure descriptions often do not specify a level of consciousness?

    <p>The concept of consciousness is too complicated.</p> Signup and view all the answers

    What is the primary goal of the revised operational classification of seizure types?

    <p>To improve the classification of seizures</p> Signup and view all the answers

    What is the consequence of impaired awareness during any part of the seizure?

    <p>It is classified as a focal impaired awareness seizure.</p> Signup and view all the answers

    What is the reason why some terms in current use lack high levels of community acceptance or public understanding?

    <p>They are not clearly defined or explained.</p> Signup and view all the answers

    What is the consequence of both motor and non-motor signs being present at the seizure start?

    <p>The motor signs will usually dominate, unless non-motor symptoms are prominent.</p> Signup and view all the answers

    What is the primary limitation of the 1981 system?

    <p>It does not include all seizure types.</p> Signup and view all the answers

    What is the characteristic of focal impaired awareness seizures?

    <p>They are characterized by impaired awareness during the seizure.</p> Signup and view all the answers

    What is the reason why some seizure types are not included in the classification system?

    <p>They are not well understood.</p> Signup and view all the answers

    What is the consequence of lack of knowledge about the onset of seizures?

    <p>It makes the seizure unclassifiable and difficult to discuss.</p> Signup and view all the answers

    What is the significance of the 2017 classification in epilepsy?

    <p>It allows for the appending of qualifiers to seizures of unknown onset</p> Signup and view all the answers

    What is the difference between an 'absent stare' and an absence seizure?

    <p>An 'absent stare' is a symptom of absence seizures, but not synonymous with it</p> Signup and view all the answers

    What is the purpose of video-EEG recordings in epilepsy diagnosis?

    <p>To show focal onsets of seizures</p> Signup and view all the answers

    Why is retrospective determination of state of consciousness difficult in epilepsy?

    <p>Because awareness and responsiveness can be difficult to measure</p> Signup and view all the answers

    What is the significance of the 1981 classification in epilepsy?

    <p>It established the importance of awareness and responsiveness in seizure classification</p> Signup and view all the answers

    What is the significance of clinicians being aware that so-called generalized spike-waves do not manifest equally in all parts of the brain?

    <p>It emphasizes the complexity of seizure classification and the need for careful consideration</p> Signup and view all the answers

    What is the significance of the concept of 'unknown onset' in epilepsy?

    <p>It allows for the appending of qualifiers to seizures of unknown onset</p> Signup and view all the answers

    What is the primary difference between seizures of unknown onset and generalized onset seizures?

    <p>Seizures of unknown onset can be presumed even if they are unwitnessed, while generalized onset seizures cannot</p> Signup and view all the answers

    What is the primary goal of the ILAE Classification of the Epilepsies?

    <p>To provide a framework for diagnosis and treatment of epilepsy</p> Signup and view all the answers

    What is the name of the scale used to investigate impaired consciousness in epilepsy?

    <p>Responsiveness in Epilepsy Scale (RES)</p> Signup and view all the answers

    What is the term for the update on a slowly emerging epileptic syndrome characterized by transient amnesia?

    <p>Transient Epileptic Amnesia</p> Signup and view all the answers

    What is the purpose of the ILAE Task Force on ICD codes in epilepsy?

    <p>To revise the ICD codes for epilepsy and seizures</p> Signup and view all the answers

    Study Notes

    Classification of Epilepsies

    • Purpose: for clinical diagnosis
    • Uses transparent language: words that mean what they say

    Seizure Types

    • Focal: originates within networks limited to one hemisphere, may be discretely localized or more widely distributed
    • Generalized: originates at some point within and rapidly engages bilaterally distributed networks, can include cortical and subcortical structures
    • Unknown: unclear or unspecified onset

    Epilepsy Types

    • Focal: combined focal and generalized epilepsies, examples include Dravet syndrome
    • Generalized: includes Genetic Generalized Epilepsies, formerly known as Idiopathic Generalized Epilepsies (e.g. Childhood Absence Epilepsy, Juvenile Absence Epilepsy)
    • Unknown: unclear or unspecified etiology

    Etiology

    • Structural: related to brain structure
    • Genetic: related to genetic predisposition, but not necessarily inherited
    • Infectious: related to infection
    • Metabolic: related to metabolic issues
    • Immune: related to immune system
    • Unknown: unclear or unspecified etiology

    Epilepsy Syndromes

    • No approved ILAE epilepsy syndromes
    • Examples include Temporal Lobe Epilepsy, Generalized Tonic-Clonic Seizures in a 5-year-old with generalized spike-wave

    Seizure Classification

    • Focal and generalized seizures can be further classified as aware or impaired awareness
    • Atonic seizures and epileptic spasms do not have level of awareness specified
    • Cognitive seizures include impaired language, other cognitive domains, and positive features like déjà vu, hallucinations, and perceptual distortions
    • Emotional seizures include anxiety, fear, joy, etc.

    Terminology Changes

    • Old terms no longer in use: complex partial, simple partial, partial, psychic, dyscognitive, secondarily generalized tonic-clonic
    • Replaced terms: Self-limited, Pharmacoresponsive, Developmental and/or Epileptic Encephalopathies
    • No longer use: benign, malignant, catastrophic

    Developmental and/or Epileptic Encephalopathies

    • Epileptic activity itself contributes to severe cognitive and behavioral impairment above and beyond that expected from the underlying pathology
    • Developmental component independent of the epileptic encephalopathy, may begin in utero or post-birth
    • Examples include KCNQ2 and STXBP1 encephalopathies

    ILAE Classification of the Epilepsies

    • Simplified framework, etiology considered at all stages
    • Developmental and/or Epileptic Encephalopathies
    • Self-limited, pharmacoresponsive
    • Genetic Generalized Epilepsies
    • Symptomatic Generalized Epilepsies replaced with Developmental and Epileptic Encephalopathies and (static) Encephalopathy with Epilepsy

    Classification of Epilepsies

    • Purpose: for clinical diagnosis
    • Uses transparent language: words that mean what they say

    Seizure Types

    • Focal: originates within networks limited to one hemisphere, may be discretely localized or more widely distributed
    • Generalized: originates at some point within and rapidly engages bilaterally distributed networks, can include cortical and subcortical structures
    • Unknown: unclear or unspecified onset

    Epilepsy Types

    • Focal: combined focal and generalized epilepsies, examples include Dravet syndrome
    • Generalized: includes Genetic Generalized Epilepsies, formerly known as Idiopathic Generalized Epilepsies (e.g. Childhood Absence Epilepsy, Juvenile Absence Epilepsy)
    • Unknown: unclear or unspecified etiology

    Etiology

    • Structural: related to brain structure
    • Genetic: related to genetic predisposition, but not necessarily inherited
    • Infectious: related to infection
    • Metabolic: related to metabolic issues
    • Immune: related to immune system
    • Unknown: unclear or unspecified etiology

    Epilepsy Syndromes

    • No approved ILAE epilepsy syndromes
    • Examples include Temporal Lobe Epilepsy, Generalized Tonic-Clonic Seizures in a 5-year-old with generalized spike-wave

    Seizure Classification

    • Focal and generalized seizures can be further classified as aware or impaired awareness
    • Atonic seizures and epileptic spasms do not have level of awareness specified
    • Cognitive seizures include impaired language, other cognitive domains, and positive features like déjà vu, hallucinations, and perceptual distortions
    • Emotional seizures include anxiety, fear, joy, etc.

    Terminology Changes

    • Old terms no longer in use: complex partial, simple partial, partial, psychic, dyscognitive, secondarily generalized tonic-clonic
    • Replaced terms: Self-limited, Pharmacoresponsive, Developmental and/or Epileptic Encephalopathies
    • No longer use: benign, malignant, catastrophic

    Developmental and/or Epileptic Encephalopathies

    • Epileptic activity itself contributes to severe cognitive and behavioral impairment above and beyond that expected from the underlying pathology
    • Developmental component independent of the epileptic encephalopathy, may begin in utero or post-birth
    • Examples include KCNQ2 and STXBP1 encephalopathies

    ILAE Classification of the Epilepsies

    • Simplified framework, etiology considered at all stages
    • Developmental and/or Epileptic Encephalopathies
    • Self-limited, pharmacoresponsive
    • Genetic Generalized Epilepsies
    • Symptomatic Generalized Epilepsies replaced with Developmental and Epileptic Encephalopathies and (static) Encephalopathy with Epilepsy

    ILAE Classification of the Epilepsies

    • The International League Against Epilepsy (ILAE) has updated its classification of the epilepsies to reflect advances in understanding of the epilepsies and their underlying mechanisms.
    • The revised framework aims to assist in improving epilepsy care and research in the 21st century.

    Purpose of Classification

    • Classification provides a framework for understanding the type of seizures a patient has, other seizure types that are likely to occur, potential triggers, and prognosis.
    • It serves clinical and basic epilepsy research, development of novel therapies, and communication worldwide.

    Key Points

    • The ILAE presents a revised framework for the classification of the epilepsies, designed to work with transparent terminology.
    • The emphasis is on employing words that mean what they say.
    • The 2010 publication triggered extensive discussion and commentaries.

    Classification Framework

    • The framework consists of three levels: Epilepsy Syndrome, Epilepsy Type, and Epilepsy with Comorbidity.
    • Epilepsy Syndrome is a diagnosis based on the 2014 definition of epilepsy.
    • Epilepsy Type includes categories such as Generalized Epilepsy, Focal Epilepsy, and Combined Generalized and Focal Epilepsy.
    • Epilepsy with Comorbidity includes learning, psychological, and behavioral problems.

    Epilepsy Type

    • Generalized Epilepsy is diagnosed based on generalized spike-wave activity on EEG.
    • Individuals with generalized epilepsies may have a range of seizure types, including absence, myoclonic, atonic, tonic, and tonic-clonic seizures.
    • Focal Epilepsy may be diagnosed based on focal EEG abnormalities.

    Unknown Etiology

    • Unknown means that the cause of the epilepsy is not known.
    • In this category, it is not possible to make a specific diagnosis apart from the basic electroclinical semiology.

    Comorbidities

    • Many epilepsies are associated with comorbidities such as learning, psychological, and behavioral problems.
    • Modern technology calls for a modern approach to classification of epileptic seizures and the epilepsies.

    Epileptic Encephalopathy

    • Epileptic encephalopathy is a condition where abundant epileptiform activity interferes with development, resulting in cognitive slowing and often regression.
    • The epileptiform activity can cause regression in an individual with normal development or preexisting developmental delay.
    • Amelioration of the epileptiform activity may have the potential to improve the developmental consequences of the disorder.

    ILAE Position Paper: Operational Classification of Seizure Types

    • The International League Against Epilepsy (ILAE) presents a revised operational classification of seizure types, recognizing that some seizure types can have either a focal or generalized onset.
    • The purpose of the revision is to:
      • Recognize that some seizure types can have either a focal or generalized onset
      • Allow classification when the onset is unobserved
      • Include some missing seizure types
      • Adopt more transparent names

    Changes to the Classification

    • "Partial" becomes "focal"
    • Awareness is used as a classifier of focal seizures
    • The terms "dyscognitive", "simple partial", "complex partial", "psychic", and "secondarily generalized" are eliminated
    • Seizures are divided into those of focal, generalized, and unknown onset, with subcategories of motor and non-motor signs and symptoms

    Key Concepts

    • A seizure is defined as "a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain"
    • The clinician's first task is to determine that an event has the characteristics of a seizure and not one of the many imitators of seizures
    • Focal impaired awareness seizures are characterized by impaired awareness during any part of the seizure
    • Focal seizures can be subgrouped as those with motor and nonmotor signs and symptoms

    Classification of Seizures with Unknown Onset

    • The 2017 classification allows appending of a limited number of qualifiers to seizures of unknown onset, in order to better characterize the seizure
    • If the epilepsy type is known, the onset can be presumed even if it is unwitnessed

    Importance of Awareness and Responsiveness

    • Awareness and responsiveness are important classifiers of seizure types
    • Retrospective determination of state of consciousness can be difficult
    • A sense of self as distinct from others is an important aspect of awareness
    • Measurements of awareness, responsiveness, memory, and a sense of self are important in classifying seizure types

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    Related Documents

    ILAE 2017 Classification of Seizures PDF
    ILAE Classification of the Epilepsies Position Paper PDF
    ILAE Operational Classification of Seizure Types 2017 PDF

    Description

    This quiz covers the classification of epilepsies for clinical diagnosis, including seizure types and etiology. It's designed to help medical professionals understand and identify different types of epilepsy.

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