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seizures in children.pptx
Classification of the Epilepsies
Purpose: for clinical diagnosis
Transparent language: use words that mean what they say
Unknown
Immune
Infectious
Structural
Etiology
Metabolic
Genetic
Co-morbidities
Epilepsy types
Focal Generalized
Combined
Generalized
& Focal
Unknown
Focal
Epilepsy Syndromes
Seizure types
Generalized
onset
Unknown
onset
Focal
onset
1. Seizure types
•Certain that events are epileptic seizures – not referring
to distinguishing epileptic versus non-epileptic
•In some settings  classification according to seizure
type may be maximum level of diagnosis possible
•In other cases  simply too little information to be able
to make a higher level diagnosis
•eg. when a patient has only had a single event
Seizure types
Generalized
onset
Unknown
onset
Focal
onset
Generalized seizures
• Originate at some point
within and rapidly engage
bilaterally distributed
networks
• Can include cortical and
subcortical structures
but not necessarily the
entire cortex
seizures in children.pptx
• Originate within
networks limited
to one hemisphere
• May be discretely
localized
or more widely
distributed.…
Focal seizures
seizures in children.pptx
Notes
• Atonic seizures and epileptic spasms would
not have level of awareness specified
• Pedalling grouped in hyperkinetic rather
than automatisms (arbitrary)
• Cognitive seizures
• impaired language
• other cognitive domains
• positive features eg déjà vu,
hallucinations, perceptual distortions
• Emotional seizures: anxiety, fear, joy, etc
seizures in children.pptx
seizures in children.pptx
Note
When a seizure type begins with ”focal,
generalized or absence” then the word
“onset” can be presumed
Terms no longer in use
• Complex partial
• Simple partial
• Partial
• Psychic
• Dyscognitive
• Secondarily generalized tonic-clonic
seizures in children.pptx
Note
Clarify features of seizures
but do not define unique
seizure types
Free text descriptors
encouraged
seizures in children.pptx
Etiology
Tuberous Sclerosis
GLUT1 deficiency
Unknown
Immune
Infectious
Structural
Metabolic
Genetic
Seizure types
Generalized
onset
Unknown
onset
Focal
onset
Epilepsy types
Focal Generalized
Combined
Generalized
& Focal
Unknown
Focal
• Where unable to make an Epilepsy Syndrome
diagnosis or a diagnosis of Etiology
• Many examples
– Temporal lobe epilepsy
– Generalized tonic-clonic seizures in a 5 year old with
generalized spike-wave
– Both focal impaired awareness seizures and absence
seizures in a patient
– Cannot tell if tonic-clonic seizure is focal or generalized
Generalized and Focal Epilepsies
• Combined focal and generalized epilepsies
Examples
–Dravet syndrome
• What do with
– Multifocal epilepsies?
– Hemispheric epilepsies?
 focal
 focal
Unknown
Immune
Infectious
Structural
Etiology
Metabolic
Genetic
Epilepsy types
Focal Generalized
Combined
Generalized
& Focal
Unknown
Focal
Epilepsy Syndromes
Seizure types
Generalized
onset
Unknown
onset
Focal
onset
Old term
‘Idiopathic Generalized Epilepsies’
Idiopathic Generalized
Epilepsies
Childhood
Absence
Epilepsy
Generalized
Tonic-Clonic
Seizures Alone
Juvenile
Absence
Epilepsy
Juvenile
Myoclonic
Epilepsy
Genetic versus idiopathic
• ‘Idiopathic’ = presumed hereditary predisposition
• Genetic ≠ inherited
– Importance of de novo mutations in both
mild and severe epilepsies
• Critical problem of stigma in some parts of the world
Genetic ≠ Gene testing
– Usually the mutation is not known
– Access to molecular genetic testing not necessary
– Diagnosed on clinical research eg. twin, family studies
JME pair; Lennox 1941 CAE pair; Lennox 1950
Co-morbidities
Etiology
Epilepsy types
Focal Generalized
Combined
Generalized
& Focal
Unknown
Focal
Epilepsy Syndromes
Generalized
Unknown
Immune
Infectious
Structural
Metabolic
Genetic
Genetic
Seizure types
Generalized
onset
Unknown
onset
Focal
onset
Epilepsy syndromes
• There are no approved ILAE
epilepsy syndromes
https://www.epilepsydiagnosis.org
seizures in children.pptx
seizures in children.pptx
seizures in children.pptx
seizures in children.pptx
Benign
• Many epilepsies not benign
– CAE – psychosocial impact
– BECTS – learning concerns
• Replaced by terms:
–Self-limited
–Pharmacoresponsive
• No longer use
– Malignant
– Catastrophic
Epileptic activity itself
contributes to severe cognitive and
behavioral impairment above and
beyond that expected from the
underlying pathology and that these
can worsen over time
Developmental and/or Epileptic encephalopathies
Berg et al 2010
Developmental and/or Epileptic Encephalopathy
• For many encephalopathies, there is a developmental
component independent of the epileptic encephalopathy
• Developmental delay may precede seizure onset
• Co-morbidities
eg. cerebral palsy, autism spectrum disorder, intellectual
disability
• Outcome poor even though seizures stop
eg. KCNQ2, STXBP1 encephalopathies
Developmental and/or Epileptic Encephalopathy
• Developmental encephalopathy
• May begin in utero
• Post birth
• Epileptic encephalopathy
• Can occur at any age
• May have remediable component – right vs wrong AED
• Move towards GENE encephalopathy
• eg. CDKL5 encephalopathy, SCN2A encephalopathy
Old terms
‘Symptomatic Generalized Epilepsies’
• Used for two different
groups of disorders
Symptomatic Generalized
Epilepsies
Developmental
and/or
Epileptic
Encephalopathies
(Static)
Encephalopathies
ILAE Classification of the Epilepsies
• Simplified the framework
• Etiology – consider at all stages
• Developmental and/or Epileptic Encephalopathies
• Self-limited, pharmacoresponsive
• Genetic Generalized Epilepsies
– Idiopathic Generalized Epilepsies = CAE, JAE, JME, GTCA
• Symptomatic Generalized Epiliepsies used for both
 Developmental and Epileptic Encephalopathies
 (static) Encephalopathy with Epilepsy
Impact on Clinical Care and Practice
• New classification framework will
• Change the approach to diagnosis in the clinic
• Be applied to patients and guide management
• Updates terminology to reflect
current thinking
• Scientific advances
ILAE Classification Task Force 2013-7
Torbjörn Tomson, Emilio Perucca, Ingrid Scheffer, Jackie French, Yue-Hua Zhang
Satish Jain, Gary Mathern, Sam Wiebe, Edouard Hirsch, Sameer Zuberi, Nico Moshe

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seizures in children.pptx

  • 2. Classification of the Epilepsies Purpose: for clinical diagnosis Transparent language: use words that mean what they say
  • 3. Unknown Immune Infectious Structural Etiology Metabolic Genetic Co-morbidities Epilepsy types Focal Generalized Combined Generalized & Focal Unknown Focal Epilepsy Syndromes Seizure types Generalized onset Unknown onset Focal onset
  • 4. 1. Seizure types •Certain that events are epileptic seizures – not referring to distinguishing epileptic versus non-epileptic •In some settings  classification according to seizure type may be maximum level of diagnosis possible •In other cases  simply too little information to be able to make a higher level diagnosis •eg. when a patient has only had a single event
  • 6. Generalized seizures • Originate at some point within and rapidly engage bilaterally distributed networks • Can include cortical and subcortical structures but not necessarily the entire cortex
  • 8. • Originate within networks limited to one hemisphere • May be discretely localized or more widely distributed.… Focal seizures
  • 10. Notes • Atonic seizures and epileptic spasms would not have level of awareness specified • Pedalling grouped in hyperkinetic rather than automatisms (arbitrary) • Cognitive seizures • impaired language • other cognitive domains • positive features eg déjà vu, hallucinations, perceptual distortions • Emotional seizures: anxiety, fear, joy, etc
  • 13. Note When a seizure type begins with ”focal, generalized or absence” then the word “onset” can be presumed
  • 14. Terms no longer in use • Complex partial • Simple partial • Partial • Psychic • Dyscognitive • Secondarily generalized tonic-clonic
  • 16. Note Clarify features of seizures but do not define unique seizure types Free text descriptors encouraged
  • 19. Epilepsy types Focal Generalized Combined Generalized & Focal Unknown Focal • Where unable to make an Epilepsy Syndrome diagnosis or a diagnosis of Etiology • Many examples – Temporal lobe epilepsy – Generalized tonic-clonic seizures in a 5 year old with generalized spike-wave – Both focal impaired awareness seizures and absence seizures in a patient – Cannot tell if tonic-clonic seizure is focal or generalized
  • 20. Generalized and Focal Epilepsies • Combined focal and generalized epilepsies Examples –Dravet syndrome • What do with – Multifocal epilepsies? – Hemispheric epilepsies?  focal  focal
  • 21. Unknown Immune Infectious Structural Etiology Metabolic Genetic Epilepsy types Focal Generalized Combined Generalized & Focal Unknown Focal Epilepsy Syndromes Seizure types Generalized onset Unknown onset Focal onset
  • 22. Old term ‘Idiopathic Generalized Epilepsies’ Idiopathic Generalized Epilepsies Childhood Absence Epilepsy Generalized Tonic-Clonic Seizures Alone Juvenile Absence Epilepsy Juvenile Myoclonic Epilepsy
  • 23. Genetic versus idiopathic • ‘Idiopathic’ = presumed hereditary predisposition • Genetic ≠ inherited – Importance of de novo mutations in both mild and severe epilepsies • Critical problem of stigma in some parts of the world
  • 24. Genetic ≠ Gene testing – Usually the mutation is not known – Access to molecular genetic testing not necessary – Diagnosed on clinical research eg. twin, family studies JME pair; Lennox 1941 CAE pair; Lennox 1950
  • 25. Co-morbidities Etiology Epilepsy types Focal Generalized Combined Generalized & Focal Unknown Focal Epilepsy Syndromes Generalized Unknown Immune Infectious Structural Metabolic Genetic Genetic Seizure types Generalized onset Unknown onset Focal onset
  • 26. Epilepsy syndromes • There are no approved ILAE epilepsy syndromes
  • 32. Benign • Many epilepsies not benign – CAE – psychosocial impact – BECTS – learning concerns • Replaced by terms: –Self-limited –Pharmacoresponsive • No longer use – Malignant – Catastrophic
  • 33. Epileptic activity itself contributes to severe cognitive and behavioral impairment above and beyond that expected from the underlying pathology and that these can worsen over time Developmental and/or Epileptic encephalopathies Berg et al 2010
  • 34. Developmental and/or Epileptic Encephalopathy • For many encephalopathies, there is a developmental component independent of the epileptic encephalopathy • Developmental delay may precede seizure onset • Co-morbidities eg. cerebral palsy, autism spectrum disorder, intellectual disability • Outcome poor even though seizures stop eg. KCNQ2, STXBP1 encephalopathies
  • 35. Developmental and/or Epileptic Encephalopathy • Developmental encephalopathy • May begin in utero • Post birth • Epileptic encephalopathy • Can occur at any age • May have remediable component – right vs wrong AED • Move towards GENE encephalopathy • eg. CDKL5 encephalopathy, SCN2A encephalopathy
  • 36. Old terms ‘Symptomatic Generalized Epilepsies’ • Used for two different groups of disorders Symptomatic Generalized Epilepsies Developmental and/or Epileptic Encephalopathies (Static) Encephalopathies
  • 37. ILAE Classification of the Epilepsies • Simplified the framework • Etiology – consider at all stages • Developmental and/or Epileptic Encephalopathies • Self-limited, pharmacoresponsive • Genetic Generalized Epilepsies – Idiopathic Generalized Epilepsies = CAE, JAE, JME, GTCA • Symptomatic Generalized Epiliepsies used for both  Developmental and Epileptic Encephalopathies  (static) Encephalopathy with Epilepsy
  • 38. Impact on Clinical Care and Practice • New classification framework will • Change the approach to diagnosis in the clinic • Be applied to patients and guide management • Updates terminology to reflect current thinking • Scientific advances
  • 39. ILAE Classification Task Force 2013-7 Torbjörn Tomson, Emilio Perucca, Ingrid Scheffer, Jackie French, Yue-Hua Zhang Satish Jain, Gary Mathern, Sam Wiebe, Edouard Hirsch, Sameer Zuberi, Nico Moshe

Editor's Notes

  1. Use words that mean what they say!! Target audience – not epileptologists GPs, medical students, paediatricians, neurologists, internists, psychiatrists, health workers, nurses, etc > 80% of medical population
  2. Here is a diagram that shows a conceptual network for generalized seizures involving the corticothalamic circuitry. Theoretically a generalized seizure could start at different points in the network and engage bilaterally distributed networks. Thus a seizure could start frontally or even parietally.The key point is that a generalized seizure can start from a focal point.
  3. Conceptual diagram with fMRI of GSW network
  4. Awareness means person is aware of self and envt through sz even if immobile. Earliest prominent feature defines the sz type which might then progress to other signs and sx Impaired awareness at any time during a focal onset sz renders it a FIAS Specific motor and non motor classifiers can be added.
  5. Classify aware or impaired awareness at any time during the sz then optionally add motor or non motor onset feature reflecting earliest motor or non motor sign or symptom other than awareness In some settings may not want to comment on awareness eg neonate so can omit this and classify by earliest motor or non motor feature Free text can be added to characterise sz
  6. Classify aware or impaired awareness at any time during the sz then optionally add motor or non motor onset feature reflecting earliest motor or non motor sign or symptom other than awareness In some settings may not want to comment on awareness eg neonate so can skip this Free text can be added to characterise sz
  7. Classify aware or impaired awareness at any time during the sz then optionally add motor or non motor onset feature reflecting earliest motor or non motor sign or symptom other than awareness In some settings may not want to comment on awareness eg neonate so can skip this Free text can be added to characterise sz
  8. Free text can be added to characterise sz
  9. Laterality is an important descriptor
  10. Often a diagnosis regarding the type of epilepsy can be made (level 2: epilepsy classified by seizure type) and clinicians should strive to make a diagnosis at this level wherever possible. Added categories of “generalized and focal epilepsy” and “unknown if generalized or focal epilepsy”
  11. Increasingly observed in clinical practice Previously incorrectly allocated to unknown