Table of Contents
- Introduction
- Evolution of Classification
- Table 1: Expanded version of 2017 International League Against Epilepsy seizure type classification
- Table 2: Focal vs Generalized vs Combined Onset in Epilepsy Classification
- Table 3: Classification of Epilepsy Syndromes
- Table 4: Etiological Classification of Epilepsy
- Further Reading
- Bibliography
Primary Category
Epilepsy
P-Category
Secondary Category
S-Category
Introduction
- The 2017 ILAE classification of seizures, epilepsies, and epilepsy syndromes aims to group
- seizures according to
- clinical presentation and its origin in the brain. and
- epilepsies according to
- seizure type, age of onset, probability of remission, EEG findings, radiologic findings, and genetics.
- An emphasis is now placed on etiology.
- The epilepsy syndrome is a new addition to the current classification system and is defined as
- “a cluster of features incorporating seizure types, EEG, and imaging that tend to occur together”.
- Factors that contribute to epilepsy syndrome include
- Age of onset
- Remission
- Triggers
- Diurnal variation
- Intellectual and psychiatric dysfunction
- EEG findings
- Imaging studies
- Family history and genetics.
Evolution of Classification
The International League Against Epilepsy (ILAE) over the years has evolved the classification of epilepsy.
These are highlights
- 1981: Difference between partial or generalized seizures.
- 1985: Difference between idiopathic or symptomatic.
- 1989: Added cryptogenic epilepsies and termed them symptomatic.
- 2005: Updated seizure definition is a “transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain”
- 2014: ILAE redefined epilepsy as a “disease” and not as a“disorder”
- Epilepsy is two unprovoked seizure events.
- Epilepsy is diagnosed if an individual has seizure and his or her “brain demonstrates a pathologic tendency to have one unprovoked or reflex recurrent seizures.”
- After the initial event the probability was considered to be at least 60% within the next 10 years.
Table 1: Expanded version of 2017 International League Against Epilepsy seizure type classification
Classification | Focal Onset | Generalised Onset | Unknown Onset | Unclassified Onset |
Description | Originate from one hemisphere that may be localised or widely distributed. | Originates from more than one region of the brain. | Unknown onset | Seizure not under any of the previous classification |
Awareness | Awake/Impaired awareness | Impaired | Awake/Impaired awareness | These seizures do not fall in a specific category or possible with new genetic and radiology information. Hence ILAE left an option for unclassified epilepsies. |
Motor | - Tonic
- Atonic
- Clonic
- Myoclonic
- Automatisms
- Epileptic Spasms
- Hyperkinetic | - Tonic
- Clonic
- Tonic -Clonic
- Atonic
- Myoclonic
- Myoclonic-tonic-clonic
- Myoclonic-atonic
- Epileptic spasms | - Tonic-clonic
- Epileptic spasms | ㅤ |
Nonmotor onset | - Autonomic
- Cognitive
- Behavior Arrest
- Emotional
- Sensory | - Typical
- Atypical
- Myoclonic
- Eyelid Myoclonia | ㅤ | ㅤ |
Evolution | Focal to bilateral
Tonic-Clonic | ㅤ | ㅤ | ㅤ |
Derived From: Fisher, Robert S. et al. “Operational Classification of Seizure Types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology.” Epilepsia 58.4 (2017): 522–530. Web.
Table 2: Focal vs Generalized vs Combined Onset in Epilepsy Classification
Classification | Focal Onset | Generalised Onset | Combined Onset |
Description | One or more of the generalised seizures | One or more of the focal seizures | Combined generalised and focal epilepsy |
Types | - Unifocal
- Multifocal | - Myoclonic Jerks | - Lennox - Gastaut Syndrome
- Dravet Syndrome |
EEG | Focal slowing or epileptiform discharges | Generalised spike-wave activity | EEG findings of both generalised and focal epilepsy |
Derived From: Fisher, Robert S. et al. “Operational Classification of Seizure Types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology.” Epilepsia 58.4 (2017): 522–530. Web.
Table 3: Classification of Epilepsy Syndromes
Epilepsy Syndromes | Idiopathic or Genetic Syndrome | Reflux Syndrome | Well-described Focal Syndrome |
Description | Genetic or self | Seizures are provoked by a specific stimulus | Childhood epilepsy with centrotemporal spikes and panayiotopoulos syndrome. |
Types | - Childhood Absence
- Juvenile Absence
- Juvenile Myoclonic
- Generalised Tonic-clonic | - Generalised tonic-clonic
- Photosensitive
- Reading
- Startle | - Focal motor hemifacial
- Nocturnal focal motor evolving to bilateral tonic-clonic seizures.
- Focal autonomic seizures. |
Derived From: Fisher, Robert S. et al. “Operational Classification of Seizure Types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology.” Epilepsia 58.4 (2017): 522–530. Web.
Table 4: Etiological Classification of Epilepsy
Genetic | Genetic mutation either inherited or new, family history or syndromes |
Infectious | Meningitis and Encephalitis-Neurocysticercosis, HIV, Cytomegalovirus, and cerebral toxoplasmosis |
Metabolic | Secondary to a known metabolic disorder with seizures as one of the main symptom |
Immune | anti-N-methyl-D-aspartate, anti-leucine-rich, glioma inactivated 1 encephalitis |
Structural | Stroke, trauma, tumour, malformations of cortical development, and infection |
Unknown | No clear etiology |
Derived From: Fisher, Robert S. et al. “Operational Classification of Seizure Types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology.” Epilepsia 58.4 (2017): 522–530. Web.
Further Reading
- Pack AM. Epilepsy Overview and Revised Classification of Seizures and Epilepsies. Continuum (Minneap Minn). 2019 Apr;25(2):306-321. doi: 10.1212/CON.0000000000000707. PMID: 30921011.
- Fisher, Robert S. et al. “Operational Classification of Seizure Types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology.” Epilepsia 58.4 (2017): 522–530. Web.
Bibliography
- Fisher RS, Cross JH, French JA, Higurashi N, Hirsch E, Jansen FE, Lagae L, Moshé SL, Peltola J, Roulet Perez E, Scheffer IE, Zuberi SM. Operational classification of seizure types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017 Apr;58(4):522-530. doi: 10.1111/epi.13670. Epub 2017 Mar 8. PMID: 28276060.
- Fisher RS, Cross JH, D'Souza C, French JA, Haut SR, Higurashi N, Hirsch E, Jansen FE, Lagae L, Moshé SL, Peltola J, Roulet Perez E, Scheffer IE, Schulze-Bonhage A, Somerville E, Sperling M, Yacubian EM, Zuberi SM. Instruction manual for the ILAE 2017 operational classification of seizure types. Epilepsia. 2017 Apr;58(4):531-542. doi: 10.1111/epi.13671. Epub 2017 Mar 8. PMID: 28276064.
- Scheffer IE, Berkovic S, Capovilla G, Connolly MB, French J, Guilhoto L, Hirsch E, Jain S, Mathern GW, Moshé SL, Nordli DR, Perucca E, Tomson T, Wiebe S, Zhang YH, Zuberi SM. ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017 Apr;58(4):512-521. doi: 10.1111/epi.13709. Epub 2017 Mar 8. PMID: 28276062; PMCID: PMC5386840.
- Proposal for revised clinical and electroencephalographic classification of epileptic seizures. From the Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia. 1981 Aug;22(4):489-501. doi: 10.1111/j.1528-1157.1981.tb06159.x. PMID: 6790275.
- Proposal for classification of epilepsies and epileptic syndromes. Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia. 1985 May-Jun;26(3):268-78. PMID: 3924589.
- Proposal for revised classification of epilepsies and epileptic syndromes. Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia. 1989 Jul-Aug;30(4):389-99. doi: 10.1111/j.1528-1157.1989.tb05316.x. PMID: 2502382.
- Berg AT, Berkovic SF, Brodie MJ, Buchhalter J, Cross JH, van Emde Boas W, Engel J, French J, Glauser TA, Mathern GW, Moshé SL, Nordli D, Plouin P, Scheffer IE. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005-2009. Epilepsia. 2010 Apr;51(4):676-85. doi: 10.1111/j.1528-1167.2010.02522.x. Epub 2010 Feb 26. PMID: 20196795.
- Fisher RS, Acevedo C, Arzimanoglou A, Bogacz A, Cross JH, Elger CE, Engel J Jr, Forsgren L, French JA, Glynn M, Hesdorffer DC, Lee BI, Mathern GW, Moshé SL, Perucca E, Scheffer IE, Tomson T, Watanabe M, Wiebe S. ILAE official report: a practical clinical definition of epilepsy. Epilepsia. 2014 Apr;55(4):475-82. doi: 10.1111/epi.12550. Epub 2014 Apr 14. PMID: 24730690.
- Thomas RH, Berkovic SF. The hidden genetics of epilepsy-a clinically important new paradigm. Nat Rev Neurol. 2014 May;10(5):283-92. doi: 10.1038/nrneurol.2014.62. Epub 2014 Apr 15. PMID: 24733163.
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Written by
Founder NeuroCare.AI, Practicing Neurologist, sub-specialized in the field of Neurocritical Care, Stroke & Epilepsy