The ILAE Classification of the Epilepsies was updated in 2017[1][2] to reflect the advances made in recent years in the understanding of the epilepsies and their pathophysiologies since the classification was last ratified in 1989[3]. The current classification is based on the 1981 classification[4] which was extended in 2010[5]. It addresses the three levels of (1) identifying epilepsy (2) identifying the epilepsy type and (3) identifying the epilepsy syndrome where possible. In addition at each level it takes into account the aetiological diagnosis.

Generalised seizures

  • Tonic-Clonic (in any combination)
    • typical
    • atypical
    • absence with special features
    • Myoclonic
      • myoclonic
      • myoclonic atonic
      • myoclonic tonic
  • Clonic
  • Tonic
  • Atonic

Focal seizures
Unknown
Epileptic spasms

  • Electroclinical syndromes arranged by age at onset
    • Neonatal period
    • Infancy
      • Epilepsy of infancy with migrating focal seizures
      • West syndrome
      • Myoclonic epilepsy in infancy (MEI)
      • benign infantile epilepsy
      • Dravet syndrome
      • Myoclonic encephalopathy in nonprogressive disorders
    • Childhood
    • Adolescence – adult
      • juvenile absence epilepsy (JAE)
      • Epilepsy with generalized tonic–clonic seizures alone
      • progressive myoclonic epilepsies (PME)
      • autosomal dominant epilepsy with auditory features (ADEAF)
      • Other familial temporal lobe epilepsies
    • Less specific age relationship
      • Familial focal epilepsy with variable foci (childhood to adult)
      • Reflex epilepsies
  • Distinctive constellations
    • Mesial Temporal Lobe Epilepsy withy Hippocampal Sclerosis (MTLE with HS)
    • Rasmussen syndrome
    • gelastic seizures with hypothalamic hamartoma
    • hemiconvulsion hemiplegia epilepsy
    • Epilepsies that do not fit into any of these diagnostic categories can be distinguished first on the basis of the presence or absence of a known structural or metabolic condition (presumed cause) and then on the basis of the primary mode of seizure onset (generalized vs. Focal)
    • Epilepsies attributed to and organized by structural-metabolic causes
    • Malformations of cortical development (hemimegalencephaly, etc.)
    • Neurocutaneous syndromes (tuberous sclerosis complex, etc.)
    • Tumor
    • Infection
    • Trauma
    • Angioma
    • Perinatal insults
    • Stroke Etc
  • Epilepsies of unknown cause
  • Conditions with epileptic seizures that are traditionally not diagnosed as a form of epilepsy per se
    • benign neonatal seizures (BNS)
    • febrile seizures (FS)

References

1. 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 Y, Zuberi SMILAE 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: 5386840] [DOI: 10.1111/epi.13709]
2. Fisher RS, Cross JH, French JA, Higurashi N, Hirsch E, Jansen FE, Lagae L, Moshé SL, Peltola J, Roulet Perez E, Scheffer IE, Zuberi SMOperational 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] [DOI: 10.1111/epi.13670]
3. 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] [DOI: 10.1111/j.1528-1157.1989.tb05316.x]
4. 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] [DOI: 10.1111/j.1528-1157.1981.tb06159.x]
5. 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 IERevised 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] [DOI: 10.1111/j.1528-1167.2010.02522.x]

Footnotes

a can start in infancy
bpreviously astatic seizures