Classification of the Epilepsies

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)
• absence seizures
  • typical
  • atypical
  • absence with special features
    • myoclonic absence
    • eyelid myoclonia
  • Myoclonic
    • myoclonic
    • myoclonic atonic
    • myoclonic tonic
• Clonic
• Tonic
• Atonic

Focal seizures
Unknown
Epileptic spasms

• Electroclinical syndromes arranged by age at onset
  • Neonatal period
    • Benign familial neonatal epilepsy (BFNE)
    • Early myoclonic encephalopathy (EME)
    • Ohtahara syndrome
  • Infancy
    • Epilepsy of infancy with migrating focal seizures
    • West syndrome
    • Myoclonic epilepsy in infancy (MEI)
    • benign infantile epilepsy
    • benign familial infantile epilepsy
    • Dravet syndrome
    • Myoclonic encephalopathy in nonprogressive disorders
  • Childhood
    • Febrile seizures plus (FS+)^a
    • Panayiotopoulos syndrome
    • epilepsy with myoclonic atonic seizures^b
    • Benign epilesy with centrotemporal spikes (BECTS)
    • autosomal dominant frontal lobe epilepsy (ADNFLE)
    • late onsert childhood occipital epilepsy (Gastaut type)
    • epilepsy with myoclonic absences
    • Lennox-Gastuat syndrome
    • epileptic encephalopathy with continous spike and wave during sleep(csws)
    • Landau-Kleffner syndrome (LKS)
    • childhood absence epilepsy (CAE)
  • Adolescence – adult
    • juvenile absence epilepsy (JAE)
    • juvenile myoclonic epilepsy (JME)
    • 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

Footnotes