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Journal of the Formosan Medical Association (2017) xx, 1e6

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Review

Classifications of seizures and epilepsies,

where are we? e A brief historical review
and update
Richard Shek-kwan Chang a,*, Chun Yin William Leung a,
Chi Chung Alvin Ho b, Ada Yung b

a
Department of Medicine, Queen Mary Hospital, University of Hong Kong, Hong Kong Special
Administrative Region
b
Department of Paediatrics & Adolescent Medicine, Queen Mary Hospital, University of Hong Kong,
Hong Kong Special Administrative Region

Received 19 February 2017; received in revised form 31 May 2017; accepted 6 June 2017

KEYWORDS Abstract In March 2017, the International League Against Epilepsy (ILAE) announced their
Classification; new classifications of seizures and epilepsies. Development of these classification systems
Seizure; led by the ILAE is a long and complicated process. Outsiders may find it difficult to understand
Epilepsy; the arguments behind. We summarize the major developmental milestones of the ILAE classi-
ILAE; fication schemata. An update of the latest classification is also included. It is hope that this
Taxonomy review can serve as an outline in learning the taxonomy in epileptology.
Copyright 2017, Formosan Medical Association. Published by Elsevier Taiwan LLC. This is an
open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-
nc-nd/4.0/).

Introduction and Terminology is a group of experts responsible to review

The establishment of seizure and epilepsy classification
systems has been a long journey and full of controversies.
The International League Against Epilepsy (ILAE) which is
the largest academic body in epilepsy community has been
taking a leading role. The ILAE Commission on Classification
* Corresponding author. 4th Floor, Professorial Block, Queen Mary
Hospital, Pokfulam, Hong Kong Special Administrative Region. Fax:
852 22555322.
E-mail address: changsk@ha.org.hk (R.S.-k. Chang).
the classification systems regularly.1 Their proposals have
been adopted globally due to their authoritativeness and
effectiveness in clinical practice. New schemes have been
published in recent years and some of them have provoked
a lot of debate. Many people, including health care pro-
fessionals, who are not specialized in epileptology may
encounter difficulties in following the rationales behind.
Just like learning other diseases, nosology is the entrance
to epileptology. This review is aimed to briefly summarize
the history of ILAE classification development in the recent
decades and provide an update on the latest progress.

http://dx.doi.org/10.1016/j.jfma.2017.06.001
0929-6646/Copyright 2017, Formosan Medical Association. Published by Elsevier Taiwan LLC. This is an open access article under the CC
BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Please cite this article in press as: Chang RS-k, et al., Classifications of seizures and epilepsies, where are we? e A brief historical review
and update, Journal of the Formosan Medical Association (2017), http://dx.doi.org/10.1016/j.jfma.2017.06.001
 + MODEL
2 R.S.-k. Chang et al.
The 1981 international classification of
epileptic seizures (ICES)
The ILAE published a classification of epileptic seizures, the
ICES, in 1981.2 Thanks to the advance in technology,
particularly the video recording with simultaneous elec-
troencephalogram (EEG) technique, seizures could be
studied sophisticatedly by epileptologists around the world.
In continuation of the previous efforts by ILAE in 60se70s,
an important classification scheme of epileptic seizures was
announced.3,4 The essence was to dichotomize epileptic
seizures into partial and generalized groups (Table 1).
The partial seizure was defined as an epileptic seizure in
which the first clinical and EEG changes indicate initial
activation of a system of neurons limited to part of one
cerebral hemisphere. Partial seizures were further classi-
fied into three groups according to whether consciousness is
maintained. They included the 1) simple partial seizure
(SPS); 2) complex partial seizure (CPS); and 3) partial
seizure evolving to generalized convulsion. The SPS
referred to an attack with retained consciousness. They
were further described into four groups according to the
ictal signs and symptoms. They included 1) motor signs,
such as focal motor with or without Jacksonian march; 2)
somatosensory or special sensory symptoms, such as visual,
olfactory or gustatory; 3) autonomic features, such as
epigastric sensation, piloerection or pupillary dilatation;
and 4) psychic symptoms, for example dysphasia, affective
symptoms, illusions or hallucinations. The ictal EEG of a SPS
usually showed focal abnormal discharges over the corre-
sponding area of cortical representation on the contralat-
eral side. The CPS was an attack with impaired
consciousness. The loss of consciousness could develop at
the seizure onset or evolve from a SPS. The EEG in the CPS
could show bilateral abnormal discharges especially after
the initial ictal discharges had propagated from epileptic
focus. The partial seizure evolving to generalized seizure or
commonly known as secondarily generalized seizure
referred to any partial seizures that subsequently involved
both cerebral hemispheres, resulting in generalized
seizures.
The generalized seizure was defined as a seizure in
which the first clinical changes indicate initial involvement
of both hemispheres. Six types of generalized were
described. They were 1) absence; 2) myoclonic; 3) clonic;
4) tonic; 5) tonic-clonic; and 6) tonic seizures. Any motor
manifestations in generalized seizures usually manifested
Table 1 Seizure classification of 1981 proposal for revised
clinical and electroencephalographic classification of
epileptic seizures.
Partial seizures Simple partial
Complex partial
Generalized seizures Absence
Myoclonic
Clonic
Tonic
Tonic-clonic
Atonic
Please cite this article in press as: Chang RS-k, et al., Classifications of seizures and epilepsies, where are we? e A brief historical review
and update, Journal of the Formosan Medical Association (2017), http://dx.doi.org/10.1016/j.jfma.2017.06.001
bilaterally. Consciousness was impaired. Each generalized
seizure subtype had their own characteristic EEG features
with bilateral ictal onset. Typical examples were regular
and symmetrical 3 Hz spike-and-slow-wave complexes in
the absence seizure and polyspikes in the myoclonic
seizure.
The ILAE 1981 ICES has laid the cornerstone of contem-
porary seizure classification. It has been widely accepted as
an effective clinical tool and is still commonly adopted
nowadays.
The 1985 and 1989 international
classifications of epilepsies and epileptic
syndromes (ICE)
In 1985, the ILAE published a proposal of epilepsy classifi-
cation, the ICE.5 It is a dual dichotomies scheme. In terms
of semiology, it divided epilepsies into those with focal
seizures versus generalized seizures. In terms of etiology, it
divides epilepsies into idiopathic versus symptomatic.
Idiopathic referred to absence of well-defined etiology
except possible genetic cause. The ICE included a list of
idiopathic epilepsies grouped according to onset age. Most
of these were of young onset before adulthood. Examples
included Childhood Absence Epilepsy and Juvenile
Myoclonic Epilepsy. Symptomatic referred to the conse-
quence of a known disorder or lesion in the central nervous
system such as tumor, infection and trauma. This proposal
also pointed out that epilepsies were mostly syndromes
rather than diseases. A disease was an entity tended to
have more uniform etiology and prognosis. A syndrome, on
the contrary, was defined as a disorder characterized by a
constellation of specific clinical or investigational features.
A syndrome did not necessarily have a definite etiology or
prognosis.
Four years after the release of the ICE, the ILAE revised
their proposal in 1989.6 The key point was the addition of a
group of cryptogenic epilepsies (Table 2). This group
embraced epilepsies which were presumed to be symp-
tomatic but the lesion could not be identified. Example was
a patient with focal epilepsy without significant finding in
neuroimaging. Since then epilepsies were classified into
three classes, namely idiopathic, cryptogenic and
Table 2 Epilepsy classification of 1989 proposal for
revised Classification of Epilepsies and Epileptic Syndromes.
Localization-related Idiopathic
(partial/focal/local) Symptomatic
epilepsies and Cryptogenic
syndromes
Generalized epilepsies Idiopathic
and syndromes Cryptogenic or
symptomatic
Symptomatic
Epilepsies and syndromes With both generalized and
undetermined to be focal seizures
generalized or focal Without unequivocal
generalized or focal features
Special Syndromes
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Classifications of seizures and epilepsies 3

Table 3 Seizure classification of 2010 revised terminology and concepts for organization of seizures and epilepsies: report of
the ILAE Commission on Classification and Terminology, 2005e2009.
Generalized Seizures Toniceclonic (in any combination)
Absence Typical
Atypical
Absence with special features Myoclonic absence
Eyelid myoclonia
Myoclonic Myoclonic
Myoclonic atonic
Myoclonic tonic
Clonic
Tonic
Atonic
Focal seizures Without impairment of consciousness or awareness
With impairment of consciousness or awareness
Evolving to a bilateral, convulsive seizure
Unknown Epileptic spasms

symptomatic. The ICE also included a categorization of
partial epilepsies according to anatomical location of
epileptic foci. Subtypes included temporal, frontal, parie-
tal and occipital. This provided clinicians a guidance to
localize the foci of partial epileptic disorders, which may
direct management formulation especially for epileptic
surgery.
Since then, classifications of seizures and epilepsies
have been directed by the 1981 and 1989 proposals for
more than two decades. Though the ICS and ICE are
considered to be imperfect, they have been popular in the
epileptology field and only minor modifications have been
made throughout the years. In 2001, a five axes diagnostic
scheme was proposed.7 The five axes were namely 1) ictal
phenomenology; 2) seizure type 3) syndrome; 4) etiology;
and 5) impairment. The aim was to provide clinicians a
flexible clinical approach to facilitate clinical management.
In 2006, the ILAE issued an update.8 The dichotomy of
partial and generalized groups in both seizures and epi-
lepsies were basically unchanged. The list of epileptic
syndromes was updated in the report.
THE 2010 concepts for organization of seizures
and epilepsies
The ILAE issued a report on revised terminology and orga-
nization of seizures and epilepsies in 2010.9 The term or-
ganization was emphasized, hoping it could promote
flexibility in categorization. This report resulted in a great
debate in epileptology field. There were two main points in
the proposal. One was the redefinition of focal and gener-
alized seizures. The term focal was officially used to
replace the word partial. The other was the reorganiza-
tion of epilepsies. Though the dichotomy of generalized and
focal seizures was retained, the 2010 scheme introduced a
new concept of network. A focal seizure was a seizure
that originated within neuronal network limited to unilat-
eral hemisphere only. A generalized seizure was defined as
a seizure originated at some points within neuronal net-
works which were bilaterally distributed within the brain
allowing rapid discharge propagation. These networks could
include both cortical and subcortical structures, but did not
necessarily involve the entire cortex. Generalized seizures
could involve both cerebral hemispheres asymmetrically.
Also, terms of simple, complex and secondarily
generalized seizure, which had been used to describe the
conscious level of a focal seizure or propagation to gener-
alized attack, were replaced by without or with
impaired consciousness or awareness and evolving to a
bilateral, convulsive seizure respectively (Table 3).
For the epilepsy, the 2010 report proposed to abandon
the subtyping of idiopathic, symptomatic and cryptogenic.
More specific terms in etiological standpoint including ge-
netic, structural, metabolic and unknown were recom-
mended (Table 4). These categories were not mutually
exclusive. An epileptic disorder could fall into more than
one class. Tuberous sclerosis, as an example, could be
considered as both structural and genetic.10 The concept of
epileptic syndromes is retained. A term of constellation
was proposed to denote the electroclinical syndromes with
specific associations such as radiological or pathological
findings.
These changes had provoked much controversy.11e15
Criticism included insufficient scientific understanding in
epileptogenesis to support a novel classification scheme.
There was no ground to replace the existing time-proven
systems. Others considered it was too complicated to be
employed in daily practice.
The 2017 ILAE operational classification of
seizure types and classification of epilepsies
In preparing their next revision after the 2010 proposal, the
ILAE Commission had made use of the internet to collect
comments globally. In 2017, the ILAE announced new clas-
sification schemes for both seizures and epilepsies.16e18
The ILAE proposes to classify epilepsies on three levels.
They are the categorizations of seizure types, epilepsy
types and epileptic syndromes. For seizures, the ILAE
stresses new classification system is based on clinical rather
than pathogenic mechanisms. The scheme divides seizures
into focal onset and generalized onset (Table 5). Focal

Please cite this article in press as: Chang RS-k, et al., Classifications of seizures and epilepsies, where are we? e A brief historical review
and update, Journal of the Formosan Medical Association (2017), http://dx.doi.org/10.1016/j.jfma.2017.06.001
 + MODEL
4 R.S.-k. Chang et al.

onset seizures can be subdivided into focal retained or

Table 4 Epilepsy classification of 2010 revised terminol-
ogy and concepts for organization of seizures and epi-
lepsies: report of the ILAE Commission on Classification and
Terminology, 2005e2009.
Electroclinical Arranged by age of onset
syndromes Examples: West syndrome,
Lennox-Gastaut Syndrome
and Juvenile Myoclonic
Epilepsy
Constellations Based on presence of
specific lesions or other
causes with implication on
clinical treatment,
particularly surgery
Examples: Mesial temporal
lobe epilepsy with
hippocampal sclerosis,
gelastic seizures with
hypothalamic hamartoma
Epilepsies Examples: Malformations of
associated with structural cortical development
or metabolic conditions (hemimegalencephaly,
heterotopias, etc.),
Neurocutaneous syndromes
(tuberous sclerosis complex,
Sturge-Weber, etc)
Epilepsies of unknown cause
impaired awareness seizures. However, this description is
optional as alterated consciousness, which is usually a
retrospective recall, is often considered as non-specific.
Both focal and generalized seizures then could be divided
into motor and non-motor onset types according to the first
prominent signs or symptoms in the seizure. Different de-
scriptors are available to further categorize different sei-
zures. They are applied to denote the earliest symptoms.
For example, if a focal seizure starts with fear emotion and
then evolves to focal clonic activity, this seizure attack is
still classified as focal emotional seizure. Focal seizure
which subsequently becomes generalized is now named as
focal to bilateral tonic-clonic seizure. The terms second-
arily generalization and secondary generalization are
abandoned for reserving generalized to seizures with
generalized onset. For generalized onset seizures, there
are also various descriptive terms to characterize both
motor and nonmotor generalized seizures. Compared with
the 1981 seizure classification, new generalized seizure
types are included in this update. They include absence
with eyelid myoclonia, myoclonic absence, myoclo-
nicetoniceclonic, myocloniceatonic, and epileptic spasms.
Besides focal and generalized, there is also a new category
of seizure type e seizure of unknown onset. This reflects
many patients have presented with seizures that cannot be
determined to be focal or generalized in initial clinical
assessment. It is a temporarily holding place for uncate-
gorized seizures. Once further information from clinical
workup is available, a seizure of unknown onset can be
relabeled as either a focal or generalized seizure. It is

Table 5 Seizure classification of 2017 Operational classification of seizure types by the International League Against Epilepsy:
position paper of the ILAE Commission for Classification and Terminology.
Focal onset Aware Motor onset Automatisms Focal to bilateral tonic-clonic
Atonic
Clonic
Epileptic spasms
Hyperkinetic
Myoclonic
Tonic
Impaired awareness Nonmotor onset Autonomic
Behavior arrest
Cognitive
Emotional
Sensory
Generalized onset Motor Tonic-clonic
Clonic
Tonic
Myoclonic
Myoclonic- tonic-clonic
Myoclonic-atonic
Epileptic spasms
Nonmotor (absence) Typical
Atypical
Myoclonic
Eyelid myoclonia
Unknown onset

Please cite this article in press as: Chang RS-k, et al., Classifications of seizures and epilepsies, where are we? e A brief historical review
and update, Journal of the Formosan Medical Association (2017), http://dx.doi.org/10.1016/j.jfma.2017.06.001
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Classifications of seizures and epilepsies
Table 6 Levels of diagnosis and epilepsy classification of 2017 ILAE classification of the epilepsies: position paper of the ILAE
Commission for Classification and Terminology.
First level Second level
Focal onset seizures Focal epilepsies
Generalized onset seizures Generalized epilepsies
Combined generalized and focal epilepsies
Unknown onset seizures Unknown epilepsies
noticeable that certain seizure semiology, such as epileptic
spasms, myoclonic and tonic, could be classified into more
than one seizure types. For example, epileptic spasms can
of focal, generalized or unknown onset. The distinction
mainly depends on clinical-electroencephalographic corre-
lation by video-EEG.
The 2017 epilepsy classification divides epilepsies into
four types, they are focal, generalized, combined general-
ized and focal, and unknown (Table 6). The categorization
of an epilepsy is based on its type of seizure. A focal epi-
lepsy is an epilepsy with focal seizure and a generalized
epilepsy is an epilepsy with generalized seizure. A combined
generalized and focal epilepsy is an epilepsy which has both
focal and generalized seizures. The unknown epilepsy type
is the epilepsy in which the seizures are of unknown onset
type or the clinician has not yet gather sufficient clinical
information to be certain about the epilepsy classification.
Similar to seizure of unknown onset, unknown epilepsy type
may be relabeled as other types of epilepsy once adequate
clinical information allows further classification.
The third level of the new classification is the diagnosis of
epilepsy syndrome. An epilepsy syndrome is a combination
of specific features such as clinical presentation e particu-
larly the onset age and natural course, seizure types, EEG,
and neuroimaging features occur together. The diagnosis of
epilepsy syndrome is important as it has treatment and
prognostic implications. Recognized epilepsy syndromes
include Lennox-Gastaut Syndrome, West Syndrome, Dravet
Syndrome, etc. The official website of ILAE has a full list of
them.19 The ILAE still retains the term of Idiopathic
Generalized Epilepsies, but limits the term to four epilepsy
syndromes only, they are the Childhood Absence Epilepsy,
Juvenile Absence Epilepsy, Juvenile Myoclonic Epilepsy and
Generalized ToniceClonic Seizures Alone.
Etiological classification, which was advocated in the
2010 report, has been incorporated into current system.
Clinicians are encouraged to classify an epileptic disorder
into one of the six etiological subgroups at every stage of
epilepsy diagnosis. The six etiological subgroups are struc-
tural, genetic, infectious, metabolic, immune and un-
known. A seizure or an epilepsy can belong to more than
one subgroup.
Discussion
Recently efforts have been invested to incorporate etiol-
ogies in the classification systems. It seems to be an
Please cite this article in press as: Chang RS-k, et al., Classifications of seizures and epilepsies, where are we? e A brief historical review
and update, Journal of the Formosan Medical Association (2017), http://dx.doi.org/10.1016/j.jfma.2017.06.001
5
Third level Etiologies
Epilepsy syndromes Structural
Genetic
Infectious
Metabolic
Immune
Unknown
inevitable trend as technology, such as genetics and imag-
ing, is rapidly advancing. Some may expect a paradigm shift
from phenomenological approach to pathomechanical
approach in classifying seizures and epilepsies. However,
many practical problems have to be solved before this
fundamental change can become feasible. Though some
seizures or epilepsies have well defined etiologies, the
causes are still elusive in many other cases. Determination
of etiology sometimes depends on the thoroughness of
investigation. This in turn depends on the availabilities of
expertise and resource. A classification system of seizures
or epilepsies has to be simple and convenient to be applied
in daily clinical practice. Otherwise, it may have difficulties
to be accepted globally as majority of the epileptic patients
are still living in resource limited developing countries.20
Besides a sound conceptual framework, a good classifica-
tion system should have implication in clinical practice. The
division between partial and generalized seizures or epi-
lepsies can guide medical and surgical treatments. The
2017 classification systems have retained the principal of
classifying seizures and epilepsies basing on semiological
and EEG features. It introduces etiological categorization in
different levels of diagnosis. Both ictal phenomenology and
pathomechanism are categorized independently. It may
retain the advantages of old systems while provide the
flexibility to describe the etiology.
This review aims to objectively summarize the process
of establishing seizure and epilepsy classifications. The
major steps and key points in recent decades are outlined.
It is impossible to exhaust every detail in each proposal. We
hope this review can help readers to have grasp the
evolving process of taxonomy in epileptology.
Conflict of interest
All authors declare no potential financial and nonfinancial
conflicts of interest.
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Please cite this article in press as: Chang RS-k, et al., Classifications of seizures and epilepsies, where are we? e A brief historical review
and update, Journal of the Formosan Medical Association (2017), http://dx.doi.org/10.1016/j.jfma.2017.06.001