REVIEW ARTICLE

C O N T I N UU M A UD I O
I NT E R V I E W A V AI L A B L E
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CITE AS:
CONTINUUM (MINNEAP MINN)
2019;25(2, EPILEPSY):306–321.
Address correspondence to
Dr Alison M. Pack, Columbia
University, Department of
Neurology, 710 W 168th St,
New York, NY 10032,
ap390@cumc.columbia.edu.
RELATIONSHIP DISCLOSURE:
Dr Pack receives research/grant
support from the National
Institutes of Health/National
Institute of Neurological
Disorders and Stroke as
co-investigator of a multicenter
study and receives royalties from
UpToDate, Inc.
UNLABELED USE OF
PRODUCTS/INVESTIGATIONAL
USE DISCLOSURE:
Dr Pack reports no disclosure.
© 2019 American Academy
of Neurology.
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Epilepsy Overview and
Revised Classification of
Seizures and Epilepsies
By Alison M. Pack, MD, MPH
ABSTRACT
PURPOSE OF REVIEW: The classification of seizures, epilepsies, and epilepsy
syndromes creates a framework for clinicians, researchers, and patients
and their families. This classification has evolved over the years, and in
2017 the International League Against Epilepsy (ILAE) published an
operational classification of seizures and epilepsies. Understanding
this classification is important in the diagnosis, treatment, and
understanding of seizures and epilepsies, including epilepsy
incidence.
RECENT FINDINGS: The 2017 ILAE classification system builds on newly
formulated definitions of seizures and epilepsy. Seizure classification
begins by determining whether the initial manifestations of the seizure
are focal or generalized. If the onset of the seizure is missed or unclear,
the seizure is of unknown onset. Focal seizures are classified according
to the individual’s level of awareness, the most prominent motor or
nonmotor features of the seizure, and whether the focal seizure evolves
to a bilateral tonic-clonic seizure. Similarly, generalized seizures are
classified according to motor or nonmotor manifestations. Motor seizures
are either tonic-clonic or other motor seizures. Nonmotor generalized
seizures primarily refer to absence seizures. Similar to seizure
classification, the epilepsies can be classified as focal or generalized.
In addition, the new classification system recognizes two new categories:
combined generalized and focal epilepsy and unknown epilepsy.
The concept of an epilepsy syndrome has been introduced under the
new classification system and refers to a cluster of features incorporating
seizure types, EEG, imaging, and other features including genetics.
The new classification system emphasizes the etiology of seizures
and epilepsies.
SUMMARY: The recent ILAE seizure and epilepsy classification system
aims to create a framework to better classify seizures and the epilepsies.
Universal adoption and implementation of this system will enable patients,
their families, clinicians, and researchers to better define and treat
the epilepsies. Incidence studies have not generally classified seizures
and the epilepsies, and use of this classification system, which
emphasizes etiology, will lead to a better understanding of epilepsy
incidence.
APRIL 2019
INTRODUCTION

S
eizure and epilepsy classification systems have been used in clinical
practice and research since the 1970s. Over the years, multiple
revisions have been implemented, the most recent of which is the 2017
International League Against Epilepsy (ILAE) operational epilepsy
classification system.1–3 This system aims to better define seizures and
epilepsies by classifying them using key clinical features, EEG findings, imaging,
and genetics. This article reviews the history of epilepsy classification and the
details of the 2017 system, with an emphasis on the importance of classification in
epilepsy incidence studies.

HISTORICAL OVERVIEW OF SEIZURE, EPILEPSY, AND EPILEPSY

SYNDROME CLASSIFICATION
Seizure and epilepsy classification has evolved over the years. Prior to the first
modern seizure classification by Gastaut in 1969,4,5 seizures and epilepsy types
were not distinctly recognized. Although initially met with resistance, this
system gained international recognition after 1970 and was widely used. In 1981,
the ILAE, informed by advances in technology—notably video recording with
simultaneous EEG—published a classification of seizures6 followed by a proposal
of epilepsy classification in 1985,7 which was then revised in 1989,8 wherein the
concept of an epilepsy syndrome was introduced.
The ILAE then began an effort to revise seizure terminology and the
organization of seizures and the epilepsies.9 This effort culminated in the 2017
published ILAE operational classification of seizure types1,2 and epilepsies.3
The 1981 ILAE seizure classification system dichotomized seizures into either
partial or generalized seizures. Partial seizures were 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.”6 Partial
seizures were subdivided by level of consciousness: simple partial seizures were
associated with no impairment of awareness, and complex partial seizures
were associated with impairment of awareness. A third partial seizure type
included seizures that evolved to a secondary generalized convulsion. A
generalized seizure was defined as a seizure “in which the first clinical changes
indicate initial involvement of both hemispheres.”6 Six generalized seizure types
were identified: absence, myoclonic, clonic, tonic, tonic-clonic, and atonic.
The 1985 epilepsy classification was also a dichotomized system dividing
epilepsies into either idiopathic or symptomatic. The term idiopathic derives
from the Greek idios, meaning self, own, and personal. Idiopathic epilepsies and
syndromes were described as disorders “not preceded or occasioned by another.”
In these disorders, no underlying cause is present other than a possible hereditary
predisposition.7 Notable idiopathic epilepsies included juvenile myoclonic
epilepsy and childhood absence epilepsy. Symptomatic epilepsies occurred
because of a known disorder or lesion. A revision of the epilepsy classification in
1989 added cryptogenic epilepsies, which were likely symptomatic, but a cause
was not identified.8
Since the seizure and epilepsy classification systems were developed in the
1980s, many advances in neuroimaging, genetics, and molecular biology have
occurred. Although the newly published seizure classification primarily
incorporates the signs and symptoms of seizures and EEG findings, the
classification of epilepsy type and syndrome encompasses these new advances.1–3

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CLASSIFICATION OF SEIZURES AND EPILEPSIES

The current classification systems build on newly formulated definitions of

seizures and epilepsy. The 2005 updated seizure definition is a “transient
occurrence of signs and/or symptoms due to abnormal excessive or synchronous
neuronal activity in the brain.”10 Further, in 2014 the ILAE redefined epilepsy as
a disease and not a disorder to emphasize the importance and impact of epilepsy.
Epilepsy occurs when an individual has an epileptic seizure and his or her
“brain…demonstrates a pathologic and enduring tendency to have recurrent
seizures.”11
Previously, epilepsy was diagnosed when an individual had at least two
unprovoked or reflex seizures more than 24 hours apart. Reflex seizures are
stimulus-sensitive or sensory-evoked seizures. Although the new definition of
epilepsy includes this presentation, epilepsy is also diagnosed if a person has one
unprovoked or reflex seizure and has a probability of at least 60% of having
another seizure within the next 10 years.11 The probability of at least 60%
was chosen for this definition because this is the lower limit of the confidence
interval for someone with two unprovoked seizures having another seizure
within 10 years.12
In addition, epilepsy is diagnosed when an individual has an epilepsy
syndrome. An analysis of retrospective cases supports the applicability of
this new definition.13 In patients with a single unprovoked seizure, a second
seizure occurred in more than 80%. The definition, however, does not clarify
what metrics should be used to identify patients at risk for a recurrent seizure.
Just as multiple scenarios lead to an initial diagnosis of epilepsy, multiple
circumstances can contribute to epilepsy being considered resolved. Epilepsy is
considered resolved when a patient with an age-dependent epilepsy syndrome
is older than the age in which this syndrome was active or when a patient has
been seizure free for 10 or more years and has been off all antiepileptic drugs for
5 or more years.14
CLASSIFYING SEIZURES, EPILEPSIES, AND EPILEPSY SYNDROMES
The report by the Institute of Medicine, “Epilepsy Across the Spectrum,”
emphasizes the heterogeneity of seizures and the epilepsies.15 Making sense of
this heterogeneity requires a system to provide a common language. To classify
means to arrange something into classes or categories according to shared
qualities or characteristics. The prior and current classification systems aim to
group seizures according to clinical presentation and brain region onset and to
group epilepsies according to seizure type, age of onset, probability of remission,
EEG findings, radiologic findings, and genetics. Classification of seizures,
epilepsies, and epilepsy syndromes creates a framework for patients, their
families, clinicians, and researchers.
For patients, the universal common language of the classification system
provides a name and diagnosis, which improves understanding and recognition
of the disease. Moreover, this common language enhances communication
between patients, their families, and providers.
Similarly, clinicians can use the language to better communicate with patients
and colleagues. The classification systems allow clinicians to consider history
and include data from new technologies when making a diagnosis, choosing
treatment, and assessing prognosis.
For researchers, these systems enable standardized investigation of seizure
and epilepsy presentation, etiologies, and drug or surgical treatments. For
example, when studying incidence patterns of epilepsy, using a common

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language and classification enables comparison across multiple populations KEY POINTS
and studies.
● In 2014 the International
UPDATED SEIZURE CLASSIFICATION League Against Epilepsy
redefined epilepsy as a
With the exception of neonatal seizures, the new seizure classification applies to disease and not a disorder to
adults and children. The new classification addresses the limitations of the 1981 emphasize the importance
seizure classification, which include the following: (1) some seizure types can and impact of epilepsy.
have either focal or generalized onset, (2) lack of knowledge about seizure onset Epilepsy occurs when an
individual has an epileptic
makes a seizure unclassifiable and difficult to place within the 1981 system,
seizure and his or her “brain
(3) retrospective seizure descriptions often do not include the level of demonstrates a pathologic
consciousness, (4) terms used in the 1981 seizure classification such as complex and enduring tendency to
partial or simple partial are difficult to understand, and (5) some seizure types have recurrent seizures.”
are not included in the 1981 classification.1
● Prior and current
To address needs of different clinicians and researchers, both basic classification systems aim to
(FIGURE 1-1) and expanded (FIGURE 1-2) versions of seizure classification were group seizures according to
created. The basic version of the seizure classification is a contracted form of the clinical presentation and
expanded classification and is intended to be more useful for pediatricians, brain region onset and
epilepsies according to
non-neurologists, general neurologists, physicians in general practice, nurses, seizure type, age of onset,
and health care workers. The expanded version is more detailed and will likely be probability of remission,
used more by epileptologists/neurophysiologists and researchers. EEG findings, radiologic
Seizure classification starts with whether the initial manifestations of the findings, and genetics.
seizure are focal or generalized. Focal seizures originate within a neuronal
● The 2017 International
network limited to one hemisphere that may be discretely localized or more League Against Epilepsy
widely distributed, whereas generalized seizures originate at some point within seizure classification
the brain and rapidly engage bilateral distributed networks.9 If the onset of the addresses limitations of the
1981 seizure classification,
seizure is missed or is unclear, the seizure is of unknown onset.
which include the following:
Beginning in 2010 the term focal seizure officially replaced partial seizure.9 (1) some seizure types can
The term focal seizure had been used prior to the 1981 classification system, at have either focal or
which time partial seizure had replaced focal because of concern that its use was generalized onset, (2) lack of
confusing and inferred onset in a very restricted area of brain (some seizures may knowledge about seizure
onset makes a seizure
involve large parts of a hemisphere). It is, however, now felt that the term focal unclassifiable and difficult
seizure is more widely understood. to place within the 1981
system, (3) retrospective
Focal Seizures seizure descriptions often
Focal seizures are classified according to the patient’s level of awareness and the do not include a level of
consciousness, (4) terms
first most prominent motor or nonmotor features of the seizure. These early used in the 1981 seizure
classification such as
complex partial or simple
partial are difficult to
understand, and (5) some
seizure types are not
included in the 1981
classification.

FIGURE 1-1
Basic version of 2017 International League Against Epilepsy seizure type classification.
Reprinted with permission from Fisher RS, et al, Epilepsia.1 © 2017 John Wiley and Sons.

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CLASSIFICATION OF SEIZURES AND EPILEPSIES

FIGURE 1-2
Expanded version of 2017 International League Against Epilepsy seizure type classification.
Reprinted with permission from Fisher RS, et al, Epilepsia.1 © 2017 John Wiley and Sons.

prominent features are important to consider when localizing the seizure onset or
epileptogenic zone. The final feature used in classification of focal seizures is
whether the focal seizure evolves to a bilateral tonic-clonic seizure. The term
secondary generalized tonic-clonic seizure is no longer used because the term
focal seizure more completely differentiates this type from generalized seizures.
Awareness is defined as knowledge and understanding that something is
happening or exists. When a person is having a focal seizure, his or her awareness
is determined by whether the person knows who they are and what is going on
in his or her surroundings during the seizure; it does not refer to awareness of the
seizure occurring. Awareness is also distinct from responsiveness. If awareness
is impaired for any portion of the seizure, then the seizure is classified as a focal
seizure with impaired awareness.
Awareness may be considered a surrogate for consciousness. Impaired
awareness or consciousness during a seizure is likely secondary to depressed
subcortical arousal systems, leading to deep sleep activity in widespread
neocortical regions, hence the involvement of both subcortical and cortical
structures.16 A focal aware seizure replaces the previously termed simple partial
seizure, and a focal impaired awareness seizure replaces the term complex partial
seizure.1 If unknown, the level of awareness does not need to be included.
Focal motor seizures can be more specifically defined. Motor-onset
manifestations include automatisms, epileptic spasms, and atonic, clonic,
hyperkinetic, myoclonic, or tonic seizures. Automatisms are coordinated,
purposeless, repetitive motor activities that may appear normal in other
circumstances. Examples include oral automatisms such as lip smacking and
manual automatisms including repetitive hand movements such as patting (CASE 1-1).

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Of note, automatisms can also be seen in absence seizures, which are discussed KEY POINTS
in the next section.
● Focal seizures originate
Focal atonic seizures are characterized by loss of tone in one body part. within a neuronal network
Clonic seizures are repeated, regularly spaced stereotypical jerking movements. limited to one hemisphere
Epileptic spasms were previously only considered generalized seizures. that may be discretely
Clinically, epileptic spasms present in young children with flexion of the waist localized or more widely
distributed, whereas
and flexion or extension of the arms, usually in clusters. If epileptic spasms
generalized seizures
occur in infants or early in life, they can be referred to as infantile spasms. originate at some point
Differentiating focal epileptic spasms from generalized epileptic spasms may within the brain and rapidly
require careful observation of clinical and electrographic features. Hyperkinetic engage bilateral distributed
networks.
or excessive muscular movement seizures can have variable features clinically,
including thrashing or pedaling. Focal myoclonic seizures present with jerking ● Focal seizures are
but, in contrast to clonic seizures, the jerking is irregular and not rhythmic. Tonic classified according to the
seizures refer to motor seizures with increased tone or stiffening of the limb patient’s level of awareness
or neck. and the first most prominent
motor or nonmotor features
Focal seizures with nonmotor symptoms as the first prominent feature of the seizure. These early
include autonomic, behavior arrest, cognitive, emotional, or sensory seizures. prominent features are
Autonomic seizures present with changes in heart rate, blood pressure, sweating, important to consider when
skin color, piloerection, or gastrointestinal sensations. Behavioral arrest seizures localizing the seizure onset
or epileptogenic zone. The
are characterized by cessation of movement, which should be the dominant final feature used in
feature throughout the entire seizure and not just a brief part of the seizure; classification is whether the
clinical symptoms include a blank stare and cessation from talking or moving. focal seizure evolves to a
Patients with nonmotor cognitive seizures can experience changes in language bilateral tonic-clonic
seizure.
function, thinking, or associated higher cortical functions; more specific
examples include déjà vu, jamais vu (a feeling of unfamiliarity), or hallucinations. ● Generalized seizures are
Emotional seizures appear with clear emotional changes such as dread, fear, classified according to
anxiety, or pleasure. Focal sensory seizures are classified according to changes in motor or nonmotor
sensory phenomena such as taste, smell, hearing, vision, pain, numbness, manifestations. Broadly,
motor seizures are either
or tingling. tonic-clonic or other motor
Focal seizures can be further classified as to whether they evolve to a bilateral seizures. Nonmotor
tonic-clonic seizure. As discussed previously, this classification replaces generalized seizures
secondary generalized tonic-clonic to avoid any confusion between generalized primarily refer to absence
seizures.
and focal seizures. These seizures start in one area of the brain (as with all focal
seizures) and then spread to both sides of the brain. This spread is typically
clearly seen on EEG.

Generalized Seizures
Similar to focal seizures, generalized seizures are classified according to motor
or nonmotor manifestations. Broadly, motor seizures are either tonic-clonic
or other motor seizures. Nonmotor generalized seizures primarily refer to
absence seizures.
Motor onset more specifically includes tonic-clonic, clonic, tonic, myoclonic,
myoclonic-tonic-clonic, myoclonic-atonic, atonic, or epileptic spasms. Generalized
tonic-clonic seizures generally last 1 to 3 minutes and result in immediate loss of
awareness or consciousness. The initial tonic phase is a stiffening of all limbs. The
patient may groan or cry in the beginning as air is forced past the vocal cords.
The tongue may also be bitten during this phase.
The clonic phase occurs after the tonic phase and is characterized by sustained
rhythmic jerking of the limbs. If the person has impaired breathing, he or she
may look dusky. Incontinence of either bladder or bowel occurs when the body

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CLASSIFICATION OF SEIZURES AND EPILEPSIES

relaxes. A generalized clonic seizure is characterized by bilateral and sustained

rhythmic jerking. A patient with generalized tonic seizures will have stiffening of
all limbs.
In contrast to generalized clonic seizures, generalized myoclonic seizures are
associated with irregular and not necessarily synchronous bilateral jerking of
limbs, face, eyes, or eyelids. Myoclonic-tonic-clonic seizures are a new seizure

CASE 1-1 A 27-year-old man presented after experiencing an episode of loss of

awareness. His friend reported that he had been eating dinner when he
acutely stared off, which was followed by lip smacking, chewing
movements, and clenching of his
left hand, lasting a total of
90 seconds. He then appeared
confused and was back to baseline
approximately 10 minutes after
the episode began.
The patient’s past medical history
was notable for a prolonged febrile
seizure at age 18 months but was
otherwise unremarkable. He took no
medications and had no family
history of seizures. He drank two to
three glasses of wine a week. He
denied tobacco or illicit drug use.
Physical and neurologic examination
were unremarkable. Brain MRI
revealed atrophy of the right mesial FIGURE 1-3
temporal region and increased T2 Imaging of the patient in CASE 1-1.
signal in the right hippocampus Coronal MRI reveals atrophy of the
right mesial temporal region and
(FIGURE 1-3). EEG revealed right increased signal in the right
temporal slowing and epileptiform hippocampus consistent with mesial
sharp waves (FIGURE 1-4). temporal sclerosis.

COMMENT This patient’s clinical presentation was consistent with a focal seizure. His
awareness was impaired, and early prominent features included nonmotor
oral automatisms followed by motor left hand dystonia. This seizure
would, therefore, be classified as a focal impaired awareness seizure with
automatisms. The seizure combined with the MRI and EEG findings
suggests a focal epilepsy with at least a 60% risk of a having a second
unprovoked seizure within 10 years. Specifically, this patient has right
temporal lobe epilepsy, with right mesial temporal sclerosis as the etiology.
Treatment with an antiepileptic medication should be recommended, and
if he continues to have seizures despite treatment with two antiepileptic
medications, he would be considered refractory, and referral to an
epilepsy center for epilepsy surgery should be recommended.

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designation and begin with irregular jerking on both sides followed by a
tonic-clonic seizure. Myoclonic-tonic-clonic seizures are common in juvenile
myoclonic epilepsy (CASE 1-2). Myoclonic-atonic seizures are also a new seizure
designation and are characterized by an initial irregular jerking followed by
loss of tone on both sides. These seizures are common in epilepsy with
myoclonic-atonic seizures (Doose syndrome).

FIGURE 1-4
EEG of the patient in CASE 1-1. Longitudinal bipolar montage EEG recording shows right
temporal slowing and anterior temporal spike-and-slow-wave epileptiform discharge.

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CLASSIFICATION OF SEIZURES AND EPILEPSIES

Atonic seizures are brief and occur when there is bilateral loss of tone and the
muscles become limp. If the person is standing when the seizure occurs, he or she
will fall, often resulting in injury. Epileptic spasms are also brief and typically
occur in clusters with flexion at the trunk and flexion or extension of the limbs.
As with focal epileptic spasms, an EEG may be needed to distinguish whether
the seizure is generalized.

CASE 1-2 A 13-year-old girl presented for evaluation after experiencing a single
generalized tonic-clonic seizure that occurred in the morning upon
awakening. Her history revealed that for the past year, she would often
drop her toothbrush in the morning.
Her past medical history and family history were unremarkable.
Menarche had occurred at age 12. She took no medications and had no
history of alcohol, tobacco, or illicit drug use. Her physical and neurologic
examinations were unremarkable. Brain MRI was normal, and EEG
revealed generalized spike-wave discharges (FIGURE 1-5). The patient’s
parents questioned whether she had epilepsy and whether she should
be treated.

FIGURE 1-5
EEG of the patient in CASE 1-2. Longitudinal bipolar montage shows frontally predominant
3-Hz to 4-Hz generalized spike-wave discharges.

COMMENT Epilepsy is diagnosed if a person has one unprovoked seizure and at least a
60% risk of having a second unprovoked seizure within 10 years. This girl
presented with a single generalized seizure with no focal features and had
a history suggestive of morning myoclonus. The clinical presentation and
EEG were consistent with a generalized epilepsy syndrome, likely juvenile
myoclonic epilepsy. She was diagnosed with epilepsy, and antiepileptic
drug treatment was recommended.

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 Nonmotor or absence seizures include typical, atypical, myoclonic, or eyelid KEY POINTS
myoclonia. Typical absence seizures present with a sudden cessation of activity
● Seizures of unknown
sometimes with eye fluttering, head nodding, or other automatisms followed onset can be classified by
by an immediate recovery. EEG always reveals generalized spike-wave activity motor (tonic-clonic,
during the seizure. Atypical absence seizures are similar to absence seizures epileptic spasms) or
but have other features including slower onset, prolonged recovery, and more nonmotor (behavior arrest)
presentation. If information
pronounced changes in tone. A myoclonic absence seizure begins with a few
is inadequate or if the
irregular jerks followed by an absence seizure. Eyelid myoclonia is defined by seizure cannot be
jerks of the eyelids and upward deviation of the eyes. Light and closing the categorized, then the
eyes can precipitate these generalized seizures. Eyelid myoclonia with absence seizure is considered
unclassified.
seizures is seen in Jeavons syndrome.
● Similar to seizure
Unknown Seizures classification, the epilepsies
Seizures of unknown onset can be classified by motor (tonic-clonic, epileptic are classified as generalized
spasms) or nonmotor (behavior arrest) presentations. If information is or focal. The new
classification system
inadequate or if the seizure cannot be categorized, then the seizure is considered additionally recognizes two
unclassified. new categories: combined
generalized and focal
UPDATED EPILEPSY CLASSIFICATION epilepsy and unknown
epilepsy.
The second level of classification is the epilepsy type. This classification
assumes the patient has epilepsy as defined by the previously discussed updated ● Patients with generalized
definition.11 The epilepsy type is predominantly determined clinically; epilepsy have one or more
characteristic EEG findings provide supportive evidence. Similar to seizure of the generalized seizure
types, and their EEGs
classification, the epilepsies are classified as generalized or focal. The new
typically display generalized
classification system additionally recognizes two new categories: combined spike-wave activity. For
generalized and focal epilepsy and unknown epilepsy.3 individuals who have
Patients with generalized epilepsy have one or more of the generalized seizure generalized seizure types
types, and their EEGs typically display generalized spike-wave activity. For and a normal EEG, other data
are needed to determine
individuals who have generalized seizure types and a normal EEG, other data are whether the epilepsy is
needed to determine whether the epilepsy is generalized. Having myoclonic generalized.
jerks or a pertinent family history supports the diagnosis of a generalized
epilepsy type. ● Patients with one or more
focal seizure types have
Clinically, patients with one or more focal seizure types have focal epilepsy. focal epilepsy. These
These epilepsies can be either unifocal or multifocal. Although not always seen, epilepsies can be either
focal EEG findings such as focal slowing or epileptiform discharges support the unifocal or multifocal.
diagnosis of focal epilepsy. Concordant focal MRI findings are also supportive.
● Designation of combined
Designation of combined generalized and focal epilepsy is for patients with
generalized and focal
both focal and generalized seizures. EEG may reveal both focal and generalized epilepsy is for patients with
electrographic findings. Examples of combined generalized and focal epilepsy both focal and generalized
include Dravet syndrome and Lennox-Gastaut syndrome. seizures. EEG may reveal
When the patient has epilepsy as defined by the ILAE but it remains both focal and generalized
electrographic findings.
undetermined whether the patient has focal or generalized epilepsy, the Examples of combined
classification of unknown epilepsy type is used. Patients with this classification generalized and focal
may not have an available EEG or the EEG may be indeterminate. Other epilepsy include Dravet
supporting studies such as MRI and family history are also either not available syndrome and
Lennox-Gastaut syndrome.
or do not clarify the epilepsy classification.

NEW CLASSIFICATION OF EPILEPSY SYNDROME

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

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CLASSIFICATION OF SEIZURES AND EPILEPSIES

features that tend to occur together.”3 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. The ILAE has never formally classified a list of epilepsy syndromes;
however, well-known and accepted syndromes are described, and some are
reviewed here. For a full list of ILAE-accepted epilepsy syndromes, refer to the
ILAE website.17
Previously, the term benign was used to describe some of the epilepsy
syndromes, but it is no longer used as it infers the epilepsy has minimal effect
on the patient. It is now more clearly understood that any epilepsy can have
social effects and can be associated with other comorbidities such as learning
disorders or psychiatric conditions. The term self-limiting is now used.

Idiopathic or Genetic Generalized Epilepsy Syndromes

Idiopathic generalized epilepsies include childhood absence epilepsy, juvenile
absence epilepsy, juvenile myoclonic epilepsy, and generalized tonic-clonic
seizures alone (TABLE 1-1). Controversy surrounds the use of the term idiopathic,
and removing it from epilepsy classification has been advocated. Idiopathic
was meant to refer to self or genetic. There is, however, concern that use of the
word genetic infers inherited, and many patients with epilepsy have de novo
mutations or have complex genetic syndromes that occur with or without
environmental factors. Many in the epilepsy community want to continue using
the term idiopathic generalized epilepsy, and the ILAE task force decided to use
it to refer to the previously mentioned epilepsies. When the clinician determines
that a clear genetic etiology is present, the term genetic generalized epilepsy may
be used to refer to the epilepsy syndrome. EEGs of the idiopathic generalized

TABLE 1-1 Idiopathic or Genetic Epilepsy Syndromes

Self-
limiting
Epilepsy Syndrome Seizure Types Age of Onset (Yes or No) EEG Findings

Childhood absence Absence, 4 to 10 years Yes Normal background, occipital intermittent

epilepsy generalized tonic- rhythmic delta activity, 3–3.5 Hz
clonic (rare) generalized spike-wave discharges

Juvenile absence Absence, Adolescence to No Normal background, polyspikes may be

epilepsy generalized tonic- early adulthood present, 3–3.5 Hz generalized spike-wave
clonic, myoclonic discharges
(rare)

Juvenile myoclonic Myoclonic, 10 years to mid- No Normal background, 3–3.5 Hz generalized

epilepsy generalized tonic- twenties spike-wave discharges, >4 Hz generalized
clonic, absence (rare) spike-wave discharges, high-amplitude
polyspike-wave discharges with
myoclonic seizures, photoparoxysmal
response in up to 40% of patients

Epilepsy with Generalized tonic- Childhood to No Normal background, generalized spike/

generalized tonic- clonic mid-adulthood polyspike-wave discharges
clonic seizures alone

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epilepsies reveal a normal electrographic background and characteristic KEY POINTS
generalized spike-wave findings (TABLE 1-1).18
● When the patient has
Childhood absence epilepsy affects neurologically normal girls more than epilepsy as defined by the
boys and is typically self-limiting. Onset is usually between 4 and 10 years of age, International League Against
with remission usually occurring in adolescence. Patients present with absence Epilepsy, but it remains
seizures and occasionally with generalized tonic-clonic seizures. Early undetermined whether the
patient has focal or
occurrence of generalized tonic-clonic seizures is associated with a poorer
generalized epilepsy, the
prognosis. classification of unknown
Juvenile absence epilepsy has onset in adolescence and early adulthood, with epilepsy type is used.
peak onset between 10 and 13 years of age. Girls and boys are affected equally.
Absence seizures occur less frequently than in childhood absence epilepsy. ● The epilepsy syndrome is
a new addition to the current
Generalized tonic-clonic seizures occur early in the presentation, and myoclonic classification system and is
seizures, although rare, may also occur. In contrast to childhood absence defined as “a cluster of
epilepsy, this syndrome is not self-limiting. features incorporating
Juvenile myoclonic epilepsy is one of the most typical epilepsy syndromes. seizure types, EEG, and
imaging features that tend to
Onset ranges from before age 10 through the mid-twenties and later in some occur together.”
cases. Juvenile myoclonic epilepsy occurs more commonly in women. All
patients have myoclonic seizures and commonly have generalized tonic-clonic ● Idiopathic generalized
seizures. Absence seizures rarely occur. Most patients do not have spontaneous epilepsies include
childhood absence
remission and require lifelong treatment with antiepileptic medication.
epilepsy, juvenile
Epilepsy with generalized tonic-clonic seizures alone is characterized by absence epilepsy, juvenile
presentation of generalized tonic-clonic seizures with an age range of childhood myoclonic epilepsy, and
through mid-adulthood with peak onset in the second decade of life. Previously it generalized tonic-clonic
seizures alone.
was referred to as generalized tonic-clonic seizures upon awakening but was
changed after recognition that seizures can occur at any time of day. Similar to ● Reflex epilepsy
juvenile absence epilepsy and juvenile myoclonic epilepsy, epilepsy with syndromes are epilepsies in
generalized tonic-clonic seizures alone is not self-limiting, and lifelong which seizures are provoked
antiepileptic drug treatment is typically required. by a specific stimulus.

● Well-described focal
Reflex Epilepsy Syndromes epilepsy syndromes include
Reflex epilepsy syndromes are epilepsies in which seizures are provoked by a childhood epilepsy with
specific stimulus. Seizures are typically generalized tonic-clonic seizures, but centrotemporal spikes and
Panayiotopoulos syndrome.
other generalized seizure types may also occur. Rarely, focal seizures may
present as a reflex epilepsy. The most common reflex epilepsy syndrome is
photosensitive epilepsy. Other reflex epilepsy syndromes include reading
epilepsy and startle epilepsy.17,18

Focal Epilepsy Syndromes

Well-described focal epilepsy syndromes include childhood epilepsy with
centrotemporal spikes and Panayiotopoulos syndrome. Previously, childhood
epilepsy with centrotemporal spikes was referred to as benign epilepsy with
centrotemporal spikes. Childhood epilepsy with centrotemporal spikes is a
self-limited epilepsy that presents in the school years with brief focal motor
hemifacial seizures and nocturnal focal motor seizures evolving to bilateral
tonic-clonic seizures. EEG background is normal with sleep-activated
centrotemporal spikes. Panayiotopoulos syndrome is also a self-limited epilepsy
characterized by having focal autonomic seizures, often prolonged, and focal
occipital high-amplitude sleep-activated spikes seen on EEG. Possible autonomic
symptoms include vomiting, pallor, mydriasis, cardiorespiratory, gastrointestinal,
and thermoregulatory symptoms, incontinence, and hypersalivation.19

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CLASSIFICATION OF SEIZURES AND EPILEPSIES

EPILEPSY ETIOLOGY
The etiology of seizures and epilepsies is emphasized in the new classification
system. In the prior classification system of the 1980s, etiology was inferred
when classifying the epilepsy. Idiopathic primarily referred to genetic causes,
symptomatic referred to the presence of a known disorder or lesion, and
cryptogenic referred to a presumed but unknown symptomatic cause. As discussed,
the term idiopathic is now used to refer to four well-described epilepsy syndromes.
The terms symptomatic and cryptogenic are no longer used.
Six etiologic categories (structural, genetic, infectious, metabolic, immune,
unknown) have been defined. When multiple potential etiologies are present,
priority should be given to the etiology with more relevant management issues.
Ongoing consideration of the etiology has clear implications for patient
management as well as research efforts as we continue to study why patients
develop seizures and we determine optimal treatments.
A structural etiology is determined when a structural abnormality is seen on
neuroimaging and when the signs and symptoms of seizures, in combination
with EEG data, suggest this abnormality is the probable cause of the seizures. If
the clinical and EEG data are discordant with localization of the visible structural
abnormality, then the imaging abnormality is not relevant to the patient’s
epilepsy. Structural abnormalities may be genetic, acquired, or both. Possible
structural abnormalities include stroke, trauma, tumor, malformations of
cortical development, and infection.
Genetic etiologies are determined if there is a known or presumed genetic
mutation in which seizures are a core symptom of the disorder.3 Genetic
epilepsies are diverse, and the list grows each year. Importantly, genetic does not
always mean inherited. Although some epilepsies are inherited, many occur
secondary to a de novo (new) mutation in the affected individual. In some cases,
the genetic mutation is not identified, but the clinical presentation, EEG findings,
and family history suggest a genetic etiology. In addition, the genetic etiology
for some epilepsy syndromes such as juvenile myoclonic epilepsy is inferred from
research studies including twin and familial aggregation studies. Overall, genetic
etiology is defined by having a known mutation, clinical presentation with
supportive data and family history, or a syndrome with evidence from research
studies to suggest a genetic etiology.
Infectious etiologies are the most common worldwide etiology. An important
distinguishing point is that the patient has epilepsy secondary to an infectious
etiology and not seizures in the setting of an acute infectious illness. Prototype
infectious etiologies include neurocysticercosis, HIV, cytomegalovirus, and
cerebral toxoplasmosis. Epilepsy onset secondary to a prior infectious insult such
as meningitis or encephalitis is also considered an infectious etiology.
Epilepsies with a metabolic etiology occur secondary to a known or presumed
metabolic disorder in which seizures are a core symptom of the disorder.3
Overlap with a genetic etiology may occur as many metabolic disorders have
known genetic mutations. Of course, identifying a genetic etiology early in
presentation is important because management interventions such as a change in
diet or supplementation can affect its natural course.
Immune etiologies are increasingly recognized as potential causes of
epilepsy. As with the other etiologies, seizures are a core symptom of the
immune disorder. In patients with identified immune etiologies, immunotherapy
should be considered. Examples of immune etiologies for seizures include

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anti–N-methyl-D-aspartate (anti-NMDA) receptor encephalitis and anti–leucine- KEY POINTS
rich, glioma inactivated 1 (anti-LGI1) encephalitis.
● The etiology of seizures
The last etiologic category is unknown; up to one-third of patients with and epilepsies is
epilepsy have no clear etiology.20 A cause likely exists, but its identification may emphasized in the 2017
be limited by inadequate resources such as poor access to up-to-date brain International League Against
imaging, immune antibody testing, or genetic testing. It is hoped that with Epilepsy classification
system.
improved access to care as well as continued research into understanding the
epilepsies the percentage of patients who have an unknown etiology will ● A structural etiology is
diminish and that the structure of the new classification system will provide a determined when a
framework to better understand the epilepsies. structural abnormality is
seen on neuroimaging and
when the signs and
INCIDENCE OF EPILEPSIES AND ITS RELATION TO SEIZURE AND symptoms of seizures, in
EPILEPSY CLASSIFICATION AND RISK FACTORS combination with EEG data,
The incidence of epilepsy is defined as the number of new epilepsy cases over a suggest this abnormality
specified period of time. Incidence represents the number of new cases among is the probable cause of
the seizures.
susceptible persons in a given location and over a particular time span. Incidence
studies, in contrast to prevalence studies, provide a better understanding of ● Genetic etiologies are
etiology and the natural history of epilepsy.21 Epilepsy incidence studies are, determined if there is a
however, lacking and heterogeneous. Heterogeneity among reported incidence known or presumed genetic
mutation in which seizures
population studies may be addressed by use of universally adopted seizure and
are a core symptom of
epilepsy classification systems. A well-constructed classification is needed for the disorder.
comparison among studies.22 The newly proposed 2017 ILAE classification
system of seizures and the epilepsies provide a system that promotes standardization ● Infectious etiologies
of terminology, which may lead to reduced variability among incidence studies are the most common
worldwide etiology
and may, therefore, improve our understanding of seizure and epilepsy of epilepsy.
incidence. It has, however, also been argued that the system allows flexibility
as cases may be classified in different categories depending on workup, creating ● Epilepsies with a
a potential obstacle for epidemiologic studies.23 metabolic etiology occur
secondary to a known or
The proposed 2017 epilepsy classification creates a framework to more presumed metabolic
specifically define the epilepsy, epilepsy syndrome, and etiology. The emphasis disorder in which seizures
on etiology may lead to a better understanding and determination of epilepsy are a core symptom of
incidence, which, of course, depends on classification agreement among the disorder.
physicians. Universal implementation of this classification system and further
● Immune etiologies are
study will hopefully clarify the utility of the newly proposed system and whether increasingly recognized
its use will reduce heterogeneity among incidence studies and allow us to better as potential causes of
define significant factors that contribute to epilepsy incidence in all regions of epilepsy.
the world.

CONCLUSION
Seizure and epilepsy classifications have evolved since a system was first
introduced by Gastaut in the late 1960s. The 2017 ILAE classification of seizures,
epilepsies, and epilepsy syndromes aims to group seizures according to clinical
presentation and brain region onset 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. Universal adoption and use of
this classification system have direct implications on our understanding of
epilepsy incidence. Multiple worldwide studies have found variable results with
marked heterogeneity. In addition, incidence studies are limited in number and
scope and rarely consider seizure type. Future studies using this standardized

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CLASSIFICATION OF SEIZURES AND EPILEPSIES

classification system will potentially clarify epilepsy incidence and hopefully

reduce heterogeneity. In addition, further study is needed to evaluate the utility
of the 2017 classification system. Although it establishes standard terminology,
potential variability in coding and poor agreement among physicians may limit
its use.

USEFUL WEBSITE
INTERNATIONAL LEAGUE AGAINST EPILEPSY
The International League Against Epilepsy (ILAE)
is an international organization whose goals
include advancement and dissemination of
knowledge about epilepsy. The organization
promotes research, education, and training and
improves services and care for patients with
epilepsy. Links to published articles on seizure
and epilepsy classification are available on
this website.
epilepsydiagnosis.org

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