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ILAE classification of seizures and epilepsy

AUTHOR: Elaine Wirrell, MD
SECTION EDITORS: Steven C Schachter, MD, Douglas R Nordli, Jr, MD
DEPUTY EDITOR: John F Dashe, MD, PhD

All topics are updated as new evidence becomes available and our peer review process is complete.

Literature review current through: Oct 2023.

This topic last updated: Jul 11, 2023.

INTRODUCTION

Epilepsy is defined as a disorder of the brain characterized by an enduring predisposition to

epileptic seizures [1]. It is a heterogeneous condition characterized by multiple possible seizure
types and syndromes, diverse etiologies, and variable prognoses. Accurate classification is
essential for several reasons [2]:

● To provide a framework for understanding the type(s) of seizure(s) a patient has, other
types that are more likely to occur, potential seizure triggers, and prognosis
● To inform the risk of comorbidities (eg, psychiatric, cognitive) and mortality, including
sudden unexpected death in epilepsy
● To guide choice of optimal antiseizure medication and surgical therapies

Over the past several decades, significant advances in neuroimaging, genomic technologies,
and molecular biology have improved the understanding of the pathogenesis of seizures and
epilepsy [3]. The International League Against Epilepsy (ILAE) Commission on Classification and
Terminology proposed substantial changes to the 1989 classification system in 2010 [4] and
made further revisions in the position paper on ILAE Classification of the Epilepsies in 2017
[2,5,6]. The framework allows for diagnosis at three levels (seizure types, epilepsy types, and
epilepsy syndromes), depending on information and resources that are available, and also
addresses the broad concepts of etiology and associated comorbidities at all three levels
( figure 1). In resource-poor settings with limited access to electroencephalography (EEG),
neuroimaging, or specialized genetic and metabolic studies, or in situations in which limited
history is present, the maximal level of diagnosis may be limited to seizure type only (level 1).
Conversely, higher levels of diagnosis will likely be possible with more detailed history and
greater availability of testing.

The most important concepts developed in the ILAE Classification of the Epilepsies are
described here. The evaluation and diagnosis of seizures and epilepsy in children and adults are
discussed separately. (See "Seizures and epilepsy in children: Clinical and laboratory diagnosis"
and "Seizures and epilepsy in children: Classification, etiology, and clinical features" and
"Epilepsy syndromes in children" and "Evaluation and management of the first seizure in adults"
and "Seizures and epilepsy in older adults: Etiology, clinical presentation, and diagnosis".)

LEVEL 1: SEIZURE TYPE

Diagnosis at level 1 requires that the clinician identify that the patient has had an epileptic
seizure rather than some other type of paroxysmal event, and then establish the type of
seizure(s) ( table 1) [2,5,6]. (See "Evaluation and management of the first seizure in adults",
section on 'Differential diagnosis' and "Nonepileptic paroxysmal disorders in infancy" and
"Nonepileptic paroxysmal disorders in children" and "Nonepileptic paroxysmal disorders in
adolescents and adults".)

Seizure type is classified based on the initial manifestation of the seizure as generalized, focal,
or unknown (if seizure onset is either missed or obscured) ( table 1). Of note, the terms
simple partial, complex partial, and secondarily generalized have been eliminated, since they
were difficult to define pragmatically and were often used incorrectly.

Generalized seizures are further broken down into motor and nonmotor (absence) seizures.
Focal seizures are further subdivided based on level of awareness (aware, impaired awareness,
or unknown awareness). Additionally, focal seizures are subgrouped into motor and nonmotor
seizures, based on signs and symptoms at onset. Additional descriptors for both generalized
and focal seizures may be added based on specific motor or nonmotor symptoms. Focal
seizures can also be more clearly described based on their elemental features ( table 2).

The term "focal to bilateral tonic clonic" is used to describe a seizure that begins focally but then
spreads to engage bilateral networks.

LEVEL 2: EPILEPSY BASED ON SEIZURE TYPE

The 1989 classification categorized epilepsies as either focal or generalized. The 2017
classification retains these terms, but acknowledges that not all epilepsies can be dichotomized
into these two categories [2,5,6]. Thus, two additional categories have been added: generalized
and focal epilepsy and unknown if generalized or focal epilepsy.

Generalized epilepsy — Epilepsy is considered generalized if the seizures originate at some

point within, or rapidly engage bilaterally distributed networks, which can be subcortical or
cortical structures and are frequently both. Generalized seizures do not need to necessarily
include the entire cortex, however, and they may be asymmetric. Individuals with generalized
epilepsy typically show generalized spike-wave or generalized paroxysmal fast activity on
electroencephalogram (EEG). (See "Electroencephalography (EEG) in the diagnosis of seizures
and epilepsy", section on 'EEG findings in patients with epilepsy'.)

Focal epilepsy — The term focal has replaced partial to describe epilepsy associated with
seizures that are inferred from clinical or EEG data to originate in networks limited to one
hemisphere [2,4-6]. Focal seizures may arise from either subcortical structures or neocortex.
Most individuals with focal epilepsy show focal or multifocal discharges on interictal EEG.

Generalized and focal epilepsy — This term should be used for epilepsies that have both
generalized and focal seizures. This category includes several epilepsy syndromes, particularly
those with onset in early childhood, such as Dravet syndrome or Lennox-Gastaut syndrome, but
may also be relevant for epilepsies associated with diffuse or focal structural, genetic, or
metabolic etiologies. The interictal EEG may show both generalized and focal/multifocal
discharges, or epileptiform discharges may be absent. (See "Dravet syndrome: Genetics, clinical
features, and diagnosis" and "Lennox-Gastaut syndrome".)

Unknown if generalized or focal epilepsy — This term is used for epilepsies with seizures in
which it cannot be clearly determined whether onset is focal or generalized. A key example is
epileptic spasms, which may appear generalized despite being caused by a focal lesion. The
term unknown should also be used in an individual who presents with a generalized tonic-clonic
seizure and normal examination but whose EEG and neuroimaging is either noninformative or
unavailable.

LEVEL 3: EPILEPSY SYNDROME

An epilepsy syndrome represents a complex of clinical features, signs and symptoms that
together define a distinctive, recognizable clinical seizure disorder [2,5,6]. Some syndromes are
highly correlated with a single specific etiology (eg, severe loss of function SCN1A variants in
Dravet syndrome), whereas others may be due to a broad range of causes (eg, infantile spasms
syndrome or Lennox-Gastaut syndrome).

Many syndromes can be identified based on age at onset, seizure type(s),

electroencephalogram (EEG) characteristics, etiology, and associated comorbidities ( figure 2
and table 3). The ILAE website EpilepsyDiagnosis.org is a resource for the diagnosis of
epilepsy syndromes and contains parameters for diagnosis, as well as videos of specific seizure
types and images of characteristic EEG findings.

Epilepsy syndromes are divided into epilepsy type (focal, generalized, or focal and generalized)
with a separate category for syndromes with developmental and epileptic encephalopathy or
with progressive neurological deterioration.

● Neonatal – In the neonatal period, recognized epilepsy syndromes ( table 4) include

(see "Overview of neonatal epilepsy syndromes"):

• Self-limited familial and nonfamilial neonatal epilepsy

• Early infantile developmental and epileptic encephalopathy, which encompasses
syndromes formerly known as early myoclonic encephalopathy and Ohtahara
syndrome

● Infancy – Epilepsy syndromes of infancy ( table 4) include (see "Epilepsy syndromes in

children"):

• Focal epilepsy syndromes:

- Self-limited familial and nonfamilial neonatal and infantile epilepsy
- Self-limited familial and nonfamilial infantile epilepsy

• Generalized epilepsy syndromes:

- Myoclonic epilepsy in infancy

• Focal and generalized epilepsy syndromes:

- Genetic epilepsy with febrile seizures plus

• Syndromes with developmental and epileptic encephalopathies:

- Epilepsy of infancy with migrating focal seizures
- Infantile spasms syndrome
- Dravet syndrome
- Gelastic seizures with hypothalamic hamartoma
● Childhood – Epilepsy syndromes of childhood include (see "Epilepsy syndromes in
children"):

• Focal epilepsy syndromes:

- Self-limited epilepsy with autonomic seizures (SeLEAS), formerly known as
Panayiotopoulos syndrome
- Self-limited epilepsy with centrotemporal spikes (SeLECTS), formerly known as
benign epilepsy with centrotemporal spikes
- Childhood occipital visual epilepsy – formerly known as late-onset childhood
occipital epilepsy (Gastaut type)

• Generalized epilepsy syndromes:

- Childhood absence epilepsy
- Epilepsy with myoclonic absences
- Epilepsy with eyelid myoclonia (formerly known as Jeavons syndrome)

• Focal and generalized epilepsy syndromes:

- Photosensitive occipital lobe epilepsy

• Syndromes with developmental and epileptic encephalopathies:

- Epilepsy with myoclonic-atonic seizures (EMAtS), formerly known as Doose
syndrome or myoclonic-atonic epilepsy
- Lennox-Gastaut syndrome
- Developmental and epileptic encephalopathy with spike-wave activation in sleep
(DEE-SWAS), which encompasses the former syndromes of epileptic
encephalopathy with continuous spike-and-wave during sleep (CSWS) and Landau-
Kleffner syndrome
- Hemiconvulsion-hemiplegia-epilepsy syndrome (HHE)
- Febrile infection–related epilepsy syndrome (FIRES)

● Adolescence, adulthood, or variable age – Epilepsy syndromes that begin in

adolescence, adulthood, or at variable ages include:

• Focal epilepsy syndromes:

- Sleep-related hypermotor epilepsy
- Epilepsy with auditory features
- Familial focal epilepsy with variable foci (FFEVF)
- Mesial temporal lobe epilepsy with hippocampal sclerosis

• Generalized epilepsy syndromes:

 - Juvenile absence epilepsy
- Juvenile myoclonic epilepsy
- Generalized tonic-clonic seizures alone

• Focal and generalized epilepsy syndromes:

- Epilepsy with reading-induced seizures (ERIS)

• Syndromes with developmental and epileptic encephalopathies or with progressive

neurological deterioration:
- Rasmussen syndrome
- Progressive myoclonic epilepsy

Specific epilepsy syndromes are more commonly identified in children and adolescents than in
adults. The diagnosis can provide specific information on natural history, associated
comorbidities, particularly intellectual disability and psychiatric features, and management. (See
"Overview of neonatal epilepsy syndromes" and "Epilepsy syndromes in children".)

OTHER CLASSIFICATION VARIABLES

Etiology — Etiologic classification and terminology has evolved over the years. The 2017 ILAE
Classification of the Epilepsies recognizes six etiologic categories: genetic, structural, metabolic,
immune, infectious, and unknown [2,5,6]. The terms idiopathic, symptomatic, and cryptogenic
were eliminated as of the 2010 revision [4]. Etiology of epilepsy should be considered at all
three levels of diagnosis.

Some etiologies are best described by a combination of categories. For example, tuberous
sclerosis would be described as a genetic-structural etiology, and Leigh syndrome as a genetic-
metabolic etiology.

Genetic — A genetic etiology is defined when epilepsy is the direct result of a known or
presumed genetic defect and seizures are the core symptom of the disorder [4]. A genetic
etiology is most frequently based on family aggregation and twin studies; only a minority of
patients have a known pathogenic genetic variant, but this is changing rapidly with advances in
molecular technologies.

This category includes the syndromes of the idiopathic generalized epilepsies, a subgroup of
the genetic generalized epilepsies [7]. Examples include childhood absence epilepsy, juvenile
absence epilepsy, juvenile myoclonic epilepsy, and generalized tonic-clonic seizures alone, in
which there is strong evidence from both family and twin studies of a heritable etiology. These
conditions are caused by the summed final effect of multiple gene abnormalities and variations,
which increase susceptibility to seizures (polygenic), often with additional environmental
contributions (complex). In these syndromes, cognition is usually normal and seizure control is
most often favorable. (See "Childhood absence epilepsy" and "Juvenile myoclonic epilepsy".)

Other genetic causes may be associated with intellectual disability and poor prognosis for
seizure control, including Dravet syndrome, PCDH19-related epilepsy, and Down syndrome. (See
"Epilepsy syndromes in children" and "Dravet syndrome: Genetics, clinical features, and
diagnosis".)

Structural — Distinct structural causes may be associated with a substantially increased risk of
developing epilepsy. Structural etiologies may be congenital (eg, cortical dysplasia, tuberous
sclerosis) or acquired (eg, stroke, trauma, infection, immune-based).

Metabolic — A metabolic etiology is defined when a patient has a documented metabolic

condition that is associated with a substantially increased risk of developing epilepsy. Examples
include glucose transporter deficiency, creatine deficiency syndromes, and mitochondrial
cytopathies. Many of these conditions are also detected using genetic studies.

Immune — Distinct immune mediated etiologies are defined as those in which there is
evidence of central nervous system inflammation that results in epilepsy. Examples of immune
etiologies include Rasmussen encephalitis, anti-N-methyl-D-aspartate (NMDA) receptor
encephalitis. (See "Autoimmune (including paraneoplastic) encephalitis: Clinical features and
diagnosis".)

Infectious — Central nervous system infection may result in both acute symptomatic seizures
as well as epilepsy. Infections are one of the most important causes of epilepsy worldwide.
Examples of infectious etiologies of epilepsy include HIV, neurocysticercosis, malaria, and
tuberculosis.

Unknown — The term unknown has replaced the term cryptogenic, and simply means that
the nature of the underlying cause is not currently known. All types of epilepsies with normal
imaging and no documented genetic, metabolic, immune or infectious etiology are included in
this category.

This category should also be used for disorders previously classified as idiopathic focal epilepsy,
including self-limited epilepsy with centrotemporal spikes and self-limited epilepsy with
autonomic seizures; in such disorders, there may be some genetic contribution to the epilepsy,
but current evidence does not suggest that genetic factors are paramount. Generalized
epilepsies that do not fit into one of the generalized genetic epilepsy syndromes, and for which
there is insufficient evidence to support a heritable cause, should also be classified as unknown.

Epilepsies of unknown cause are common, accounting for approximately one-third of all cases.
In this heterogeneous group of disorders, it is particularly important to collect further
information on other relevant features, including cognitive and developmental antecedents and
consequences, abnormalities on neurological examination, electroencephalography (EEG)
features, provoking or triggering factors, natural history, age at onset, and other features.

Comorbidities — All epilepsies, at any level of the diagnostic framework, can have associated
cognitive, psychological, and behavioral comorbidities, which may impact quality of life even
more profoundly than the seizures. (See "Comorbidities and complications of epilepsy in adults"
and "Seizures and epilepsy in children: Initial treatment and monitoring", section on 'Psychiatric
and behavioral health screening'.)

Identification of specific etiologies and syndromes may assist in prediction of type and severity
of comorbidity. As an example, infantile spasms syndrome predicts significantly greater risk of
intellectual disability and autism spectrum disorder, and girls with pathogenic PCDH19 variants
are also at high risk of behavioral problems and autism spectrum disorder. (See "Infantile
epileptic spasms syndrome: Clinical features and diagnosis" and "Dravet syndrome: Genetics,
clinical features, and diagnosis", section on 'Differential diagnosis'.)

OTHER TERMINOLOGY

The term epileptic encephalopathy applies to epilepsies with encephalopathic features that may
present or worsen after onset of epilepsy [4]. This means that ongoing epileptic activity
adversely impacts cognition and behavior, above and beyond what can be ascribed to the
underlying etiology, if one is present (eg, cortical dysplasia).

Inherent to this concept is that amelioration of epileptiform activity has the potential to improve
the developmental consequences of the disorder. However, many etiologies for epileptic
encephalopathies also result in cognitive slowing and regression, independent of epilepsy
(developmental encephalopathy), and it can be challenging to determine the degree to which
developmental impact is caused by frequent seizures and epileptiform discharges versus the
underlying etiology.

The 2017 proposal suggests broadening of this terminology to include both developmental
and/or epileptic encephalopathy, to emphasize that both the underlying etiology and the
epileptic process may independently impact development [2,5,6]. Acceptable terms include
epileptic encephalopathy, developmental encephalopathy, or developmental epileptic
encephalopathy. The term "gene name" encephalopathy (eg, KCNQ2 encephalopathy) can be
used when a genetic pathogenic variant of major effect is identified.

The terms benign, malignant, and catastrophic have been used in the past to describe the
natural history of epilepsy. The 2010 proposal suggested replacing the term benign with more
descriptive terms, such as self-limited (for epilepsies in which spontaneous remission is likely)
and pharmacoresponsive (for those in which there is high likelihood of rapid control with
medication) [4]. Pharmacoresistent is the preferred term to replace malignant or catastrophic.

SUMMARY

● Significant advances in neuroimaging, genomic technologies, and molecular biology have

highlighted the need for an evolving system of classification and terminology for seizures
and epilepsy. The International League Against Epilepsy (ILAE) classification system is
widely used and has been updated most recently in 2010 and 2017, with several key
changes from prior versions. The ILAE classification allows for diagnosis at three levels
(see 'Introduction' above):

• Seizure types
• Epilepsy types
• Epilepsy syndromes

● The diagnosis of seizure requires that the clinician identify that the patient has had an
epileptic seizure as opposed to some other type of paroxysmal event, and then establish
the type of seizure(s) ( table 1). The four major seizure types are generalized, focal,
unknown, and unclassified. (See 'Level 1: Seizure type' above.)

● Epilepsies can be subdivided into generalized, focal, generalized and focal, and unknown
( figure 1). The latter includes epileptic spasms, for which current knowledge is
inadequate to determine whether onset is focal or generalized. (See 'Level 2: Epilepsy
based on seizure type' above.)

● An epilepsy syndrome represents a distinct clinical entity, defined as a complex of clinical

features, signs and symptoms that together define a distinctive, recognizable clinical
disorder ( table 3). (See 'Level 3: Epilepsy syndrome' above.)

● The 2017 ILAE classification recognizes six etiologic categories: genetic, structural,
metabolic, immune, infectious, and unknown. (See 'Etiology' above.)
ACKNOWLEDGMENT

The editorial staff at UpToDate would like to acknowledge Christian M Korff, MD, who
contributed to an earlier version of this topic review.

Use of UpToDate is subject to the Terms of Use.

REFERENCES

1. Fisher RS, Acevedo C, Arzimanoglou A, et al. ILAE official report: a practical clinical
definition of epilepsy. Epilepsia 2014; 55:475.

2. Scheffer IE, Berkovic S, Capovilla G, et al. ILAE classification of the epilepsies: Position paper
of the ILAE Commission for Classification and Terminology. Epilepsia 2017; 58:512.
3. Bosak M, Słowik A, Kacorzyk R, Turaj W. Implementation of the new ILAE classification of
epilepsies into clinical practice - A cohort study. Epilepsy Behav 2019; 96:28.
4. Berg AT, Berkovic SF, Brodie MJ, et al. Revised terminology and concepts for organization of
seizures and epilepsies: report of the ILAE Commission on Classification and Terminology,
2005-2009. Epilepsia 2010; 51:676.
5. Fisher RS, Cross JH, French JA, et al. Operational classification of seizure types by the
International League Against Epilepsy: Position Paper of the ILAE Commission for
Classification and Terminology. Epilepsia 2017; 58:522.

6. Fisher RS, Cross JH, D'Souza C, et al. Instruction manual for the ILAE 2017 operational
classification of seizure types. Epilepsia 2017; 58:531.

7. Wirrell EC, Nabbout R, Scheffer IE, et al. Methodology for classification and definition of
epilepsy syndromes with list of syndromes: Report of the ILAE Task Force on Nosology and
Definitions. Epilepsia 2022; 63:1333.
Topic 14094 Version 19.0
GRAPHICS

International League Against Epilepsy (ILAE) framework for seizure and epileps
classification

From: Scheffer IE, Berkovic S, Capovila G, et al. ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classific
Terminology. Epilepsia 2017. DOI: 10.1111/epi.13709. http://onlinelibrary.wiley.com/wol1/doi/10.1111/epi.13709/abstract. Copyright
International League Against Epilepsy. Reproduced with permission of John Wiley & Sons Inc. This image has been provided by or is ow
Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared or emailed. Please contact Wiley's per
department either via email: permissions@wiley.com or use the RightsLink service by clicking on the 'Request Permission' link accomp
article on Wiley Online Library (http://onlinelibrary.wiley.com).

Graphic 112425 Version 1.0

International League Against Epilepsy (ILAE) classification of seizure types

Generalized onset seizures

Motor Nonmotor (absence)

Tonic-clonic Typical
Clonic Atypical
Tonic Myoclonic
Myoclonic Eyelid myoclonia
Myoclonic-tonic-clonic
Myoclonic-atonic
Atonic
Epileptic spasms

Focal onset seizures

Motor onset Nonmotor onset

Aware Aware
Impaired awareness Impaired awareness
Unknown awareness Unknown awareness

Automatisms Autonomic
Atonic* Behavior arrest
Clonic Cognitive
Epileptic spasms* Emotional
Hyperkinetic Sensory
Myoclonic
Tonic

Focal to bilateral tonic-clonic Focal to bilateral tonic-clonic

Unknown onset seizures

Motor Nonmotor

Tonic-clonic Behavior arrest

Epileptic spasms

Unclassified seizures ¶

ILAE: International League Against Epilepsy.

* Degree of awareness usually is not specified.

​¶ Due to inadequate information or inability to place in other categories.

Adapted from: Fisher RS, Cross JH, French JA, et al. Operational classification of seizure types by the International League
Against Epilepsy: Position paper of the ILAE Commission for Classification and Terminology. Epilepsia 2017.

Graphic 91409 Version 4.0

Common descriptors of behaviors during focal seizures (alphabetically)

Cognitive Automatisms

Acalculia Aggression
Aphasia Eye-blinking
Attention impairment Head-nodding
Déjà vu or jamais vu Manual
Dissociation Oral-facial
Dysphasia Pedaling
Hallucinations Pelvic thrusting
Illusions Perseveration
Memory impairment Running (cursive)
Neglect Sexual
Forced thinking Undressing
Responsiveness impairment Vocalization/speech
Walking

Emotional or affective Motor

Agitation Dysarthria
Anger Dystonic
Anxiety Fencer's posture (figure-of-4)
Crying (dacrystic) Incoordination
Fear Jacksonian
Laughing (gelastic) Paralysis
Paranoia Paresis
Pleasure Versive

Autonomic Sensory

Asystole Auditory
Bradycardia Gustatory
Erection Hot-cold sensations
Flushing Olfactory
Gastrointestinal Somatosensory
Hyper/hypoventilation Vestibular
Nausea or vomiting Visual
Pallor
Laterality
Palpitations
Piloerection Left
Respiratory changes Right
Tachycardia Bilateral
From: Fisher RS, Cross JH, D'Souza C, et al. Instruction manual for the ILAE 2017 operational classification of seizure types.
Epilepsia 2017. DOI: 10.1111/epi.13671. http://onlinelibrary.wiley.com/wol1/doi/10.1111/epi.13671/abstract. Copyright ©
2017 The International League Against Epilepsy. Reproduced with permission of John Wiley & Sons Inc. This image has been
provided by or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared
or emailed. Please contact Wiley's permissions department either via email: permissions@wiley.com or use the RightsLink
service by clicking on the 'Request Permission' link accompanying this article on Wiley Online Library
(http://onlinelibrary.wiley.com).

Graphic 94645 Version 3.0

Classification of epilepsy syndromes based on age at presentation
Classification of epilepsy syndromes based on age at presentation. Shown are the typical ages of presentatio
ranges indicated by arrows. Focal epilepsy syndromes are indicated in blue, generalized epilepsy syndromes
green, focal and generalized syndromes in pink, and syndromes with developmental and/or epileptic
encephalopathy or with progressive neurologic deterioration in red.

From: Wirrell EC, Nabbout R, Scheffer IE, et al. Methodology for classification and definition of epilepsy syndromes with list of syndrom
Report of the ILAE Task Force on Nosology and Definitions. Epilepsia 2022; 63:1333. Copyright © 2022 The Authors. Available at:
https://onlinelibrary.wiley.com/doi/10.1111/epi.17237 (Accessed on June 8, 2023). Reproduced under the terms of the Creative Commo
Attribution License 4.0.

Graphic 141935 Version 1.0

Epilepsy syndrome abbreviations

Syndrome
Syndrome name Abbreviation
group

Neonatal-infant CDKL5-developmental and epileptic encephalopathy CDKL5-DEE

Dravet syndrome DS

Early infantile developmental and epileptic encephalopathy EIDEE

Epilepsy of infancy with migrating focal seizures EIMFS

Genetic epilepsy with febrile seizures plus GEFS+

Gelastic seizures with hypothalamic hamartoma GS-HH

Glucose transporter 1 deficiency syndrome GLUT1DS

Infantile epileptic spasm syndrome IESS

KCNQ2-developmental and epileptic encephalopathy KCNQ2-DEE

Myoclonic epilepsy in infancy MEI

Protocadherin 19 clustering epilepsy PCDH19

clustering
epilepsy

Pyridoxine-dependent (ALDH7A1) developmental and PD-DEE

epileptic encephalopathy

Pyridox(am)ine 5'-phosphate deficiency (PNPO) P5PD-DEE

developmental and epileptic encephalopathy

Self-limited familial neonatal-infantile epilepsy SeLFNIE

Self-limited infantile epilepsy SeLIE

Self-limited neonatal epilepsy SeLNE

Sturge-Weber syndrome SWS

Child Childhood occipital visual epilepsy COVE

Developmental and epileptic encephalopathy with spike- DEE-SWAS

and-wave activation in sleep

Epileptic encephalopathy with spike-and-wave activation in EE-SWAS

sleep

Epilepsy with eyelid myoclonia EEM

Epilepsy with myoclonic absences EMA

Epilepsy with myoclonic-atonic seizures EMAtS

Febrile infection-related epilepsy syndrome FIRES

 Hemiconvulsion-hemiplegia epilepsy syndrome HHE

Lennox-Gastaut syndrome LGS

Photosensitive occipital lobe epilepsy POLE

Self-limited epilepsy with autonomic seizures SeLEAS

Self-limited epilepsy with centrotemporal spikes SeLECTS

Idiopathic Childhood absence epilepsy CAE

generalized
Epilepsy with generalized tonic-clonic seizures alone GTCA
epilepsies
Juvenile absence epilepsy JAE

Juvenile myoclonic epilepsy JME

Variable age Epilepsy with auditory features EAF

Epilepsy with reading-induced seizures EwRIS

Familial focal epilepsy with variable foci FFEVF

Familial mesial temporal lobe epilepsy FMTLE

Mesial temporal lobe epilepsy with hippocampal sclerosis MTLE-HS

Progressive myoclonus epilepsies PME

Rasmussen syndrome RS

Sleep-related hypermotor (hyperkinetic) epilepsy SHE

From: Wirrell EC, Nabbout R, Scheffer IE, et al. Methodology for classification and definition of epilepsy syndromes with list
of syndromes: Report of the ILAE Task Force on Nosology and Definitions. Epilepsia 2022; 63(6):1333-1348.
https://onlinelibrary.wiley.com/doi/10.1111/epi.17237. Copyright © 2022 The Authors. Published on behalf of The
International League Against Epilepsy. Reproduced with permission of John Wiley & Sons Inc. This table has been provided by
or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared or emailed.
Please contact Wiley's permissions department either via email: permissions@wiley.com or use the RightsLink service by
clicking on the 'Request Permission' link accompanying this article on Wiley Online Library (https://onlinelibrary.wiley.com/).

Graphic 91410 Version 4.0

Epilepsy syndromes with neonatal/infantile onset

Focal

Self-limited neonatal epilepsy (SeLNE)

Self-limited familial neonatal and infantile epilepsy (SeLFNIE)

Self-limited infantile epilepsy (SeLIE)

Focal and/or generalized

Genetic epilepsy with febrile seizures plus (GEFS+)

Generalized

Myoclonic epilepsy in infancy (MEI)

Developmental and epileptic encephalopathies (DEEs)

Early infantile DEE (EIDEE)

Epilepsy in infancy with migrating focal seizures (EIMFS)

Infantile epileptic spasms syndrome (IESS)

Dravet syndrome (DS)

Etiology-specific syndromes*
KCNQ2 DEE (KCNQ2-DEE)

Pyridoxine-dependent (ALDH7A1) DEE (PD-DEE)

Pyridoxamine 5'-phosphate deficiency (PNPO) DEE (P5PD-DEE)

CDKL5 DEE (CDKL5-DEE)

Protocadherin 19 clustering epilepsy (PCDH19 clustering epilepsy)

Glucose transporter 1 deficiency syndrome (GLUT1DS)

Sturge-Weber syndrome (SWS)

Gelastic seizures with hypothalamic hamartoma (GS-HH)

* Most etiology-specific syndromes that begin in the neonatal or infantile period are DEEs.

From: Zuberi SM, Wirrell E, Yozawitz E, et al. ILAE classification and definition of epilepsy syndromes with onset in neonates
and infants: Position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia 2022; 63(6):1349-1397.
https://onlinelibrary.wiley.com/doi/10.1111/epi.17239. Copyright © 2022 The Authors. Published on behalf of The
International League Against Epilepsy. Adapted with permission of John Wiley & Sons Inc. This table has been provided by or
is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared or emailed.
Please contact Wiley's permissions department either via email: permissions@wiley.com or use the RightsLink service by
clicking on the 'Request Permission' link accompanying this article on Wiley Online Library (https://onlinelibrary.wiley.com/).
Contributor Disclosures
Elaine Wirrell, MD Other Financial Interest: Acadia [Data Safety Monitoring Board - Rett syndrome];
Amicus [Data Safety Monitoring Board - Batten disease]; Encoded Therapeutics [Data Safety Monitoring
Board - Genetic treatment for Dravet syndrome]; Longboard [Data Safety Monitoring Board - Epilepsy];
Neurocrine [Data Safety Monitoring Board - Epilepsy]. All of the relevant financial relationships listed have
been mitigated. Steven C Schachter, MD Patent Holder: Supernus Pharmaceuticals [Epilepsy].
Consultant/Advisory Boards: Supernus Pharmaceuticals [Epilepsy]. All of the relevant financial
relationships listed have been mitigated. Douglas R Nordli, Jr, MD No relevant financial relationship(s)
with ineligible companies to disclose. John F Dashe, MD, PhD No relevant financial relationship(s) with
ineligible companies to disclose.

Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
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