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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: Jan 2022. | This topic last updated: Aug 17, 2021.

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).

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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.

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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 "Epilepsy syndromes in children",
section on '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.

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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 (
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 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 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:

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- 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:

- Myoclonic-atonic epilepsy (formerly known as Doose syndrome)
- 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

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- 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

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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 "Paraneoplastic and autoimmune encephalitis".)

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

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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 "Clinical features and diagnosis of infantile spasms" 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

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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.)

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● 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

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epilepsy syndromes: Report of the ILAE Task Force on Nosology and Definitions.
Epilepsia 2021.
Topic 14094 Version 18.0

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GRAPHICS

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

From: Scheffer IE, Berkovic S, Capovila G, et al. ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and
Terminology. Epilepsia 2017. DOI: 10.1111/epi.13709. http://onlinelibrary.wiley.com/wol1/doi/10.1111/epi.13709/abstract. Copyright © 2017 Th
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 thi
article on Wiley Online Library (http://onlinelibrary.wiley.com).

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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 ¶

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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.

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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

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▪ 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).

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Examples of epilepsy syndromes and other epilepsies arranged according to

age

Neonatal period

▪ Self-limited (benign) neonatal seizures

▪ Self-limited (benign) familial neonatal epilepsy
▪ Early myoclonic encephalopathy (EME)
▪ Ohtahara syndrome

Infancy (onset under 2 years)

▪ Febrile seizures
▪ Febrile seizures plus (FS+)
▪ Epilepsy of infancy with migrating focal seizures
▪ West syndrome
▪ Myoclonic epilepsy in infancy (MEI)
▪ Benign infantile epilepsy
▪ Benign familial infantile epilepsy
▪ Dravet syndrome
▪ Myoclonic encephalopathy in non-progressive disorders

Childhood

▪ Febrile seizures
▪ Febrile seizures plus (FS+)
▪ Early onset childhood occipital epilepsy (Panayiotopoulos type)
▪ Epilepsy with myoclonic atonic (previously astatic) seizures
▪ Benign epilepsy with centrotemporal spikes (BECTS)
▪ Autosomal-dominant nocturnal frontal lobe epilepsy (ADNFLE)
▪ Late onset childhood occipital epilepsy (Gastaut type)
▪ Childhood absence epilepsy (CAE)
▪ Epilepsy with myoclonic absences
▪ Lennox-Gastaut syndrome
▪ Epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS)
▪ Landau-Kleffner syndrome

Adolescent to adult

▪ Juvenile absence epilepsy (JAE)

▪ Juvenile myoclonic epilepsy (JME)
▪ Epilepsy with generalized tonic-clonic seizures alone (GTCA)
▪ Autosomal dominant epilepsy with auditory features (ADEAF)

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▪ Other familial temporal lobe epilepsies

Familial epilepsy syndromes

▪ Familial focal epilepsy with variable foci (FFEVF)

▪ Genetic epilepsy with febrile seizures plus (GEFS+)

ILAE: International League Against Epilepsy.

Adapted from: 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.
http://onlinelibrary.wiley.com/doi/10.1111/j.1528-1167.2010.02522.x/abstract. Copyright © 2010 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 on 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).

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Contributor Disclosures
Elaine Wirrell, MD Consultant/Advisory Boards: Biomarin [Epilepsy]; Biocodex Inc [Dravet
syndrome/epilepsy]; Encoded Therapeutics [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 addressed by vetting through a multi-level review process, and through requirements for
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