Revista Mexicana de Neurociencia

REVIEW ARTICLE

Clinical guideline: definition and classification of epilepsy

Juan C. Reséndiz-Aparicio1, Juan C. Pérez-García2, Efraín Olivas-Peña3, Enrique García-Cuevas4, Yuridia
L. Roque-Villavicencio5, Marisela Hernández-Hernández6, Jaime I. Castro-Macías7 and
Jesús D. Rayo-Mares8
1PPE, Instituto
Nacional de Neurología y Neurocirugía Dr. Manuel Velasco Suárez y Hospital Psiquiátrico Infantil Dr. Juan N. Navarro, Mexico City;
2HospitalChristus Muguerza UPAEP, Puebla; 3Hospital de la Mujer, Yautepec, Morelos; 4Hospital General Acapulco, Guerrero; 5Hospital Civil de
Guadalajara, Jalisco; 6Hospital Central Sur de Alta Especialidad Pemex, Mexico City; 7Hospital Regional de Alta Especialidad del Bajío, León,
Guanajuato; 8Hospital de Pediatría del Centro Médico Nacional Siglo XXI IMSS, Mexico City. Mexico

Abstract
The current definition of epilepsy proposes three possibilities to consider this diagnosis, the first when a patient has two or
more unprovoked or reflex seizures in >24 h; the second, an unprovoked or reflex seizure with at least a 60% probability of
continuing to present seizures; and the third, the presence of an epileptic syndrome. The classification of the type of seizure
divides them into three possibilities depending on how they begin; they can be of focal, generalized, or unknown onset. Focal
seizures can be subclassified into those that have or have not lost consciousness, then categorized as to whether the symp-
toms are motor or non-motor, and further give a descriptor of the event, which is nothing else but the description of symptoms
and signs presented by the patient during his seizure. The classification of the type of epilepsy, proposes three diagnostic
levels, the first related to the type of seizure, the second to the type of epilepsy, and the third to the type of epileptic syndrome,
without forgetting etiology and comorbidity. These concepts are basic for the approach of any patient who presents epilepsy.

Key words: Epilepsy. Definition. Classification.

Introduction and not a review of the available evidence. The ILAE

and its workgroups have strived to provide a standard
This being a clinical guide, it is developed based on terminology to be used worldwide.
research questions under the PICO method; answers
are presented, and it concludes with recommendations.
In this clinical guide, we do not mention levels of evi- Question 1. What is an epileptic seizure?
dence since the references used are current articles An epileptic seizure is defined as the transitory ma-
from the International League Against Epilepsy (ILAE)1-3, nifestation of signs and/or symptoms caused by an
which is the official agency for this disease worldwide, anomalous excessive neuronal activity in the brain4.

Correspondence:
Juan Carlos Reséndiz-Aparicio
PPE, Instituto Nacional de Neurología y
Neurocirugía Dr. Manuel Velasco Suárez
Hospital Psiquiátrico Infantil Dr. Juan N. Navarro Date of reception: 21-01-2019 Disponible en internet: 12-04-2019
Mexico City, Mexico Date of acceptance: 28-02-2019 Rev Mex Neuroci. 2019;20(2):63-68
E-mail: [email protected] DOI: 10.24875/RMN.M19000024 www.revmexneurociencia.com
1665-5044/© 2019. Academia Mexicana de Neurología A.C. Publicado por Permanyer México. Este es un artículo Open Access bajo la licencia CC BY-NC-ND
(http://creativecommons.org/licenses/by-nc-nd/4.0/).

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Question 2. What is an unprovoked The classification is divided into three, depending on

seizure? the patient’s symptoms at seizure onset:
Focal onset seizure/Generalized onset seizure/Unk-
The term unprovoked implies the absence of a nown onset seizure.
temporal or reversible factor that reduces the convulsive Focal onset seizures originate within a network limi-
threshold and provokes a seizure at that moment. The ted to one hemisphere; they can be localized or more
opposite of this type of phenomenon is an acute symp- widely distributed. Generalized seizures are those that
tomatic seizure, defined as a seizure that occurs in tem- originate at one point with wide and rapid participation
poral relation during cerebral injury, which can be meta- of bilaterally distributed networks. Those seizures whe-
bolic, infectious, toxic, structural, or inflammatory4,5. re it cannot be distinguished whether they are of focal
or generalized onset with a confidence level of 80%,
Question 3. What is the current definition must be considered of unknown onset (Fig. 1).
of epilepsy?
A diagnosis of epilepsy is established after any of the Question 6. How are focal onset epileptic
following situations4: seizures currently classified?
1. Two or more unprovoked or reflex seizures occurring The state of awareness is a differentiating factor for
with >24 h difference. the type of seizure in focal seizures1,2. Awareness refers
2. An unprovoked or reflex seizure with a probability of at to the relationship between oneself and the external
least 60% of presenting a future seizure (similar range environment. In epilepsy, we establish awareness as the
as the general risk of recurrence, after two unprovoked mental state, with both subjective and objective aspects,
seizures that appear in the following 10 years). that encompasses the sense of self as a unique entity,
3. Diagnosed epileptic syndrome. capable of response, and memory. A  focal seizure is
classified by the degree of awareness during onset,
Question 4. How is the risk of recurrence which can be altered at any time during the event.
of an epileptic seizure defined? Focal seizures, with or without impaired awareness, can
be subclassified depending on whether they initiate with
The events that have been documented to increase motor or non-motor symptoms. In addition, the 2017 clas-
the risk of recurrence of an epileptic seizure are cere- sification recommends adding what they call “descriptors,”
brovascular disease, both ischemic and hemorrhagic, which are symptoms and signs that the patient presents
cranioencephalic trauma, abnormal electric activity in during the seizure1,2. Descriptors have been divided into
an EEG compatible with an epileptic seizure, or a cranial six groups: motor, automatisms, sensory, emotional, cog-
magnetic resonance showing a lesion compatible with nitive, or autonomic. To remember these descriptors more
a diagnosis of epilepsy5. If a patient, after a first unpro- easily, you can use the mnemotechny MASECA, which is
voked seizure is predisposed to continued generation the most famous brand of tortilla dough in Mexico8.
of seizures of at least 60%, a diagnosis of epilepsy must
be considered4. If this is not the case, to establish a
diagnosis of epilepsy one must consider option one (two
Question 7. How are the symptoms and
or more unprovoked seizures >24 h apart).
signs divided into focal seizures?
Epilepsy is considered as resolved in those patients
that have an age-dependent epileptic syndrome but
Motor
have grown older than the corresponding age for the Involvement of the musculature in any way. An in-
specific syndrome or in patients that have remained crease (positive) or decrease (negative) in muscular
seizure-free for the past ten, without taking antiepileptic contraction to produce movement. The most frequent
drugs in the last 5 years4. are: motor arrest, astatic, clonic, dysarthria, dystonic,
pelvic thrusting, figure-of-4, hypokinetic, hyperkinetic,
incoordination, Jacksonian, myoclonic, paralysis, pare-
Question 5. What is the current
sis, pedaling, fencer’s posture, and versive.
classification by type of epileptic seizure?
The most frequent clinical confusion is in differentia-
The ILAE decided to modify the classification system ting clonic seizures from myoclonic seizures. A  clonic
for epileptic seizures in 19816, and update it in 20107. seizure is a repetitive, regular movement that can be
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 J.C. Reséndiz-Aparicio, et al.: Definition and classification of epilepsy

Focal Onset Generalized Onset Unknown Onset

Aware Impaired
Awareness
MOTOR MOTOR
tonic clonic tonic clonic
clonic epileptic spasms
tonic NON MOTOR
myoclonic Behavior arrest
MOTOR ONSET
myoclonic tonic clonic
automatisms
myoclonic atonic
atonic*
atonic
clonic
epileptic spasms*
epileptic spasms*
ABSENCE
hyperkinetic
typical
myoclonic
atypical
tonic
myoclonic
NON MOTOR ONSET
palpebral myoclonia
Behavior arrest
autonomic
sensory
emotional
cognitive

Focal to bilateral Unclassified†

tónico clónica

* Can be focal or generalized, with or without alteration of awareness

†
Due to inadequate information or inability to be included in another category

Figure 1. Classification by type of seizure. ILAE, 2017.

symmetrical or asymmetrical and that involves the same oral-facial, perseveration, sexual, undressing, vocaliza-
muscle groups. A myoclonic seizure is a brief (< 100 ms), tion, walking, or running.
sudden, involuntary, single or multiple contractions of
the muscles or muscle groups, with variable topography
Sensory
(axial, proximal, or distal extremities). The myoclonic
seizure is less repetitive and sustained. Related with the senses, thus, in this case, are not
signs but symptoms. The most frequently reported
symptoms are: auditory, gustatory, olfactory, somato-
Automatisms
sensory, vestibular, visual, and pain.
Motor activity, which is more or less coordinated, that
generally occurs when awareness is deteriorated and
Emotional
after which the subject is generally (but not always)
amnesiac. It commonly resembles a voluntary move- These are seizures that present as having an emotion
ment. The most frequent include: aggression, manual, as a prominent initial feature, such as fear, pleasure or
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spontaneous euphoria, laughter, crying, expressed in seizure. It frequently occurs in clusters or bursts. Epi-
bursts, or flares. leptic spasms are more frequent in children but can
occur at all ages.
Absence seizures can be divided into four types:
Cognitive
Typical absence, atypical absence, myoclonic absence,
The most frequently reported data from this group and absence with palpebral myoclonus. Typical absen-
are: acalculia, aphasia, attention impairment, déjà vu ce seizures are of sudden onset, interrupting ongoing
or jamais vu phenomena, dysphasia, illusions, memory activities, blank stare, does not respond when spoken
impairment, forced thinking, altered responsiveness, or to, lasting from seconds to half a minute, and with very
hallucinations. quick recovery. It’s important to remember that the
word “absence” is not synonymous with blank stare
since this can also be encountered in focal onset
Autonomic
seizures2.
Alteration of the autonomic nervous system function, Atypical absence seizures show changes in tone that
which may involve the cardiovascular system, pupils, are more pronounced than in typical absence; the on-
diaphoresis, the gastrointestinal tract, vasomotor and set and cessation are not abrupt. Myoclonic absence
thermoregulation functions. presents with sudden, brief (<100 ms), involuntary,
In focal seizures, there are two sections that were not non-repetitive nor sustained, and absence. In absence
included in previous classifications, the epileptic spasm with palpebral myoclonus, we observe eyelids jerking
(previously only classified within generalized seizures) at a frequency under 3 per second, eyes commonly
and behavior arrest1,2. deviated upward, generally lasting <10 s, frequently
A focal onset seizure, with or without impaired awa- precipitated by ocular closure, with high possibility of
reness, motor or non-motor, can progress to a bilateral photosensitivity.
tonic-clonic activity. The term previously used for this
type of seizure was a secondary generalized partial
Question 9. When do we classify an
seizure. At present, we must classify it as a focal onset
epileptic seizure under the heading of
seizure that evolves into a bilateral tonic-clonic
unknown onset or unclassified?
seizure.
The term bilateral tonic-clonic is used for focal seizu- When there is no evidence about the onset of the
res that propagate to both cerebral hemispheres, while seizure, focal or generalized, it may be classified under
generalized is for seizures that originate simultaneously the heading of unknown onset seizure, and in this case,
in both cerebral hemispheres. a limited classification can be carried out with the fin-
dings that were observed.
The heading “Unclassified” applies to the type of
Question 8. ¿How are generalized
seizure when there are no data described in the ILAE
epileptic seizures currently classified?
classification of 2017, either because the information is
They are divided into seizures with motor or inadequate or because of unusual characteristics1,2.
non-motor symptoms. Among those with motor, symp-
toms are the generalized tonic-clonic, clonic, tonic,
myoclonic, myoclonic-tonic-clonic, myoclonic-atonic
Question 10. How are epilepsy and
seizures, and the epileptic spasm. For non-motor,
epileptic syndromes classified?
there are absence seizures1,2. A myoclonic-tonic-clo- Since April 2017 the epilepsy classification set forth
nic is a type of generalized seizure that was not in- by the ILAE establishes three levels of diagnosis: the
cluded in the previous classification. These seizures first level is the type of seizure, which incorporates all
imply one or various bilateral jerks (myoclonic) of the of the concepts in all diagnostic levels revised pre-
extremities, followed by a tonic-clonic seizure1,2,8. Ato- viously, focal, generalized or of unknown onset, and
nic seizures present sudden loss or reduction in mus- now we have added two important concepts at all
cle tone involving musculature of the head, trunk, diagnostic levels: patient comorbidities (associated
mandible, or extremities. pathological entities) and etiology. Some patients can
The epileptic spasm is usually more sustained than only stay at this diagnostic level because it is not pos-
a myoclonic movement, but not as sustained as a tonic sible to study more, and this can be valid or be the
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 J.C. Reséndiz-Aparicio, et al.: Definition and classification of epilepsy

Type of seizure Etiology

Focal Generalized Unknown
Structural

Genetic
Comorb d t es

Type of Epilepsy Infectious

Combined,
generalized
Focal Generalized and focal Unknown Metabolic

Immune

Unknown
Epileptic Syndrome

Figure 2. Classification of Epilepsy. ILAE 2017.

first step in their evaluation. The second diagnostic Question 11. How are seizures and
level applies when there is at least one encephalo- epilepsy in the newborn classified?
gram and a cerebral imaging study. At this level one
There are various classifications for neonatal seizu-
must establish the type of epilepsy, which can be fo-
res. The Volpe classification9 considers the clinical fin-
cal, generalized, or combined; this is for focal, gene-
dings (mostly used by pediatricians and neonatologists)
ralized (which is common in various epileptic syndro-
and the Mizrahi classification10 considers the physiopa-
mes) or unknown type seizures. The etiologic diagnosis
thologic origin, be it epileptic or non-epileptic. In 2018,
can be in any of the following fields: structural, gene-
the ILAE issued a new proposal to classify seizures in
tic, infectious, metabolic, immune, or unknown. On
the newborn (Fig. 3) that includes four parts: the pre-
occasion there can be >1 etiology in the same patient,
sentation, established in the critically ill newborn who
for example, a patient with tuberous sclerosis that has
must be under vigilance in view of the possibility of a
cortical tubers would suggest a structural etiology, but
seizure; diagnosis, by performing a video-EEG; mani-
also genetic due to the base disease. The third diag-
festation, which could be by clinical signs or only elec-
nostic level constitutes an epileptic syndrome. A group
troencephalographic data; and fourth, the type of sei-
of characteristics that incorporates types of seizure,
zure, which could be with motor symptoms. It is called
specific EEG findings, characteristics form imaging
sequential when the symptoms in the newborn show a
studies, age-dependent frequency, age at onset, and
motor sequence and the seizure is of a non-motor type.
remission when applicable, specific triggers, varia-
tions during the day, on occasion prognosis, distincti- Recommendation Level of recommendation
ve comorbidities both intellectual and psychiatric, and It’s desirable for health R‑PPE
all can have implications on etiology and treatment. professionals to know the
current definition of epilepsy to
This classification eliminates the term benign which be able to apply it
has been substituted by the terms autolimited or drug
It’s desirable that health R‑PPE
responsive (Fig. 2). professionals know and apply
Epileptic syndromes can be classified by age groups, the current classification of the
as was established by the ILEA classification of 20107, types of epileptic seizures and
epilepsies
but that is subject for another Guide.

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 Rev Mex Neuroci. 2019;20

Critically ill or with clinical suspicion Presentation

No seizure
Video EEG
(without EEG correlate) Diagnosis

Seizure
(with EEG correlate)

Without clinical signs

with clinical science (only electrographic)
Manifestation

MOTOR
automatisms
clonic
epileptic spasms
myoclonic
tonic
sequential
Seizure type
NON MOTOR
Behavior arrest
autonomic
UNCLASSIFIED

Figure 3. Diagram of the proposed classification for seizures in the newborn.

Conflicts of interest 4. Fisher R, Acevedo C, Arzimanoglou A, et al. Definición clínica practica

de la Epilepsia. Epilepsia. 2014;55:475-82.
In this article, there are no conflicts of interest by the 5. Thurman DJ, Beghi E, Begley CE, et al. Standards for epidemiologic
studies and surveillance of epilepsy. Epilepsia. 2011;52 Suppl 7:2-6.
authors or the source of funding. 6. Bancaud J, Rubio-Donnadieu F, Seino M, Dreifuss F, Penry K. Proposal
for revised clinical and electroencephalographic classification of epileptic
seizures. From the commission on classification and terminology of the
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