Classification of Epileptic Seizures

CLASSIFICATION OF
EPILEPTIC SEIZURES
Susan T. Herman
ABSTRACT
Correct classification of seizure type and epilepsy syndrome are important first steps
in the management of patients with epilepsy, providing critical information for
choice of diagnostic tests, selection of optimal treatment, and determination of
prognosis. The two currently accepted classifications are the International
Classification of Epileptic Seizures and the International Classification of Epilepsies
and Epileptic Syndromes. Epileptic seizures are divided into focal seizures, which
begin in a localized region of one hemisphere, and generalized seizures, which
involve both hemispheres at onset. Epilepsy syndromes are constellations of features
such as seizure type, age of onset, etiology, interictal deficits, and EEG and
neuroimaging findings. Epilepsy syndromes are divided into syndromes with focal
seizures, syndromes with generalized seizures, undetermined syndromes, and special
syndromes. They are further subdivided by etiology. Idiopathic syndromes have a
presumed genetic etiology, while symptomatic epilepsies have a clearly identified
cause. Probably symptomatic syndromes are those in which an etiology is suspected,
but the exact cause cannot be determined. Over the past 6 years, the International
League Against Epilepsy (ILAE) Task Force on Classification and Terminology has
proposed important modifications for these classification systems. In addition, a new
diagnostic scheme for patients with epilepsy provides structure in the form of five
diagnostic axes: ictal phenomenology, seizure type, epilepsy syndrome, etiology, and
impairment. This chapter focuses on the current classifications and proposed
diagnostic axes in adolescents and adults. Proposed modifications that reflect
important changes in classification philosophy will also be discussed.
Continuum Lifelong Learning Neurol 2007;13(4):1347.
INTRODUCTION
The first step in the treatment of epi-
lepsy is accurate classification of sei-
zure type and epilepsy syndrome. This
provides essential information regard-
ing necessary diagnostic tests, likely re-
sponse to antiepileptic drug (AED)
therapy, and long-term prognosis. The
International Classification of Epileptic
Seizures and the International Clas-
sification of Epilepsies and Epileptic
Syndromes are accepted worldwide,
providing a standardized system for
describing seizures and epilepsies. This
chapter will review the recently pro-
posed International League Against Epi-
lepsy (ILAE) diagnostic scheme, de-
tailing ictal semiology, seizure types,
and epilepsy syndromes. This classifi-
cation will set the stage for the next
chapters, including differential diagno-
sis and choice of AED therapy.
DEFINITIONS
Seizures are the hallmark symptom of
epilepsy. An epileptic seizure is a tran-
sient occurrence of signs and/or symp-
toms due to abnormal excessive or
synchronous neuronal activity in the
brain (Fisher et al, 2005). The new
13
KEY POINT:
A An epileptic
seizure is a
transient clinical
event due to
abnormal
neuronal activity
in the brain.
Note: Text referenced in the Quintessentials Preferred Responses, which appear
later in this issue, is indicated by gray shading throughout this chapter.
Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited.
definition emphasizes that seizures are
clinical phenomena; purely electroen-
cephalographic changes without clinical
manifestations are not considered in this
definition. Seizure manifestations vary
depending on whether the seizure is
focal (beginning in a limited part of one
hemisphere) or generalized (beginning
simultaneously in widespread bilateral
cortical regions), the function of the af-
fected cortex, and how the ictal dis-
charge spreads within the brain.
In the 1989 epilepsy classification,
epilepsy was defined as recurrent (two
or more) unprovoked seizures (Com-
mission on Classification and Terminol-
ogy of the International League Against
Epilepsy, 1989). This definition has
evolved in parallel with better under-
standing of the pathophysiology and
mechanism of seizure generation. Ex-
panding the definition to better reflect
the underlying mechanism of epi-
lepsy, the ILAE recently proposed that
epilepsy is a disorder of the brain
characterized by an enduring predis-
position to generate epileptic seizures
and by the neurobiologic, cognitive,
psychological, and social consequences
of this condition. The definition of epi-
lepsy requires the occurrence of at
least one epileptic seizure (Fisher et al,
2005). Now, patients with a single
seizure and a high risk of recurrence
(eg, those with a family history of epi-
lepsy, remote symptomatic brain in-
sult, or an EEG showing epileptiform
discharges) can be considered to have
epilepsy. Such patients may be started
on AEDs to prevent seizure recurrence
after a first seizure and, therefore, may
never meet the traditional definition
of epilepsy. This new mechanistic defi-
nition of epilepsy was greeted with
significant controversy (Fisher et al,
2005), including concerns that acute
symptomatic seizures would be inap-
propriately included in the definition
of epilepsy. Much of the controversy
stems from the difficulty in defining
what constitutes an enduring predis-
position to generate epileptic seizures.
Until and unless another seizure oc-
curs, it is often difficult to determine
a patients risk for subsequent sei-
zures. To make this definition useful
in clinical care and research, an opera-
tional threshold may need to be es-
tablished. For example, it might be
reasonable to set the threshold for
enduring predisposition as a higher
than 50% chance of seizure recurrence
without AED treatment.
An epilepsy syndrome is an epileptic
disorder characterized by similar fea-
tures, including seizure type(s), precip-
itating factors, age of onset, etiology,
neurologic and neuropsychological ab-
normalities, interictal and ictal EEG find-
ings, and neuroimaging findings (Engel,
2001; Engel, 2006b). Within the same
syndrome, patients share similar but not
identical underlying pathophysiologic
mechanisms, response to treatment,
and prognosis. Epilepsy syndromes
need to be flexible and modifiable as
our understanding of epilepsy evolves.
Many patients with epileptic seizures
cannot be classified according to the
current epilepsy syndrome classifica-
tion. This is especially common when
only a few seizures have occurred and
when full diagnostic testing is not
available. In these patients, the seizure
type may guide diagnosis andtreatment.
The ILAE has also proposed a defi-
nition for an epileptic disease. This is a
specific pathologic entity with a single,
well-described etiology. An example of an
epileptic disease would be Unverricht-
Lundborg disease, with a specific known
etiology. Progressive myoclonic epilepsy,
with many different etiologies, including
Unverricht-Lundborg, would be an epi-
lepsy syndrome.
CLINICAL APPROACH
A thorough history is essential for accu-
rate seizure classification. This should in-
clude detailed questions about seizure
14
KEY POINT:
A Epilepsy is
characterized by
(1) at least one
epileptic seizure;
(2) an enduring
predisposition to
epileptic seizures;
and (3) associated
cognitive,
psychological,
and social
consequences.
"
CLASSIFICATION OF EPILEPTIC SEIZURES
characteristics, from prodrome, through
initial manifestations and evolution, to
postictal symptoms. The examiner
should begin with open-ended ques-
tions, allowing the patient to describe
the seizure, but usually will need to ask
specific questions to elicit details that
the patient may not spontaneously
report. For example, a patient who de-
scribes paresthesias over the left side of
the body could be asked about where
the paresthesias begin, spread to other
body regions, time course of spread,
presence of motor symptoms, alteration
of consciousness, and postictal motor
or sensory dysfunction. If the patient
loses awareness during the seizure, a
similar description should be obtained
from one or more witnesses to the
seizure. Witnesses may miss the begin-
ning of the seizure, may not be able to
recall important localizing or lateralizing
features (Rugg-Gunn et al, 2001), or
may mislateralize motor features, espe-
cially for the first seizure or seizures that
occurred in the remote past. In some
cases, having the patient or witness act
out the typical movements of a seizure
may provide essential information. Sev-
eral structured questionnaires have
been developed that provide good
interrater reliability in seizure classifica-
tion (Ottman et al, 1993; Picot et al,
1999). Review of video and EEG record-
ings of a patients typical seizures
enables the most accurate classifica-
tions, but these are not available for
most patients. Classification by history
alone is less accurate, even when per-
formed by epilepsy specialists (Deacon
et al, 2003).
Understanding typical seizure mani-
festations and patterns of seizure
spread will often allow the examiner
to exclude nonepileptic events and to
estimate the location of the ictal-onset
zone. Ictal manifestations may be mis-
leading, however, in indicating seizure
onset. In some cases, seizures begin in
clinically silent brain regions, and the
initial clinical manifestations represent
seizure spread rather than seizure on-
set. For example, the first outward man-
ifestations of parietal seizures can
occur with spread to the motor cortex
or ipsilateral temporal lobe. Similarly,
scalp EEG may not detect the very
earliest ictal discharge and may reflect
propagation of the seizure. In patients
with multiple seizure types, differing
seizure semiology may suggest multi-
focal onsets. Ictal features in young
children differ from those in adults;
motor features such as clonus, tonic
posturing, and myoclonus are more
often present, and automatisms are
less commonly seen.
INTERNATIONAL LEAGUE
AGAINST EPILEPSY PROPOSED
DIAGNOSTIC SCHEME
In 2001, the ILAE published a new diag-
nostic scheme for patients with epi-
lepsy. The old classifications have not
been replaced, but some revisions have
been suggested. These are highlighted
in the sections below. The five diag-
nostic axes represent a flexible system
to fully describe seizures in individual
patients and to facilitate international
collaborations in teaching, clinical care,
and research. The classifications will be
treated as testable hypotheses (Engel,
2001) and will be revised as new
knowledge is accrued. Not all patients
fit into a specific epilepsy syndrome;
when a syndromic diagnosis cannot be
made, the seizure type can be used to
provide etiologic, treatment, and prog-
nostic information. The amount of
detail required for each of the axes will
depend on the purpose of the classifi-
cation. For example, different classifica-
tions may be needed for epidemiologic,
genetic, and surgical studies.
Axis 1: Ictal Phenomenology
The first axis (Engel, 2001) involves a
description of ictal clinical symptoms
and signs. This axis may not be needed
15
KEY POINTS:
A Detailed seizure
descriptions
should be
obtained from
both the patient
and, if possible,
one or more
witnesses to
the seizure.
A Seizure semiology
aids in localization
and lateralization
of seizure
onset, but both
clinical and EEG
features can be
misleading,
representing
seizure spread
rather than
seizure onset.
A The International
League Against
Epilepsy
diagnostic axes
for epilepsy
are ictal
phenomenology,
seizure type,
epilepsy
syndrome,
etiology, and
impairment.
for every patient, and the degree of
detail required in the description may
vary. The Glossary of Descriptive Ictal
Terminology (Blume et al, 2001) pro-
vides a standardized terminology to
describe ictal semiologywhat a pa-
tient reports and what observers wit-
ness during an epileptic seizure. The
glossary includes definitions of the
most common clinical manifestations
of seizures and recommendations
for how to describe the location and
evolution of ictal signs. The glossary
borrows heavily from the Cleveland
Clinic semiologic classification scheme
(Loddenkemper et al, 2005).
Use of this standardized terminology
will facilitate communication among
clinicians and researchers. Axis 1 will
be most useful for patients undergoing
evaluation for epilepsy surgery. Here,
ictal semiology, both initial manifesta-
tions and the pattern of evolution, can
give critical anatomical localizing infor-
mation. Ictal semiology may provide
clues for localizing the ictal-onset zone
in cases in which ictal EEG or neuro-
imaging studies are unhelpful. In other
patients, careful descriptions of ictal
phenomenology will facilitate classifica-
tion of seizure type and may help the
clinician choose diagnostic tests or
decide whether activity restrictions are
indicated. For example, automatisms
suggest loss or impairment of con-
sciousness with implications for safety
while swimming or driving. An epigas-
tric aura followed by quiet staring sug-
gests onset in the mesial temporal
lobe. The clinician may then choose
to order specialized MRIs looking for
mesial temporal sclerosis.
To fully describe a seizure, the
examiner should include information
about prodrome, ictal features, both
motor and nonmotor (sensory, dyscog-
nitive, autonomic), the somatotopic
location of the ictal symptoms or signs,
and postictal features. Other possible
descriptors would be seizure frequency
(per unit of time), duration, timing (eg,
sleep versus wake versus on awaken-
ing), patterns of occurrence (eg, clusters
or catamenial pattern), and provocative
factors (ie, factors causing an increase
in seizure frequency in patients with
chronic epilepsy and evoking seizures
in susceptible individuals without epi-
lepsy). A full enumeration of the termi-
nology is beyond the scope of this
chapter, but Table 1-1 and Cases 1-1
and 1-2 illustrate the concept. Exact
features to include and the degree of
detail will depend on the purpose of the
collected data, with greater detail nec-
essary for presurgical evaluations and
research studies. Table 1-2 lists com-
mon ictal manifestations and their
lateralizing or localizing significance,
and the most common ictal manifesta-
tions are briefly described below.
Motor symptoms. Elementary mo-
tor symptoms are simple movements
with one phase, such as clonus or tonic
posturing. Complex motor behaviors
include automatisms, vocalizations, and
complex postures. Automatisms are
coordinated motor movements involv-
ing multiple muscle groups and may
either be spontaneous or reactive to the
surroundings. Motor symptoms can be
either positive or negative, depending
on whether muscle activity is activated
or inhibited. Negative motor manifes-
tations include loss of tone (atonic,
negative myoclonic) and interruption of
activity (aphasia, behavioral arrest).
Sensory symptoms. Elementary
sensory symptoms affect one primary
sensory modality, such as somatosen-
sory or visual phenomena. Sensory
symptoms can also be experiential,
with either complex hallucinations or
illusions, or involvement of memory
and emotion. Dyscognitive symptoms
include changes in perception, atten-
tion, and executive function.
Autonomic symptoms. Autonomic
auras are perceived only by the patient
(eg, palpitations).
16
KEY POINT:
A Descriptions
of ictal
phenomenology
(Axis 1) include
prodrome, ictal
features,
somatotopic
localization of
ictal signs/
symptoms,
postictal features,
seizure frequency,
duration, timing,
severity, and
provoking factors.
"
Autonomic seizures have objective
outward manifestations such as tachy-
cardia or ictal vomiting.
Postictal features. Amnesia and
disorientation commonly follow sei-
zures with loss or impairment of
awareness. Amnesia can be either ret-
rograde or anterograde. The duration
of the postictal confusion is variable. A
Todds phenomenon is postictal uni-
lateral motor, sensory, or language
dysfunction. This focality implies a focal
ictal-onset zone and can aid in deter-
mination of seizure type and laterality
of onset. Severe and prolonged deficits
are more common with focal structural
lesions.
Axis 2: Seizure Type
The currently accepted seizure classifi-
cation is the International Classifica-
tion of Epileptic Seizures (Commission
17
TABLE 1-1
Descriptive Ictal Terminology
Predominant
Ictal Feature Subtype Examples
" Motor
Elementary Tonic (epileptic spasm, postural, versive,
dystonic), myoclonic (negative myoclonic,
clonic, Jacksonian), tonic-clonic, atonic, astatic
Automatism Oroalimentary, mimetic, manual, gestural,
hyperkinetic, hypokinetic, dysphasic,
dyspraxic, gelastic, dacrystic, vocal, verbal
" Nonmotor
Sensory Elementary Somatosensory, visual, auditory, olfactory,
gustatory, epigastric, cephalic, autonomic
Experiential Affective, mnemonic, hallucinatory, illusory
Dyscognitive Perception, attention, executive function
Autonomic Aura Cardiovascular, gastrointestinal, sudomotor,
vasomotor
Seizure Cardiac arrhythmia, ictal vomiting
Somatotopy Laterality Unilateral, hemilateral, generalized,
asymmetric, symmetric
Body part Arm, hand, leg, foot, trunk, neck, eyes
Centricity Axial, proximal, distal
Seizure timing Incidence Regular, irregular, cluster, provocative factor,
reactive, reflex
State Wakefulness, sleep, upon awakening
Duration Self-limited, status epilepticus
Severity Benign, severe
Prodrome
Postictal
phenomenon
Todds phenomenon, lateralizing,
nonlateralizing, impaired cognition,
amnesia, psychosis
on Classification and Terminology of
the International League Against Epi-
lepsy, 1981), although the ILAE Classi-
fication Task Force has recommended
changes to the classification, which
have not yet been formally adopted
(Engel, 2001). One of the main changes
in the proposed classification is recog-
nition of seizure type as a diagnostic
entity. When an epilepsy syndrome
diagnosis cannot be made, seizure type
can be used to provide information
useful for determination of etiology,
choice of treatment, and prognosis
18
Case 1-1
A 14-year-old boy presents with recurrent nocturnal episodes for 1 year. He himself has no
recollection of the episodes. His mother describes that he sits up suddenly, has thrashing
movements of arms and legs, and sometimes jumps out of bed and runs out of the room. The
episodes occur in clusters early in the night or sometimes during daytime naps. He has no epilepsy
risk factors, and his neurologic examination is normal. There is no family history of seizures, but
his father and a paternal uncle have a history of unusual movements during sleep. Figure 1-1
illustrates a sequence of images taken from a typical seizure.
Comment. The ictal semiology of this patients seizures could be described as the following:
Prodrome: none. Ictal: no aura, onset from sleep, sudden hyperkinetic automatisms, symmetric,
involving bilateral proximal limbs, both arms and legs. Duration: 30 seconds. Postictal: rapid
recovery, amnesia for seizure but no postictal confusion. Frequency: clusters of five to six
per night.
Provocative
factors: sleep.
Severity:
exclusively
during sleep,
no injury.
There are
no clear
lateralizing
features to
the seizures,
but the ictal
semiology
suggests
frontal lobe
onset. The
seizure type
would be
complex
partial
seizures,
frontal lobe
onset, or,
in the new
classification,
focal motor
seizures with
contralateral
spread to neocortical areas. A history of sleep-related events in paternal relatives suggests a
diagnosis of autosomal dominant nocturnal frontal lobe epilepsy.
FIGURE 1-1
Sequential images from a video EEG of the patient in Case 1-1, showing typical
hyperkinetic automatisms of frontal lobe epilepsy.
"
19
Case 1-2
A 36-year-old man has had stereotyped
seizures since age 14. He has a history
of meningitis at age 6 months, with
probable generalized tonic-clonic
seizures (GTCS) at the time of this early
illness. He has simple partial, complex
partial, and secondarily GTCS, which
have been refractory to treatment
with AEDs. Figure 1-2 shows patient
behavior approximately 30 seconds after
ictal onset.
The semiology of his complex partial
seizures is described as the following:
Prodrome: none. Ictal: sensory aura,
elementary, epigastric followed by
hypokinetic with oroalimentary
automatisms, followed by partial
head turn to left, followed by bilateral
hand automatisms, followed by
motor, dystonic, left hand with
simultaneous manual automatisms,
FIGURE 1-3
EEG of patient in Case 1-2. Interictal spikes and sharp waves in the left temporal and inferior temporal
chains.
FIGURE 1-2
Video image from Case 1-2 about
30 seconds after ictal onset, showing head
turn and bilateral hand automatisms,
suggestive of temporal lobe involvement.
continued on page 20
(Engel, 2001). This axis also includes
localization within the brain when
known and precipitating stimuli for
reflex seizures. Interictal and ictal EEG
findings are de-emphasized compared
with the current classification.
Tables 1-3 and 1-4 are the accepted
and proposed seizure classification
20
right hand.
Duration: 1 to
2 minutes.
Postictal:
anterograde
amnesia and
dysphasia
(anomia), 5 to
10 minutes.
Frequency:
3 per week.
Provocative
factors: missed
medication
doses. Severity:
history of
burns during
seizures.
Ictal
semiology
suggests onset
from the left
mesial temporal
region.
Figure 1-3 shows interictal EEG, and
Figure 1-4 shows ictal EEG findings.
Figure 1-5 is an image from his MRI.
Comment. The combination of early
risk factor, seizure semiology, and
MRI findings allows classification of this
patient as mesial temporal lobe
epilepsy with hippocampal sclerosis.
Continued from page 19
FIGURE 1-4
Ictal EEG of Patient in Case 1-2, showing rhythmic sharp theta activity over the
left temporal and inferior temporal regions.
FIGURE 1-5
MRI of patient in Case 1-2, showing atrophy
and loss of normal internal architecture of
the left hippocampus (arrow) consistent with
mesial temporal sclerosis.
"
schemes. The primary distinction is
between focal (partial) seizures, which
begin in part of one hemisphere, and
generalized seizures, which show si-
multaneous onset in widespread and
bilateral brain regions. In the proposed
classification, the term focal replaces the
old term partial. Table 1-5 summarizes
the clinical and EEG features of different
seizure types.
21
TABLE 1-2
Lateralizing and Localizing Ictal Features
" Ictal Feature Laterality
Localization
(at Time Ictal
Sign Appears)
Eye deviation Contralateral in frontal
seizures; contralateral
or ipsilateral in
occipital seizures
Frontal or occipital
Early nonforced
head turn
Ipsilateral Temporal
Late forced head
turn
Contralateral Frontal
Focal clonus Contralateral Frontal
Dystonic limb Contralateral Temporal (spread
to basal ganglia)
Tonic limb Contralateral Frontal
Motionless limb Contralateral Temporal or frontal
Asymmetric tonic,
fencing postures
Contralateral Frontal/supplementary
motor
Figure 4 sign Contralateral to
extended limb
Oroalimentary
automatisms
Temporal
Limb automatisms
(unilateral)
Ipsilateral Temporal
Hyperkinetic
automatisms
Frontal
Pedaling, bicycling Frontal
Speech arrest Dominant
hemisphere
Temporal or
frontal
" Postictal Features
Todds paresis Contralateral
Nose wiping Ipsilateral Temporal
Aphasia Dominant
hemisphere
Confusion Temporal greater
than frontal
KEY POINT:
A Seizure type
(Axis 2) is a
diagnostic entity
that can provide
important
information
regarding
etiology, choice
of treatment, and
prognosis.
Focal seizures. In the 1981 ILAE
classification (Commission on Classifi-
cation and Terminology of the Interna-
tional League Against Epilepsy, 1981),
focal seizures are further divided into
simple partial, complex partial, and
secondarily GTCS. During simple par-
tial seizures, consciousness is not im-
paired. The patient is able to interact
normally during the seizure and/or re-
members the entire seizure. During
complex partial seizures, consciousness
is impaired. Impairment of conscious-
ness can be subtle, with only minor con-
fusion or disorientation or inability to
answer questions. In other seizures,
the patient loses awareness entirely
and remembers nothing about what
occurs during the seizure. Some com-
plex partial seizures evolve from simple
partial seizures (auras), while others
have no warning and begin with im-
pairment of consciousness alone. Sec-
ondarily GTCS represent spread of
the ictal discharge from a restricted
focus to the entire brain. Conscious-
ness is impaired and generalized, but
often asymmetric, tonic-clonic move-
ments occur. Postictally, following com-
plex partial and secondarily generalized
seizures, the patient may be confused
or obtunded for a variable period of
time. Focal postictal deficits (Todds
phenomena) can provide important
localizing and lateralizing informa-
tion. The new proposed classification
(Engel, 2006b) does not distinguish
between simple and complex partial
seizures; rather, it divides seizures into
those in which the ictal discharge
remains localized and those in which
it propagates to distant ipsilateral or
contralateral brain regions. The classi-
fication system attempts to separate
seizures beginning in the neocortex
from those starting in limbic regions
(hippocampus, amygdala, or parahip-
pocampus). Spread of the ictal dis-
charge from a restricted brain region is
often indicated by the development of
bilateral symptoms or signs or by auto-
matisms, which usually occur when
consciousness is altered. The distinc-
tion between simple and complex
22
TABLE 1-3
International Classification of
Epileptic Seizures
I. Partial seizures
A. Simple partial seizures (consciousness not impaired)
1. With motor symptoms
2. With somatosensory or special sensory symptoms
3. With autonomic symptoms
4. With psychic symptoms (disturbance of
higher cerebral function)
B. Complex partial seizures (with impairment of
consciousness)
1. Beginning as simple partial seizure and
progressing to impairment of consciousness
2. With impairment of consciousness at onset
C. Partial seizures evolving to secondarily generalized
seizures
1. Simple partial seizures (A) evolving to generalized
seizures
2. Complex partial seizures (B) evolving to
generalized seizures
3. Simple partial seizures (A) evolving to complex
partial seizures (B) evolving to generalized seizures
II. Generalized seizures (bilaterally symmetric and
without local onset)
A. Absence seizures
1. Typical absence seizures
2. Atypical absence seizures
B. Myoclonic seizures
C. Clonic seizures
D. Tonic seizures
E. Tonic-clonic seizures
F. Atonic seizures (astatic)
III. Unclassified epileptic seizures (inadequate or
incomplete data)
Commission on Classification and Terminology of the International League Against
Epilepsy. Proposal for revised clinical and electroencephalographic classification of
epileptic seizures. Epilepsia 1981;22(4):489501. Reprinted with permission from
Wiley-Blackwell Publishing Ltd.
"
partial seizures was eliminated to
address several problems with the old
classification. A large proportion of
partial-onset seizures could not be
accurately classified if consciousness
during a seizure was not adequately
assessed, especially in young children
who cannot report loss of awareness.
The terms simple and complex were
used differently than in other areas of
medicine, causing confusion regarding
simple partial seizures, which can have
quite complicated motor or sensory
manifestations, and complex partial
seizures, which can sometimes show
only straightforward loss of aware-
ness. Finally, loss of awareness in and
of itself did not provide important
23
TABLE 1-4
Proposed
Classification of
Self-limited
Epileptic Seizures
I. Generalized onset
A. Seizures with tonic and/or
clonic manifestations
1. Tonic-clonic seizures
2. Clonic seizures
3. Tonic seizures
B. Absences
1. Typical absences
2. Atypical absences
3. Myoclonic absences
C. Myoclonic seizure types
1. Myoclonic seizures
2. Myoclonic astatic seizures
3. Eyelid myoclonia
D. Epileptic spasms
E. Atonic seizures
F. Negative myoclonus
II. Focal onset (partial)
A. Local
1. Neocortical
a. Without local spread
i. Focal clonic seizures
ii. Focal myoclonic
seizures
iii. Inhibitory motor
seizures
iv. Focal sensory
seizures with
elementary
symptoms
v. Aphasic seizures
b. With local spread
i. Jacksonian march
seizures
ii. Focal (asymmetric)
tonic seizures
iii. Focal sensory
seizures with
experiential
symptoms
2. Hippocampal and
parahippocampal
B. With ipsilateral propagation to:
1. Neocortical areas
(includes hemiclonic
seizures)
2. Limbic areas (includes
gelastic seizures)
C. With contralateral spread to:
1. Neocortical areas
(hyperkinetic seizures)
2. Limbic areas
(dyscognitive seizures
with or without
automatisms
[psychomotor])
D. Secondarily generalized
1. Tonic-clonic seizures
III. Neonatal seizures
Engel J Jr. Report of the ILAE classification core
group. Epilepsia 2006b;47(9):15581568. Re-
printed with permission from Wiley-Blackwell
Publishing Ltd.
TABLE 1-4
Continued
KEY POINTS:
A Focal seizures
begin in a limited
region of one
hemisphere.
A Focal seizures can
be classified by
site of onset
(hippocampal
versus
neocortical) and
extent of spread
(local, regional,
bilateral,
secondarily
generalized).
localizing or lateralizing information,
making this distinction less important
in daily practice. The older terminol-
ogy was quite helpful, however, in con-
cisely communicating that patients have
altered awareness during a seizure, in-
dicating a risk for injury from automatic
or confused behavior.
Simple partial seizures. During sim-
ple partial seizures, consciousness is
not impaired. Simple partial seizures
usually arise from a fairly restricted
anatomical region of the brain, usually
a single lobe or hemisphere. The symp-
toms and signs of the seizure reflect the
function of the brain region(s) involved
in the ictal discharge. The term aura
is often used to describe a simple par-
tial seizure with only subjective ictal
manifestations. An aura may occur in
isolation or precede a seizure with ob-
servable manifestations. When only
subjective symptoms are present, other
diagnostic possibilities (eg, transient
ischemic attacks, migraine, psychiatric
disease) must be excluded. Simple
partial seizures are usually brief in
duration, lasting approximately 15 sec-
onds to 2 minutes. Interictal EEG may
show focal epileptiform discharges,
24
TABLE 1-5
Ictal Clinical and EEG Features
Seizure Type Duration Loss of
Consciousness
Typical Clinical
Features
Postictal
Confusion
Typical Ictal EEG
Simple partial 5 to 30
seconds
No Motor; somatosensory;
special sensory;
psychic; autonomic
No Normal in most; focal
spikes or rhythmic
waveforms
Complex
partial
Variable,
1 to 3
minutes
Yes Often preceded by
simple partial; staring;
loss of awareness;
automatisms
Yes Focal rhythmic
activity spreading to
involve one or both
hemispheres
Secondarily
generalized
tonic-clonic
Variable,
1 to 2
minutes
Yes Simple or complex
partial onset; head
version; asymmetric
tonic posture;
generalized clonus
Yes
(severe)
Focal rhythmic activity,
spreading to involve
both hemispheres
symmetrically
Primary
generalized
tonic-clonic
1 to 2
minutes
Yes Sudden loss of
consciousness; ictal
cry; tonic phase;
clonic phase
Yes
(severe)
Low-voltage rhythmic
activity progressing to
generalized high-
amplitude spikes
Typical
absence
5 to 20
seconds
Yes Staring; eyelid
fluttering
No Generalized 3-Hz
spike-and-wave
Atypical
absence
15 to 45
seconds
Yes Staring, clonus,
myoclonus, atonic
Yes Generalized <2.5-Hz
spike-and-wave
Myoclonic 1 to 2
seconds
No Generalized rapid
jerks of limbs
No Generalized polyspike
and wave
Tonic 5 to 20
seconds
Yes Sustained posture
of limbs
No or
minimal
Low-voltage paroxysmal
fast activity
Atonic 5 to 10
seconds
Maybe Sudden loss of tone No or
minimal
Variable
"
often localized to the lobe of seizure
origin, but can be normal. Because the
ictal discharge involves only a small
area of the brain, ictal scalp EEG is only
abnormal about 25% of the time
(Devinsky et al, 1988).
Elementary motor seizures consist of
simple motor movements, typically a
single type of contraction of a muscle
or group of muscles such as clonus
or tonic contractions (Erkwoh and
Steinmeyer, 1996). The movement is
stereotyped from one seizure to the
next. Elementary motor seizures typi-
cally arise from or propagate to motor
regions of the frontal lobe (pre- or
postcentral gyri or the supplementary
motor area [SMA]). Seizures that arise
from primary motor cortex are charac-
terized by focal clonus, or jerky repet-
itive movements involving the same
muscle group, usually at a frequency
of 2 Hz to 3 Hz. Clonus can remain
localized to a specific body part or
spread to contiguous areas of the
somatotopically organized motor strip,
giving rise to a focal motor seizure with
march, also called a Jacksonian seizure.
The face, hands, and toes are often
involved at the onset of focal clonic
seizures because of the large represen-
tation of these body regions in the
motor homunculus. Less often, sei-
zures from primary motor cortex may
have sensory manifestations if the
seizure onset includes the postcentral
gyrus or the seizure spreads quickly to
the postcentral gyrus. Seizures arising
from or spreading to other parts of the
frontal lobe may give rise to more
complex motor movements. Focal my-
oclonus can arise from primary motor
cortex. The myoclonic jerking can
affect any region of the face, limbs, or
trunk. Nonepileptic myoclonus can be
caused by subcortical, brain stem, or
spinal cord lesions. Focal tonic seizures
are characterized by a sustained muscle
contraction, either flexion or extension,
resulting in a fixed posture of limb(s)
and/or trunk lasting for a few seconds
to minutes. These can arise from nearly
any region of the neocortex, most
often frontal or parietal. Focal motor
seizures with more extensive local
spread, such as those involving the
SMA, may show more complex move-
ments, usually tonic. Such asymmetric
tonic motor seizures are characterized
by complex postures, typically involv-
ing bilateral limbs but asymmetric.
Dystonic seizures show sustained con-
tractions of both agonist and antago-
nist muscles. This results in abnormal
fixed postures or complex movements
such as twisting or athetoid move-
ments. In versive seizures, eyes, head,
and/or trunk are forcibly deviated to
one side. Such seizures frequently arise
from the dorsolateral frontal lobe in
the region of the frontal eye fields.
Focal motor seizures can show nega-
tive or inhibitory symptoms, such as
loss of tone or weakness in a limb
or region of the body. Motor seizures
may also involve language areas with
either positive (vocalization) or nega-
tive (speech arrest) signs. Focal motor
seizures are commonly followed by
Todds paralysis, postictal localized
weakness in the limbs involved in the
seizure, lasting for minutes to hours.
In simple partial sensory seizures,
there are no outward clinical manifes-
tations, and the patients subjective
description of the seizure is most
important for classification and locali-
zation (Erkwoh and Steinmeyer, 1996).
Elementary sensory seizures involve
one primary sensory modality. Somato-
sensory seizures arise from the post-
central gyrus or, less often, from the
secondary sensory area on the upper
bank of the sylvian fissure. Somatosen-
sory seizures are characterized by a
variety of abnormal sensations, usually
positive symptoms, including paresthe-
sias or shocklike sensations, hot or
cold feelings, sensations of movement
or the desire to move, and rarely, pain.
25
KEY POINTS:
A Focal seizures
with local onset
and limited
spread show
elementary motor
signs, elementary
or experiential
sensory
symptoms, or
autonomic
symptoms
or signs.
A Focal seizures
with contralateral
spread involve
complex motor
movements
(automatisms
or bilateral
posturing),
sometimes with
impairment of
consciousness.
Negative symptoms such as numbness
are less common. The sensations typi-
cally involve a single region of the body
contralateral to the involved hemi-
sphere or may march from one body
part to contiguous areas. Rarely, sensa-
tions can be ipsilateral to the seizure
focus or bilateral, especially when
secondary sensory areas are involved.
As in elementary motor seizures, the
sensations often involve the tongue,
hands, or fingers first because of their
large representation in the sensory
homunculus. Somatosensory seizures
often spread to the precentral gyrus,
causing motor symptoms. Visual sei-
zures most often arise from the occip-
ital region, producing simple unformed
visual hallucinations, such as flashing or
flickering colored lights, simple pat-
terns, scotomata, or amaurosis. Again,
positive phenomena are more com-
mon. Rarely, visual seizures may arise
from parietal or posterior temporal
regions, with the first clinical manifes-
tations occurring with spread to the
occipital lobe. Elementary auditory
seizures arise from the lateral temporal
region (Heschls gyrus) and are char-
acterized by simple sounds, such as
buzzing, humming, whooshing, or sin-
gle tones. The seizures are usually
referred to the opposite ear. Hyper-
acusis, hypoacusis, and distortions of
sound can also occur. Vertiginous sei-
zures also often arise from the lateral
temporal region (superior temporal
gyrus), or less often, from the frontal
region. Patients describe sensations of
dizziness, disequilibrium, unsteadiness,
spinning, or room tilting. Olfactory
seizures begin in the uncinate gyrus,
orbitofrontal cortex, or mesial tempo-
ral region. Ictal olfactory hallucinations
are stereotyped, often described as
unpleasant odors, such as those of
garbage, smoke, or rotting food. Gus-
tatory seizures arise from the temporal
lobe, insula, or parietal operculum.
Again, the taste hallucinations are often
unpleasant (eg, blood, metal, bile,
acid). Epigastric sensations (abdominal
discomfort or pain, often rising into the
chest, face, or throat; nausea; churning;
or hunger) can be a subtype of auto-
nomic seizures (see below) or may be
caused by activation of the sensory
cortex for abdominal viscera. Cephalic
auras are vague and nonlocalizing,
often described as head buzzing, light-
headedness, tingling, or headache.
Simple partial psychic seizures, or
experiential seizures, involve more
complex phenomena, such as emotion,
memory, cognition, and perception.
Several experiential sensations may be
involved in a single seizure, combining
memory, perception, and affect. For
example, one patient described his
seizures as beginning with a movie
of a specific baseball game he played at
age 10. He described the sights, smells,
and sounds as if he were back in the
game, up at bat, and recalled his coach
as if he were standing beside me. He
also experienced a sense of euphoria as
his bat hit the ball and he ran for first
base. The entire seizure was extremely
stereotyped, lasting about 90 seconds.
Experiential seizures often arise from
the temporal lobe, either from limbic
structures or from association cortex.
Affective seizures are characterized by
emotional content such as fear or
anxiety, depression, feelings of doom,
euphoria, or anger. These stereotyped
emotions last for seconds to several
minutes and are not appropriate to the
context or environment of the patient.
Mnemonic seizures often involve spe-
cific memories or distortions of memory.
Deja ` vu is a sensation as if a new ex-
perience had been experienced before,
while jamais vu involves a sensation
that a familiar experience is unfamiliar.
Some patients experience forced think-
ing, depersonalizationchanges in real-
ity, the sensation of a presence, flashes
of insight, dreamy states, or distortions
of time. Complex perceptual seizures
26
"
often arise fromvisual or auditory associ-
ation areas. Both illusions (distortions
of external stimuli such as macropsia/
micropsia or hyperacusis) and struc-
tured hallucinations (perceptions with-
out an external stimulus) can occur.
Patients may describe complex visual
illusions, music, spoken words, struc-
tured scenes, or movie clips, often with a
sense of familiarity. Experiential sensory
seizures often arise from the temporal
lobe or temporal association cortices.
When they occur in isolation (ie, without
progression to impairment of conscious-
ness), they may be difficult to distinguish
from psychiatric disorders, except that
ictal hallucinations are typically very ste-
reotyped from one seizure to the next.
Simple partial autonomic seizures
suggest involvement of the autonomic
nervous system and often arise from
the mesial temporal region (amygdala),
insula, or medial prefrontal cortex, with
projections to the hypothalamus and
brain stem. Abdominal sensations can
include epigastric sensations, nausea,
vomiting, abdominal warmth, hunger,
or belching. Epigastric sensations often
have a rising quality into the chest,
neck, or face. Cardiovascular symptoms
include palpitations, cardiac arrhyth-
mias, and chest pain. Apnea and hy-
perventilation may be caused by seizures
arising from the temporal pole, hippo-
campus, or insula. Sexual or erotic auras
often arise from limbic regions, while
genital symptoms without sexual con-
tent arise from the sensory representa-
tion of the genitals in the superior
postcentral gyrus. Other autonomic sei-
zures may be characterized by lacrima-
tion, perspiration, bronchial and oral
secretions, erythema, flushing, cyanosis,
pupillary dilatation or constriction, pilo-
motor erection, and urinary urgency or
incontinence.
Complex partial seizures. Complex
partial seizures account for approxi-
mately 40% of partial seizures and 25%
of all seizures (Loiseau et al, 1991).
Complex partial seizures of temporal
lobe origin are most common, account-
ing for about 70%. Approximately 20%
are frontal lobe seizures, with the
remaining 10% divided between occip-
ital and parietal seizures.
In the 1989 ILAE Classification (Com-
mission on Classification and Terminol-
ogy of the International League Against
Epilepsy, 1989), complex partial sei-
zures are defined as stereotyped partial
seizures with impaired consciousness.
During the seizure, patients are either
unable to respond to external stimuli
or after the seizure are amnestic to
events that occurred during the sei-
zure. Impaired consciousness may be
difficult to determine unless specialized
testing is performed during the seizure,
particularly in infants and young chil-
dren. Typically, alteration of conscious-
ness occurs when an ictal discharge
involves bilateral mesial temporal struc-
tures, but alteration of consciousness
can also rarely occur with bilateral
cortical involvement outside the hip-
pocampus. Complex partial seizures do
not need to begin in the mesial
temporal region; neocortical seizures
can spread to mesial temporal struc-
tures to cause typical temporal lobe
seizure semiology.
Many complex partial seizures are
preceded by a simple partial seizure, or
aura, which may be helpful in localizing
the ictal-onset zone. Others begin with
impairment of consciousness without
warning. The clinical manifestations of
complex partial seizures are variable,
including motionless staring, behav-
ioral arrest, unresponsiveness, oral or
limb automatisms, focal limb posturing
or clonus, and postictal confusion or
focal neurologic deficits (Theodore
et al, 1983). Most complex partial seiz-
ures last from 30 seconds to less than
2 to 3 minutes. Clinical features are de-
scribed in more detail below, under the
headings for seizures arising from dif-
ferent brain regions.
27
Automatisms are somewhat coordi-
nated automatic repetitive move-
ments that occur when consciousness
is impaired. They may represent re-
lease phenomena from clouding of
consciousness during a seizure or in
the postictal period. In some cases,
they are an abnormal continuation of
the patients activities before the sei-
zure. Automatisms may be either spon-
taneous or in response to external
stimuli. Complex or semipurposeful
movements are common and may re-
semble voluntary movements. In many
patients, automatisms are stereotyped
from one seizure to the next. In tem-
poral lobe seizures, oroalimentary and
distal limb automatisms are most com-
mon. Frontal lobe seizures often show
hyperkinetic or hypermotor autom-
atisms with bilateral, predominantly
axial, limb movements and vocaliza-
tions. Table 1-6 describes common
automatisms.
Postictally, patients may be con-
fused, disoriented, or have focal neu-
rologic signs such as weakness, aphasia,
hemineglect, or visual deficits. These
may provide lateralizing and localizing
information. Fatigue and headache are
common. Postictal abnormalities can
last for minutes to, occasionally, hours.
Secondarily generalized tonic-clonic
seizures. Secondarily GTCS represent
spread from a limited focus to the
entire brain. They may begin as simple
partial seizures with subsequent gener-
alization or progress through a se-
quence from simple partial to com-
plex partial to generalized seizures
(Theodore et al, 1994). In some cases,
particularly in frontal and parietal lobe
seizures, the spread of the ictal dis-
charge is rapid, and localizing or later-
alizing features may not be present. In
contrast to the bilaterally symmetric
movements of primary generalized sei-
zures, tonic and/or clonic movements
28
TABLE 1-6
Automatisms
Automatism Examples
Oroalimentary Lip smacking, tongue movements,
chewing, swallowing, teeth grinding
Mimetic Acting out the patients emotional state
Manual or
pedal
Fumbling, tapping, grasping movements
Gestural Movement, often in response to external
stimuli
Ambulatory Wandering, walking, running
Vocal Single or repetitive utterances, not words
Verbal Single or repetitive utterances, words,
or sentences
Hypokinetic Behavioral arrest, motionless limbs
Hyperkinetic Large-amplitude movements of proximal
limbs or trunk, such as pedaling, thrashing,
rocking, or pelvic thrusting
Gelastic Bursts of laughter without mirth
Dacrystic Bursts of crying without sadness
KEY POINT:
A Secondarily
generalized tonic-
clonic seizures are
focal seizures that
spread to other
brain regions.
"
of secondarily generalized seizures are
usually asymmetric, first affecting the
side of the body contralateral to the
hemisphere of seizure origin. Forced
head deviation may occur just prior to
secondary generalization, again contra-
lateral to seizure onset (Niaz et al,
1999). The motor features follow a
progression similar to those of primary
generalized seizures, as described be-
low, with an ictal cry, generalized tonic
stiffening, and then clonic contractions
of all muscles. Postictally, the patient
is lethargic or obtunded, with deep
respirations, and typically falls asleep.
Headache and diffuse muscle aches are
common.
Focal seizures by region of onset.
Temporal lobe seizures. Temporal lobe
seizures account for 70% of partial-
onset seizures. They can be divided
into two main groups: those with onset
from mesial temporal structures, and
those from lateral temporal neocortex.
Making the distinction between mesial
and lateral seizures often requires in-
tracranial EEG recording. General fea-
tures are listed in Table 1-7.
Mesial temporal seizures typically
begin in childhood or adolescence,
although they can begin at any age.
There is often a history of a preceding
neurologic insult early in life, such as
febrile seizures, meningitis, or head
injury, or a family history of seizures.
Mesial temporal seizures arise from
limbic structures, including hippocam-
pus, amygdala, or parahippocampal re-
gion. Seizures typically begin as simple
partial seizures characterized by a ris-
ing epigastric sensation or other auto-
nomic sensation, psychic symptoms
such as deja ` vu, or olfactory symp-
toms (Gil-Nagel and Risinger, 1997). As
the seizure spreads to involve bilateral
mesial temporal regions, behavioral
arrest, quiet staring with widened
palpebral fissures, unresponsiveness,
and oroalimentary automatisms occur.
Longer seizures commonly progress
to limb automatisms, either ipsilateral
to the ictal focus or bilateral. Motor
symptoms of the contralateral hand,
arm, or leg occur with spread outside
the temporal lobe: dystonic posturing
from involvement of the basal ganglia
or clonus from primary motor cortex.
Seizures usually last from 1 to 3 min-
utes. Postictally, the patient is often
confused and amnestic for the sei-
zure. Postictal localizing or lateralizing
signs include aphasia, hemiparesis, or
nose wiping (with hand ipsilateral to
ictal onset). Secondary generalization
occurs in some patients, particularly
in seizures beginning during sleep.
Interictal EEG can be normal, show
focal slowing over the temporal region,
or show focal spike-and-wave or sharp-
and-slow-wave discharges over anterior-
to-midtemporal regions. Focal slowing
and epileptiform discharges can be
unilateral or bilateral. Ictal EEG is va-
riable, but typically shows focal tem-
poral or regional rhythmic waveforms,
characteristically 5-Hz to 7-Hz rhythmic
sharp theta activity over the anterior
and inferior temporal regions within
30 seconds of clinical seizure onset
(Maillard et al, 2004). Neuroimaging
may show mesial temporal sclerosis
(see mesial temporal epilepsy with hip-
pocampal sclerosis discussion below) or
mesial temporal focal lesions such as low-
grade tumors or vascular malformations.
Lateral temporal lobe seizures arise
from temporal neocortex. Seizures may
begin at any age. The clinical manifes-
tations are heterogeneous and may be
nearly indistinguishable from those of
mesial temporal seizures (Gil-Nagel
and Risinger, 1997). When auras occur,
they are commonly auditory hallu-
cinations, complex visual illusions or
hallucinations, or language deficits.
Temporal neocortical seizures usually
propagate to the mesial temporal re-
gion, giving rise to complex partial
seizures with late oroalimentary autom-
atisms and other symptoms consistent
29
KEY POINTS:
A Temporal lobe
seizures can arise
from mesial
temporal (limbic)
or neocortical
regions.
A Temporal lobe
seizures are
characterized
by epigastric,
experiential, or
autonomic auras,
quiet staring and
unresponsiveness,
oroalimentary
automatisms, and
contralateral
motor signs.
30
TABLE 1-7
Features of Focal Seizures by Region of Onset
" Temporal Lobe Seizures
Mesial Temporal
Aura (often epigastric, psychic, affective, olfactory)
Impaired consciousness
Fixed stare, widened palpebral fissures
Early oroalimentary automatisms
Limb automatisms (ipsilateral to seizure focus)
Dystonic posturing or clonus (contralateral to seizure focus)
Postictal confusion and amnesia
Lateral Temporal
Aura (often auditory hallucinations, complex perceptual or experiential
hallucinations, language dysfunction)
Late oroalimentary automatisms
Late manifestations may be indistinguishable from mesial temporal
" Frontal Lobe Seizures
Brief seizures, often in clusters
Little or no postictal confusion
Rapid secondary generalization
Prominent motor manifestations (clonic, tonic, postural)
Hyperkinetic complex or bizarre automatisms
Sexual automatisms
Frequent falls
Nocturnal predominance
Status epilepticus
" Occipital Lobe Seizures
Elementary visual hallucinations
Loss of vision
Sensation of eye movement
Contralateral or ipsilateral deviation of the eyes
Forced blinking
Variable spread to:
Parietal lobe (sensory symptoms)
Temporo-occipital regions (formed visual hallucinations)
Mesial temporal region
continued on next page
"
with mesial temporal involvement.
Secondary generalized seizures occur
more commonly than in mesial tem-
poral seizures. The ictal EEG shows
more variable patterns than in mesial
temporal seizures and may show focal
or regional rhythmic activity faster than
8 Hz at onset, with later ictal patterns
similar to those of mesial temporal
seizures (Maillard et al, 2004). Neuro-
imaging may show focal neocortical
lesions such as tumors, vascular mal-
formations, or cortical dysplasia. When
imaging is normal, intracranial EEG
with subdural strip or grid and depth
electrodes is often needed to reliably
distinguish mesial from lateral tempo-
ral seizures.
Frontal lobe seizures. Frontal lobe
seizures account for approximately
15% to 20% of focal seizures, although
this estimate comes largely from pop-
ulations of patients who are candidates
for epilepsy surgery. Frontal lobe
seizures can be simple partial, complex
partial, or secondarily generalized, with
a wide variety of clinical features.
General features (Salanova et al, 1995)
are listed in Table 1-7. They are
typically shorter than seizures arising
from the temporal lobe, have less se-
vere impairment of consciousness dur-
ing the seizure, and show little or no
postictal confusion. Motor symptoms
are common, sometimes bizarre, com-
plex, bilateral motor movements easily
confused with nonepileptic psycho-
genic events.
Interictal EEG may be normal in 30%
of patients, particularly if the epileptic
focus is in basal or interhemispheric
frontal regions. When epileptiform dis-
charges are present, they may be bi-
lateral (secondary bilateral synchrony)
or show misleading lateralization.
Ictal scalp EEG may be unhelpful in
localization and lateralization because
seizures may arise from small and/or
deep areas of cortex not detectable by
scalp electrodes, or because ictal EEG
is obscured by movement and muscle
artifact during motor seizures. When
ictal patterns are seen, they may show
unilateral or bilateral low-amplitude fast
activity, repetitive spikes, or spikes fol-
lowed by diffuse attenuation. Ictal pat-
terns are nonlocalizing inmore thanhalf
of patients. MRI of the brain may show
focal lesions but is often normal.
Seizures arising from different areas
of the frontal lobe show varied clinical
manifestations, although rapid spread
within the frontal lobe often makes it
difficult to precisely localize seizure
onset by clinical semiology (Shulman,
2000). The common clinical features of
seizures arising from various frontal
lobe regions are described below.
31
KEY POINT:
A Frontal lobe
seizures are
clinically variable,
characterized by
brief duration,
little or no
impairment of
consciousness,
hyperkinetic
automatisms, and
rapid secondary
generalization.
TABLE 1-7
Continued
" Parietal Lobe Seizures
Somatosensory auras
Receptive language disturbance (dominant hemisphere)
Neglect (nondominant hemisphere)
Variable spread to:
Occipital lobe (visual hallucinations)
Mesial temporal region
Precentral regions (motor)
Motor cortex seizures, arising from
the motor strip, are typically clonic
seizures, either with or without march,
involving contralateral face or limbs.
The face or distal limbs are usually
involved first. Postictal Todds paralysis
is common. In the lower prerolandic
area, expressive speech may be affected,
causing speech arrest, vocalizations,
or dysphasia. Supplementary motor
area seizures are brief and show
explosive onset with sustained asym-
metric tonic posturing of the limbs.
Patients often assume complex limb
positions (eg, fencer posture with
abduction of contralateral arm, flexion
of ipsilateral arm, and contraversive
head turn), and have facial grimacing,
vocalization, and contralateral version
of the head and eyes. Consciousness
may be preserved, and there is little or
no postictal confusion. SMA seizures
are often nocturnal and may occur
in clusters. Dorsolateral or anterior
frontopolar cortex seizures involve con-
tralateral or bilateral tonic or clonic
movements, contralateral head and eye
version, and speech arrest. Forced
thinking may occur as an aura for dor-
solateral seizures. Some patients de-
scribe simple visual hallucinations.
Spread is variable, and no pathogno-
monic features are present. Orbitofron-
tal seizures typically spread quickly to
limbic structures, giving rise to seizures
with semiology similar to mesial tem-
poral seizures. Common initial symp-
toms include autonomic signs, olfactory
hallucinations, psychic auras, and oroa-
limentary or gestural automatisms. Cin-
gulate seizures, arising fromthe anterior
cingulate region, are characterized by
complex motor automatisms, auto-
nomic symptoms, and changes in
mood or affect. In some cingulate
seizures, the sole manifestation is
behavioral arrest, mimicking absence
seizures. In others, florid emotional
outbursts and aggressive behavior oc-
cur. Motor symptoms include con-
tralateral or bilateral tonic or clonic
movements. Insular seizures, arising
from the insula or opercular area, have
prominent oral symptoms, including
chewing, salivation, repetitive swal-
lowing, speech arrest, and laryngeal
symptoms such as choking or stridor.
Autonomic, epigastric, gustatory, or
emotional auras can occur.
Parietal lobe seizures. Parietal lobe
seizures account for fewer than 10% of
focal seizures. Like frontal lobe sei-
zures, parietal lobe seizures have quite
varied clinical manifestations. Seizures
remaining restricted to the parietal
lobe are usually simple partial seizures,
but complex partial and secondarily
generalized seizures occur, with spread
outside the parietal region. Many
parietal seizures begin with a somato-
sensory aura, such as tingling, numb-
ness, electric shocklike sensations, a
sensation of movement, or pain. These
can occur unilaterally in contralateral
face, hands, or arm more commonly
than leg, or rarely bilaterally (Kim et al,
2004). Gustatory hallucinations arise
from the parietal operculum. Spatial
neglect or asomatognosia can occur
with nondominant hemisphere in-
volvement, while receptive language
disturbances suggest dominant hemi-
sphere involvement. Although auras
are common, many areas of the parietal
lobe are clinically silent, and the initial
manifestations of parietal seizures may
be seen only when the discharge
spreads to other brain regions. When
seizures spread posteriorly, compli-
cated visual hallucinations may occur.
Anterior spread into the motor regions
mimics primary motor involvement or
supplementary motor area posturing.
Other seizures spread into the tempo-
ral region, producing mesial temporal
lobe semiology.
Interictal EEG is often normal or
nonspecific. Epileptiform discharges are
rarely seen and may be poorly localiz-
ing. The EEG during seizures is poorly
32
KEY POINT:
A Parietal lobe
seizures are
characterized by
somatosensory
auras, with
variable spread to
occipital, mesial
temporal, or
frontal regions.
"
localizing (Kim et al, 2004); the first ictal
discharges often represent propaga-
tion outside the parietal lobe to other
cortical regions. Structural imaging stud-
ies may reveal focal parietal lesions.
Occipital lobe seizures. Fewer than
10% of partial seizures arise from the
occipital lobe. In most cases, occipital
seizures begin with elementary visual
hallucinations, usually simple shapes
and colors (Taylor et al, 2003). Halluci-
nations typically begin in the visual field
contralateral to the affected cortex and
then spread to the entire visual field.
Other common symptoms include loss
of vision, a sensation of movement of
the eyes, contralateral or ipsilateral
deviation of the eyes, nystagmoid eye
jerks, forced blinking or eyelid flutter,
headache, and ictal vomiting. Postictal
visual field deficits or postictal blind-
ness may occur. Seizures from the
temporo-occipital association cortex can
cause complex illusions (change in size
or shape, distortions) andformedscenes
or movies. Occipital seizures show
variable spread to sensorimotor cortex,
temporo-occipital regions, or mesial tem-
poral regions, with semiology consistent
with seizures involving these regions.
Secondary generalization can occur.
The EEG commonly shows occipital
spikes, but may be normal (Taylor et al,
2003). Spikes from the mesial occipital
region may be mislateralizing. EEG
during seizures rarely shows focal
rhythmic activity over one or both
occipital regions. Other seizures show
no ictal change or patterns consistent
with spread to other brain regions.
Generalized seizures. General-
ized seizures begin simultaneously in
widespread brain regions in both
hemispheres. Consciousness is usually
impaired. Motor manifestations are bi-
lateral and synchronous.
Typical absence seizures. Typical
absence seizures (formerly called petit
mal seizures) are characterized by
sudden behavioral arrest, loss of aware-
ness, and blank staring, sometimes
with slight upward eye deviation and
repetitive blinking (Holmes et al,
1987). They are usually brief, lasting 5
to 30 seconds. Absence seizures begin
and end abruptly, with immediate
return to normal consciousness post-
ictally. Other clinical features may be
present but are less prominent than
the behavioral arrest. These include
bilateral clonus of the eyelids, corner of
the mouth, or upper limbs; loss of tone
in the neck or trunk; mild tonic
contractions of the neck and/or limbs;
autonomic changes such as pallor,
tachycardia, or salivation; or automa-
tisms involving oropharyngeal muscles
or limbs (Penry et al, 1975). Typical
absence seizures in childhood absence
epilepsy (pyknolepsy) last 5 to 15 sec-
onds and may occur hundreds of times
per day. In juvenile absence epilepsy,
absence seizures tend to be slightly
longer (15 to 30 seconds) and occur
less frequently, once a week to once a
month (Martinez-Juarez et al, 2006).
The longer-duration and low-frequency
absence seizures may be confused with
complex partial seizures, especially if
automatisms are present. Absence of
postictal confusion may aid in diag-
nosis. Absence seizures also occur in
juvenile myoclonic epilepsy and myo-
clonic absence epilepsy.
The hallmark EEG finding of typical
absence seizures is generalized, usually
frontally maximal, 3-Hz spike-and-slow-
wave complexes. The spike-wave fre-
quency at seizure onset averages 3.5 Hz
to 4.5 Hz, but gradually slows to an
average of 2.5 Hz to 3 Hz by the end of
the seizure. In juvenile myoclonic
epilepsy, absence seizures show gen-
eralized, more irregular, 2.5-Hz to
4.0-Hz spike-wave or polyspike-wave
discharges. Absence seizures may be
activated by hyperventilation in 50%
to 80% of untreated patients.
Atypical absence seizures. Atypical
absence seizures occur in symptomatic
33
A Generalized
seizures begin
simultaneously in
widespread brain
regions in both
hemispheres.
A Typical absence
seizures show
abrupt onset
and offset of loss
of awareness,
lasting 5 to
30 seconds,
without postictal
confusion.
KEY POINTS:
A Occipital lobe
seizures are
characterized by
elementary visual
hallucinations
with variable
spread to parietal,
frontal, or mesial
temporal regions.
generalized epilepsies. Onset and offset
are less abrupt than in typical absence
seizures. Seizures last 10 to 45 seconds
(Holmes et al, 1987). Impairment of
consciousness is often incomplete, and
patients may continue purposeful ac-
tivity. Associated clinical features are
more frequent and more pronounced
than in typical absence seizures. Atonia
is most common, rarely causing falls.
Other motor findings include myoclonic
jerks of the face or mouth, tonic
stiffening, and automatisms. Seizure
offset is gradual, and patients may
remain confused postictally. The EEG
during atypical absence seizures shows
generalized irregular spike-and-slow-
wave complexes at repetition rates
slower than 3 Hz.
Myoclonic absence seizures. Myo-
clonic absence seizures show rhythmic
clonic jerking at frequencies of 2 Hz to
4 Hz, coinciding with generalized
spike-and-wave or polyspike-and-wave
discharges (Engel, 2006b). They typi-
cally last 5 to 10 seconds. In contrast to
myoclonic seizures, which have no
impairment of consciousness, myo-
clonic absence seizures are character-
ized by brief lapses of awareness.
Generalized tonic-clonic seizures.
Formerly called grand mal seizures,
GTCS show a stereotyped sequence
of movements that are bilaterally
symmetric (Duron et al, 2005). Patients
may describe a vague prodrome
of irritability, headache, and mood
changes for hours to days before a
seizure, but no specific aura is present.
In some cases, GTCS evolve from myo-
clonic or absence seizures, but in other
cases they begin abruptly. The patient
loses consciousness and has tonic
stiffening of axial and limb muscles,
typically causing a fall. Eyes deviate
upward and pupils dilate. During this
phase, a loud ictal cry may be heard as
respiratory muscles contract against a
closed larynx. The patient may bite the
tongue as the jaw clenches. Autonomic
symptoms such as tachycardia, hyper-
tension, and profuse salivation occur.
The tonic phase lasts 10 to 15 seconds.
Respiration is depressed and the pa-
tient may become cyanotic. Follow-
ing the tonic phase, the clonic phase
begins with a low-amplitude generalized
tremor that gradually slows in frequency
and increases in amplitude to become
generalized clonic jerking. Upper ex-
tremities showflexor contractions, while
lower extremities are extended. Repeti-
tive vocalizations or grunting may ac-
company the clonic jerks. GTCS last 1
to 2 minutes. At the end of the sei-
zure, urinary incontinence may occur as
sphincter muscles relax. During the pos-
tictal phase, the patient is stuporous or
obtunded, with deep sonorous respira-
tions. Consciousness is gradually recov-
ered over minutes, but the patient may
remain confused or somnolent for up to
hours. Postictal headache and diffuse
myalgias are common.
Clonic-tonic-clonic seizures are a
variation of GTCS evolving from gen-
eralized myoclonus. The myoclonus
gradually increases in frequency and
amplitude and then evolves into the
generalized tonic-clonic pattern de-
scribed above (Andermann and Berkovic,
2001). Clonic-tonic-clonic seizures occur
in primary generalized epilepsies such as
juvenile myoclonic epilepsy. Other GTCS
evolvefromgeneralizedabsenceseizures,
with initial EEG showing 3-Hz spike-and-
slow-wave activity.
Ictal EEG shows initial generalized
low-voltage rhythmic fast activity (epi-
leptic recruiting rhythm) in the beta
frequency range. Rhythmic activity
gradually decreases in frequency to
about 10 Hz and increases in amplitude
during the tonic phase. Much of the
seizure can be partially or completely
obscured by muscle artifact. During the
clonic phase, the EEG shows bursts of
generalized spike-wave activity alternat-
ing with severe diffuse background
attenuation. The periods of attenuation
34
A Generalized
tonic-clonic
seizures begin
with tonic
stiffening and loss
of awareness,
progress to
generalized clonic
jerking, and are
followed by
postictal
obtundation.
KEY POINTS:
A Atypical absence
seizures show
less abrupt
changes in
consciousness
and may show
postictal
confusion.
"
between clonic jerks increase in dura-
tion as the clonic phase progresses
(Andermann and Berkovic, 2001). Post-
ictally, the EEG shows diffuse slowing
and attenuation of amplitude.
Clonic seizures. Clonic seizures
show a similar sequence of movements
as GTCS, but no tonic phase occurs.
Clonic jerking occurs at a frequency
of 1 Hz to 4 Hz. The jerks can be
symmetric or asymmetric. Conscious-
ness is impaired in most seizures. Most
clonic seizures last less than 2 minutes,
and the postictal phase is usually
shorter and less severe than after GTCS
(Engel, 2006b). Ictal EEG shows high-
voltage generalized polyspike-and-wave
activity at 2 Hz to 4 Hz, or generalized
rhythmic fast activity.
Myoclonic seizures. Myoclonic sei-
zures or myoclonic jerks are sudden,
brief, lightninglike (less than 100/ms)
contractions of muscles (Duron et al,
2005). Generalized myoclonic seizures
involve both upper and lower extrem-
ities and the face bilaterally and rela-
tively symmetrically. The arms are
more commonly involved, frequently
resulting in dropping objects from
the hands. When legs are involved,
myoclonic jerks may cause falls. Myo-
clonic jerks occur singly or in clusters
of irregularly repeating jerks. During
brief myoclonic jerks, consciousness
is not impaired and no postictal con-
fusion is present, but alteration of
consciousness may occur during pro-
longed clusters of myoclonus. Myo-
clonic jerks occur predominantly within
1 to 2 hours after awakening. The
EEG correlate to myoclonic jerks is a
high-voltage generalized polyspike or
polyspike-and-wave discharge followed
by a high-amplitude slow wave. The
epileptiform discharges sometimes re-
peat in irregular trains at 3 Hz to 5 Hz
(Leppik, 2003). These usually show
maximal voltage over the frontal and
central regions. Myoclonic jerks are the
hallmark of juvenile myoclonic epilepsy
but can also occur in other types of
primary and symptomatic generalized
epilepsy.
Tonic seizures. Tonic seizures are
characterized by sustained contractions
of muscles, most prominent in axial
muscles, but usually involving the limbs
and face as well (Committee on Clas-
sification and Terminology of the
International League Against Epilepsy,
1981). Limbs may be fixed in either
flexor or extensor postures. Postures
can be symmetric or asymmetric, mak-
ing generalized tonic seizures difficult
to distinguish from focal asymmetric
tonic seizures. Upward deviation of the
eyes and extension of the neck may
occur. Lateral head or eye movements
can also occur. Consciousness is im-
paired during the seizure, but postictal
confusion is brief. If the patient is
standing, tonic seizures frequently cause
falls. Tonic seizures often occur in clus-
ters, usually during sleep. The EEG
during tonic seizure shows generalized
low-voltage paroxysmal fast activity at
frequencies greater than 10 Hz, usually
with diffuse attenuation of EEG voltage.
Tonic seizures are a characteristic seizure
type of the Lennox-Gastaut syndrome.
Atonic seizures. Atonic seizures are
characterized by a brief diffuse loss of
tone in postural muscles lasting more
than 1 to 2 seconds (Committee on
Classification and Terminology of the
1981). Mild seizures may cause a head
drop or loss of tone in the limbs,
causing the patient to drop objects.
More severe atonic seizures, termed
drop attacks, result in loss of all tone
and a sudden collapse or fall to the
ground. These frequently result in in-
juries such as lacerations, fractures, and
dental injuries. Atonic seizures last less
than 5 seconds and have little or no
postictal confusion. Atonic seizures are
characteristic of symptomatic general-
ized epilepsies such as the Lennox-
Gastaut syndrome. The EEG of atonic
35
A Tonic seizures are
characterized by
sustained
contractions of
axial and limb
muscles lasting 5
to 20 seconds.
A Atonic seizures
show sudden loss
of tone and
frequently cause
falls.
KEY POINTS:
A Myoclonic
seizures are brief
generalized jerks
of arms and/or
legs, often
occurring in
clusters upon
awakening.
seizures is very variable, including pat-
terns of diffuse attenuation, high-
voltage generalized polyspike-and-wave
discharges, high-voltage slow waves, or
low-voltage paroxysmal fast activity.
Negative myoclonus is a sudden,
very brief loss of tone lasting less than
500/ms. Negativemyoclonus may begen-
eralized or involve single muscles or
groups of muscles. The EEG shows gen-
eralized spike-and-wave or polyspike-
and-wave discharges.
Epileptic spasms. Epileptic spasms
(previously called infantile spasms) are
sudden flexion, extension, or mixed
flexion-extension movements of the
trunk and proximal muscles (Blume
et al, 2001). These are more sustained
than myoclonic jerks but shorter than
tonic seizures, lasting 1 to 2 seconds.
Facial grimacing and upward eye devi-
ation may occur with the spasms.
Spasms often occur in clusters, recur-
ring every 5 to 10 seconds over
minutes to hours. Epileptic spasms
are most characteristic of the West
syndrome.
Unclassified epileptic seizures. This
category is reserved for seizures in
which information is inadequate to al-
low classification (eg, nocturnal GTCS,
which could be either primary or
secondarily generalized), or when in-
formation is conflicting (some asym-
metric tonic seizures)
Axis 3: Epilepsy Syndrome
The epilepsy syndrome is chosen from
the International Classification of Epi-
leptic Syndromes (Committee on Clas-
sification and Terminology of the
1989). Not every patient can be given a
syndrome diagnosis, particularly early
after the diagnosis of epilepsy or when
full diagnostic testing has not been
performed. When a specific epilepsy
syndrome diagnosis cannot be made,
the seizure type classification can
provide important diagnostic, patho-
physiologic, and prognostic informa-
tion (Engel, 2001). To make an
epilepsy syndrome diagnosis, the ex-
aminer must gather information about
a variety of factors, including the
following: seizure type(s), both those
that are essential for diagnosis and
those that would preclude the diagno-
sis of a specific syndrome, the age of
onset, progressive nature of the syn-
drome, presence of interictal neuro-
logic and neuropsychologic deficits,
etiology (including genetic factors),
EEG features, and neuroimaging find-
ings (Engel, 2001). Patients with the
same epilepsy syndrome will show
similar, but not identical, pathophysi-
ology, response to treatment, and
treatment.
The currently accepted classification
(Committee on Classification and Ter-
minology of the International League
Against Epilepsy, 1989) divides epilepsy
syndromes into localization related,
generalized, undetermined, and
special syndromes. Generalized epi-
lepsy syndromes are those in which
generalized seizures occur, while pa-
tients with focal syndromes have focal
or multifocal seizures. Undetermined
syndromes include those in which both
focal and generalized seizures occur
and those in which no unequivocal
signs are present to indicate focal or
generalized seizure onset (eg, noctur-
nal GTCS with normal EEG). Special
syndromes describe seizures that do
not require a diagnosis of epilepsy,
including acute symptomatic seizures,
febrile seizures, and isolated seizures.
The syndromes are then further classi-
fied by etiology. Idiopathic syndromes
have a presumed genetic etiology and
are characterized only by epilepsy,
without structural brain lesions or
other neurologic deficits. Symptomatic
epilepsies have a clearly identified
cause. Cryptogenic epilepsies are those
in which an etiology is suspected but
the exact cause cannot be identified.
36
KEY POINT:
A Epilepsy
syndrome
classification
(Axis 3) includes
information
about seizure
types, age of
onset, interictal
neurologic and
neuropsychologic
deficits, etiology,
EEG features, and
neuroimaging
findings.
"
Several changes have been pro-
posed for epilepsy syndrome classifica-
tion (Engel, 2006a). Syndromes are no
longer grouped according to whether
they are focal or generalized and by
etiology. The authors propose that
various syndrome groupings may need
to be developed for specific purposes,
such as teaching, research, and clinical
care. One suggested grouping, by age,
is outlined in the 2006 proposal (Engel,
2006b). Unclassified syndromes are
not included; when a specific syn-
drome diagnosis cannot be made,
the seizure type is used for classifica-
tion. Therefore, the symptomatic focal
epilepsies become symptomatic focal
epilepsies not otherwise specified,
with the seizure type as described
above as the main classification. In the
proposed classification, the term cryp-
togenic is replaced by probably symp-
tomatic. In addition, the proposed
classification moves etiology into axis
4. The new classification aims to es-
tablish epilepsy syndromes as testable
workinghypotheses. As additional knowl-
edge is acquired, syndromes will be re-
fined, revised, or discarded as necessary.
Epilepsy syndromes recognized by
the ILAE are listed in Table 1-8. Newly
recognized syndromes are added in
italics. Only syndromes that begin in or
commonly persist into adolescence or
adulthood will be described below;
childhood syndromes are discussed in
a separate chapter.
Familial temporal lobe epilep-
sies. Autosomal dominant partial epi-
lepsy with auditory features is a familial
syndrome in which seizures arise from
the lateral temporal lobe (Ottman et al,
2004). Many of the seizures begin with
auditory auras such as buzzing, hum-
ming, or more complex auditory hallu-
cinations. Mutations in the leucine-
rich glioma inactivated (LGI1) gene
are found in about half the families,
but how these result in epilepsy is
unknown.
Mesial temporal lobe epilepsy
with hippocampal sclerosis. Mesial
temporal lobe epilepsy with hippocam-
pal sclerosis is characterized by seizures
arising from mesial temporal structures
(hippocampus, amygdala, per hippo-
campal gyrus) (Wieser, 2004). Seizures
typically begin in childhood or adoles-
cence. A history of febrile seizures or
other early insult (eg, head injury,
meningitis) is common. Seizure semi-
ology is as described in mesial tempo-
ral seizures above. Seizures typically
begin as simple partial seizures
epigastric sensations, fear or anxiety,
autonomic sensations, or deja ` vu
(Janszky et al, 2004). As seizures prog-
ress, behavioral arrest, loss or alter-
ation of consciousness, and oroalimentary
automatisms occur. With longer seizures,
ipsilateral limb automatisms and contra-
lateral dystonic posturing or clonic jerking
can occur. Secondary generalization may
occur, more frequently during seizures
arising from sleep. As seizures generalize,
the head is forced to the contralateral side
and the patient assumes a figure 4
posture, with the contralateral arm ex-
tended and the ipsilateral arm flexed.
Postictally, the patient is confused and
often amnestic for the seizure, and may
show focal weakness or aphasia. The
interictal EEG shows focal slowing and
epileptiform discharges in anterior and
inferior temporal electrodes, either uni-
lateral or bilateral. Spikes are activated by
non-REM sleep. Ictal EEG may show
hemispheric attenuation or irregular theta
or delta activity at onset and then
characteristically evolves to 5-Hz to 7-Hz
rhythmic sharp theta activity over one or
both temporal regions. The hallmark
pathology of hippocampal sclerosis is
marked neuronal loss in CA1, CA3, CA4,
and the dentate granule cells. These
abnormalities may be detected by neu-
roimaging procedures. Typical MRI ab-
normalities include atrophy of the
hippocampus, loss of internal hippocam-
pal architecture, and increase in T2 signal
37
KEY POINT:
A Proposed epilepsy
syndromes are
testable working
hypotheses and
can be modified
as new
information is
obtained.
38
TABLE 1-8
International Classification of Epilepsies, Epileptic
Syndromes, and Related Seizure Disorders
a
I. Localization-related (focal, local, partial)
A. Idiopathic
1. Benign childhood epilepsy with centrotemporal spikes
2. Childhood epilepsy with occipital paroxysms
3. Primary reading epilepsy
B. Cryptogenic (defined by seizure type, clinical features, anatomical
localization)
C. Symptomatic
1. Mesial temporal lobe epilepsy with hippocampal sclerosis
2. Autosomal dominant temporal lobe epilepsy with auditory features
3. Autosomal dominant nocturnal frontal lobe epilepsy
4. Symptomatic focal epilepsies not otherwise specified
a. Temporal lobe epilepsies
b. Frontal lobe epilepsies
c. Parietal lobe epilepsies
d. Occipital lobe epilepsies
5. Chronic progressive epilepsia partialis continua of childhood
II. Generalized
A. Idiopathic
1. Benign neonatal familial convulsions
2. Benign neonatal convulsions
3. Benign myoclonic epilepsy in infancy
4. Childhood absence epilepsy (pyknolepsy)
5. Juvenile absence epilepsy
6. Juvenile myoclonic epilepsy
7. Epilepsies with grand mal seizures (generalized tonic-clonic
seizures) on awakening
8. Generalized epilepsy with febrile seizures plus
9. Other generalized idiopathic epilepsies
B. Cryptogenic (now probably symptomatic) or symptomatic
1. West syndrome (infantile spasms)
2. Lennox-Gastaut syndrome
3. Epilepsy with myoclonic-astatic seizures
4. Epilepsy with myoclonic absences
continued on next page
"
intensity. These findings are more likely
to be seen with specific MRI protocols,
including thin T2- and fluid-attenuated
inversion recoveryweighted images per-
pendicular to the long axis of the
hippocampus. Patients with mesial tem-
poral lobe epilepsy with hippocampal
sclerosis often have seizures refractory to
AED therapy but may become seizure
free with resection of the anterior tem-
poral lobe, amygdala, and hippocampus.
Autosomal dominant nocturnal
frontal lobe epilepsy. In this syn-
drome, all affected family members
have nocturnal frontal lobe seizures.
Onset is typically in childhood or
adolescence, but seizures frequently
persist into adulthood. Some family
members have such mild seizures that
they never receive a formal diagnosis of
epilepsy. Brief hyperkinetic or tonic
seizures occur in clusters from light
non-REM sleep (Combi et al, 2004).
About 10% of patients also have
seizures during wakefulness. Several
mutations in the neuronal nicotinic
39
KEY POINT:
A Mesial temporal
lobe epilepsy with
hippocampal
sclerosis shows
typical mesial
temporal seizures
and MRI-evident
hippocampal
atrophy or
T2-weighted
signal changes.
TABLE 1-8
Continued
C. Symptomatic
1. Nonspecific etiology
a. Early myoclonic encephalopathy
b. Early infantile encephalopathy with suppression bursts
c. Other symptomatic generalized epilepsies
1. Progressive myoclonic epilepsies
2. Specific syndromes
a. Epileptic seizures may complicate many disease states
III. Undetermined epilepsies (category eliminated in new classification, but
syndromes retained)
A. With both generalized and focal seizures
1. Neonatal seizures
2. Severe myoclonic epilepsy in infancy
3. Epilepsy with continuous spike-waves during slow-wave sleep
4. Acquired epileptic aphasia (Landau-Kleffner syndrome)
B. Without unequivocal generalized or focal features
IV. Special syndromes (now called Conditions with epileptic seizures that
do not require a diagnosis of epilepsy)
A. Situation-related seizures
1. Febrile seizures
2. Isolated seizures or isolated status epilepticus
3. Seizures occurring only when there is an acute or toxic event
a
Newly recognized or modified syndromes are added in italics.
Commission on Classification and Terminology of the International League Against Epilepsy Proposal for revised
classification of epilepsies and epileptic syndromes. Epilepsia 1989;30(4):389399. Reprinted with permission
from Wiley-Blackwell Publishing Ltd.
acetylcholine receptor gene have been
identified as the etiology of this
syndrome, but in other families the
genetic etiology is unknown (Combi
et al, 2004). Inheritance is autosomal
dominant with 80% penetrance.
Idiopathic generalized epilepsies
with variable phenotypes. The new
proposed classification views the idio-
pathic generalized epilepsies as a
biological continuum and groups the
syndromes (juvenile absence epilepsy,
juvenile myoclonic epilepsy, and epi-
lepsy with GTCS on awakening) into a
single group of idiopathic generalized
epilepsies with variable phenotypes.
This grouping was based on several
observations: the clinical features of
the syndromes overlap (Reutens and
Berkovic, 1995), similar EEG findings
can be seen in all syndromes, and
family members of probands with one
syndrome may not have the same
syndrome, but have instead one of
the other idiopathic generalized epi-
lepsy syndromes (Winawer et al, 2005).
This recommendation has not yet been
universally accepted, and grouping by
predominant seizure type as below is
reasonable, especially for selection of
appropriate AED therapy and for ge-
netic studies.
Juvenile absence epilepsy. Sei-
zures in juvenile absence epilepsy
(JAE) begin around puberty, between
ages 9 and 16 years, with a peak at ages
10 to 13 years. Absence seizures are the
main seizure type. Absence seizures in
JAE are infrequent, usually occurring
less than once a day (Nordli, 2005), in
contrast to the hundreds of seizures
per day in childhood absence epilepsy.
Seizures in JAE may be slightly longer
than childhood absence seizures and
may have more prominent oral and
limb automatisms. This sometimes
leads to confusion with complex partial
seizures. Primary GTCS occur in most
patients, often in the first 1 to 2 hours
after awakening. Myoclonic jerks occur
in about 20% of patients but are
typically mild and rare. Remission is
uncommon and seizures often persist
into adulthood. Most patients achieve
seizure control with AEDs, but life-
long therapy is usually necessary. JAE
has a strong genetic component, but
the inheritance pattern is not clearly
elucidated.
Interictal EEG shows generalized
spike-and-wave activity on a normal
background (Duron et al, 2005). Oc-
cipital intermittent rhythmic delta
activity is seen less commonly than
in childhood absence epilepsy. Ictal
EEG shows 3.0-Hz to 4.5-Hz gener-
alized spike-and-slow-wave discharges
(slightly faster than in childhood ab-
sence epilepsy), often with polyspike
components. Neuroimaging studies are
normal.
Juvenile myoclonic epilepsy. Ju-
venile myoclonic epilepsy (JME) is the
most common generalized epilepsy
syndrome in adults, affecting 5% to
10% of adult patients with epilepsy
(Jallon and Latour, 2005). Forty percent
to 50% of patients have a family history
of epilepsy. JME begins around the
time of puberty (ages 12 to 18 years),
usually with frequent myoclonic sei-
zures (Sullivan and Dlugos, 2004).
Myoclonic jerks are bilateral, irregular
jerks affecting predominantly the arms.
Falls may occur when legs are involved.
There is usually no impairment of
consciousness, but confusion may oc-
cur with prolonged clusters of jerks.
Myoclonic jerks often cause morning
clumsiness and dropping of objects
from the hands. The legs are less
commonly involved, but falls can occur.
These may not be recognized as
seizures until the occurrence of a
generalized convulsion. The myoclonic
seizures may occur singly or in clusters
and may progress to generalized clonic-
tonic-clonic seizures. Patients may
not come to medical attention until
the first GTCS occurs. Both myoclonic
40
KEY POINT:
A The idiopathic
generalized
epilepsies can
be considered as
a biological
continuum, with
some patients
showing
predominance
of various
generalized
seizure types
(absence,
myoclonic,
generalized
tonic-clonic).
"
and GTCS typically occur shortly after
awakening (Duron et al, 2005). Fifteen
percent to 30% of patients also have
rare brief absence seizures (Martinez-
Juarez et al, 2006). Common seizure
precipitants are alcohol use, sleep
deprivation, and photic stimulation.
Patients are otherwise neurologically
normal. Approximately 80% of patients
will achieve complete control of sei-
zures with AEDs, but therapy needs to
be continued indefinitely to prevent
seizure recurrence. JME is genetically
heterogeneous, associated with muta-
tions in several genes and with other
loci by linkage analysis.
Interictal EEG shows generalized
frontally maximal polyspikes, polyspike-
wave discharges, and spike-and-wave
activity on a normal background. The
spike repetition rate is typically fast
(3.5 Hz to 6.0 Hz) and irregular. During
sleep, fragmentary, irregular, and asym-
metric spike-wave discharges may be
seen, often with shifting laterality. A
photoparoxysmal response is seen in
30% to 40% of patients, more com-
monly in girls. Neuroimaging studies
are normal. There is a strong gene-
tic component to JME, but the syn-
drome is genetically heterogeneous,
associated with several gene muta-
tions and chromosomal loci (Martinez-
Juarez et al, 2006) (Case 1-3).
Epilepsy with generalized tonic-
clonic seizures on awakening. Like
the other idiopathic generalized epilep-
sies, seizures typically begin in early
adolescence and have a strong genetic
component. GTCS occur almost exclu-
sively in the first 1 to 2 hours after
awakening from sleep. Seizures rarely
may occur in early evening during
periods of relaxation. If other seizure
types are present (ie, myoclonic or
absence), they are very rare and also
show morning predominance. Seizures
are often precipitated by sleep depriva-
tion and alcohol. Interictal EEG shows
generalized fast (3 Hz to 5 Hz) spike-
and-wave activity or polyspike-and-wave
activity on a normal background. A
photoparoxysmal response is common.
Neuroimaging studies are normal. This
syndrome may not be retained in the
new proposed classification.
Generalized epilepsy with fe-
brile seizures plus. Generalized
epilepsy with febrile seizures plus
(GEFS+) is a familial idiopathic epi-
lepsy syndrome in evolution. Because
several different seizure types may be
found within the same family, the
diagnosis can be made only in families,
not in individuals (Nordli, 2005). Sei-
zures typically begin within the first
decade of life. Febrile seizures in
infancy are the most characteristic
seizure type. These are usually recur-
rent simple febrile seizures in the
typical age range for febrile seizures
(age 6 months to 5 years), but seizures
with fever may continue after age 6.
Typical absence, generalized tonic-
clonic, and complex partial seizures
persist into adulthood in some family
members (Scheffer and Berkovic, 1997).
Other seizure types include myoclonic-
astatic and atonic. Interictal EEG shows
generalized spike-and-wave or polyspike-
and-wave discharges. This syndrome
is genetically heterogeneous. Muta-
tions have been described in three so-
dium channel and one -aminobutyric
acid subunit gene.
Lennox-Gastaut syndrome. The
Lennox-Gastaut syndrome typically be-
gins in childhood between the ages of
1 and 6 years but often persists into
adulthood. Lennox-Gastaut syndrome
is characterized by a triad of clinical
features: multiple seizure types refrac-
tory to AEDs (atonic, tonic, atypical
absence, myoclonic, complex partial,
and generalized tonic-clonic); cognitive
impairment or mental retardation; and
interictal EEG showing slow spike-and-
wave discharges at less than 2.5 Hz
(Shields, 2004). Lennox-Gastaut syn-
drome occurs as a consequence of
41
KEY POINT:
A Lennox-Gastaut
syndrome is a
triad of (1)
multiple seizure
types refractory
to antiepileptic
drugs, (2) mental
retardation, and
(3) slow spike-
and-wave activity
on EEG.
many symptomatic etiologies (usually
multifocal or diffuse) such as perinatal
injuries, inborn errors of metabolism,
chromosomal abnormalities, traumatic
brain injury, and mental retardation, but
is sometimes cryptogenic (Markand,
2003). Tonic, atonic, and atypical ab-
sence seizures are most common and
may occur hundreds of times per day.
Atonic seizures commonly result in
serious injuries. Most patients have at
least one episode of status epilepticus.
Prognosis for complete seizure control
is poor. Seizure frequency decreases in
adolescence and adulthood in 25% of
patients, and the characteristic EEG
pattern may no longer be seen.
Interictal EEG shows a slow spike-
and-wave pattern, with bilateral, syn-
chronous, frontally maximal spikes in
bursts or extended runs at 1.5 Hz
to 2.5 Hz. The other major interictal
42
Case 1-3
A 15-year-old girl presents with a first seizure, described as sudden loss of consciousness with a
fall to the ground, a loud scream, stiffening of her body, and generalized shaking lasting 2
minutes. She bit her tongue and had urinary incontinence but did not injure herself. In retrospect,
around age 13, she began having episodes of jerking of her arms in clusters in the morning,
sometimes causing her to drop objects from her hands. She denies any other episodes of staring or
loss of awareness. Her neurologic examination is normal, and her school performance has been
excellent. The night before her seizure, she was up until 3:00 AM studying for an examination. The
seizure happened 30 minutes after awakening in the morning. There is no family history
of seizures or epilepsy. She has no other epilepsy risk factors. Figure 1-6 demonstrates EEG
findings during photic stimulation after partial sleep deprivation. MRI of the brain is normal.
Comment. This case shows the typical presentation of JME, with morning myoclonic jerks
preceding
the onset of
GTCS. This is
an idiopathic
epilepsy
syndrome.
A family
history of
generalized
seizures is
common,
although
many cases
are sporadic.
EEG shows
generalized
spike-and-wave
or polyspike-
and-wave
discharges
at 3.5 Hz to
5.0 Hz and
commonly
shows
photosensitivity,
as in this case.
FIGURE 1-6
EEG during photic stimulation of patient in Case 1-3, showing a
photoparoxysmal response or bursts of high-voltage generalized irregular
polyspikes.
"
feature is paroxysmal fast activity or
diffuse, bilaterally synchronous bursts
of frontally maximal 15-Hz to 20-Hz ac-
tivity lasting several seconds (Brenner
and Atkinson, 1982). This pattern can
be seen during tonic seizures as well
but typically has no discernible clinical
changes. Ictal patterns are variable, in-
cluding those of atypical absence,
tonic, atonic, generalized tonic-clonic,
and complex partial seizures.
Progressive myoclonus epilepsies.
The progressive mitochondrial en-
cephalopathies are a rare group of
disorders characterized by severe my-
oclonus and other generalized sei-
zure types, progressive dementia, and
ataxia. The most common causes are
Unverricht-Lundborg disease (Baltic
myoclonus), Lafora disease, myoclonic
epilepsy with ragged red fibers, and
neuronal ceroid lipofuscinosis. Sialido-
sis, noninfantile Gauchers disease, late
infantile and juvenile GM2 gangliosido-
sis, juvenile neuroaxonal atrophy, and
dentatorubral-pallidoluysian atrophy are
rare, and myoclonus may not be the
predominant clinical feature.
Unverricht-Lundborg disease (Baltic
myoclonus) is an autosomal recessive
disorder caused by a mutation in the
cystatin B on chromosome 21 (Uthman
and Reichl, 2002). Onset is between
8 and 13 years, usually with myoclonic
or GTCS. At onset, the disorder may
be indistinguishable from JME. Myoc-
lonus can be generalized, fragmentary,
and multifocal and is often increased
with action. Some, but not all, jerks are
accompanied by epileptiform activity
on EEG. Ataxia and dementia occur
late and are typically mild. Patients also
frequently have incoordination, inten-
tion tremor, and dysarthria. The EEG
shows 3-Hz to 5-Hz generalized spike-
and-wave or polyspike-and-wave activ-
ity. Background activity may initially be
normal but slows as dementia pro-
gresses. Occipital spikes and seizures
occur independently from both hemi-
spheres. Patients are markedly photo-
sensitive. Giant somatosensory evoked
potentials may be recorded.
Lafora disease is an autosomal re-
cessive disorder. Eighty percent of
cases are caused by mutations in the
laforin gene on chromosome 6. Sei-
zures begin between ages 10 and 18
years. Progression is rapid and relent-
less, resulting in severe myoclonus,
spastic quadriparesis, dementia, and
death in 2 to 20 years. The EEG is
similar to that of Unverricht-Lundborg
disease, and marked photosensitivity
is typical (Uthman and Reichl, 2002).
Mitochondrial encephalopathy with
ragged red fibers shows mitochondrial
(ie, maternal) inheritance. Wide phe-
notypic variability occurs, even within a
single family (Uthman and Reichl,
2002). Symptoms can begin at any age
and include myoclonus, tonic-clonic
seizures, dementia, and ataxia. A host
of other features may occur, including
myopathy, neuropathy, deafness, optic
atrophy, exercise intolerance, short
stature, and lactic acidosis. The EEG
shows generalized spike-and-wave ac-
tivity at 2 Hz to 5 Hz, polyspikes, and
photoparoxysmal responses.
Axis 4: Etiology
The etiology is chosen from the Classi-
fication of Diseases Frequently Associ-
ated with Epileptic Seizures or Epilepsy
Syndromes (Engel, 2001), a specific
genetic defect, or specific pathologic
substrates for symptomatic focal epi-
lepsies. Many genes causing epilepsy
as the major feature have been identi-
fied, and these should be specified
when known. Focal lesions or neuro-
logicabnormalitiescommonlyassociated
with epileptic seizures include tumors,
malformations of cortical development,
CNS infections, CNS inflammation,
degenerative diseases, prenatal or peri-
natal insults, head trauma, chromo-
somal abnormalities, neurocutaneous
43
A Axis 4 is the
etiology of
epilepsy
(idiopathic,
probably
symptomatic, or
symptomatic),
with a specific
gene, lesion, or
disease specified
when possible.
KEY POINTS:
A Progressive
myoclonic
epilepsies are
characterized
by severe
myoclonus, other
generalized
seizures, and
progressive
dementia,
and ataxia.
syndromes, inherited metabolic dis-
eases, hippocampal sclerosis, and vas-
cular insults and malformations.
Axis 5: Impairment
This axis is optional and will be derived
from a World Health Organization
classification of impairment or disability
(Engel, 2001; Engel, 2006b). This will
need to be adapted for the special
needs of patients with epilepsy. Possi-
ble spheres of impairment include
body function (mental, sensory, motor,
speech, cardiovascular), body structure
(nervous system structures involved in
the epilepsy syndrome), activities and
participation (learning, general tasks,
communication), and environmental
(support, employment, relationships).
This axis will aid in determinations of
the impact of epilepsy on overall
quality of life and can facilitate compar-
isons with other chronic diseases.
CONCLUSION
The ILAE structured diagnostic scheme
comprises 5 axes: ictal phenomenol-
ogy, seizure type, epilepsy syndrome,
etiology, and impairment. This scheme
allows classification of most patients
with epilepsy and facilitates choice of
AED therapy and determination of
prognosis. The older international clas-
sifications of seizure type and epilepsy
syndrome have not been replaced,
although some modifications have
been proposed by the ILAE Classifica-
tion Task Force. As our understanding
of the pathophysiology and mecha-
nisms of epilepsy evolve, the classifica-
tions will be revised and adapted to
reflect current knowledge.
REFERENCES
" Andermann F, Berkovic SF. Idiopathic generalized epilepsy with generalized
and other seizures in adolescence. Epilepsia 2001;42(3):317320.
Discussion of commonalities and differences among idiopathic generalized epilepsy
syndromes, with proposal for classification scheme.
" Blume WT, Luders HO, Mizrahi E, et al. Glossary of descriptive terminology
for ictal semiology: report of the ILAE task force on classification and
terminology. Epilepsia 2001;42(9):12121218.
Standardized terminology for describing ictal clinical features.
" Brenner RP, Atkinson R. Generalized paroxysmal fast activity:
electroencephalographic and clinical features. Ann Neurol 1982;11(4):
386390.
EEG features of tonic seizures.
" Combi R, Dalpra L, Tenchini ML, Ferini-Strambi L. Autosomal dominant
nocturnal frontal lobe epilepsya critical overview. J Neurol
2004;251(8):923934.
Discussion of clinical, EEG, and genetic features and differential diagnosis of autosomal
dominant nocturnal frontal lobe epilepsy.
" Commission on Classification and Terminology of the International League
Against Epilepsy. Proposal for revised clinical and electroencephalographic
classification of epileptic seizures. Epilepsia 1981;22(4):489501.
Currently accepted classification for epileptic seizures.
44
KEY POINT:
A Axis 5 is an
optional axis
describing
associated
impairments in
body function,
body structure,
daily activities,
and psychosocial
functioning.
"
" Commission on Classification and Terminology of the International League
Against Epilepsy Proposal for revised classification of epilepsies and
epileptic syndromes. Epilepsia 1989;30(4):389399.
Currently accepted classification for epilepsy syndromes.
" Deacon C, Wiebe S, Blume WT, et al. Seizure identification by clinical
description in temporal lobe epilepsy: how accurate are we? Neurology
2003;61(12):16861689.
Comparison of clinical diagnosis by history compared with video-EEG recordings of
temporal lobe seizures.
" Devinsky O, Kelley K, Porter RJ, Theodore WH. Clinical and
electroencephalographic features of simple partial seizures. Neurology
1988;38:13471352.
" Duron RM, Medina MT, Martinez-Juarez IE, et al. Seizures of idiopathic
generalized epilepsies. Epilepsia 2005;46(suppl 9):3447.
Description of clinical and EEG features of absence, myoclonic, and generalized
tonic-clonic seizures.
" Engel J Jr. ILAE classification of epilepsy syndromes. Epilepsy Res
2006a;70(suppl 1):510.
" Engel J Jr. Report of the ILAE classification core group. Epilepsia
2006b;47(9):15581568.
Overview of new classification proposals, highlighting proposed changes and
controversial areas.
" Engel J Jr. International League Against Epilepsy (ILAE). A proposed
diagnostic scheme for people with epileptic seizures and with epilepsy:
report of the ILAE Task Force on Classification and Terminology. Epilepsia
2001;42(6):796803.
Description of the ILAE 5-axis diagnostic scheme.
" Erkwoh R, Steinmeyer EM. Phenomenology of simple partial seizures.
Seizure 1996;5(4):283289.
Description of clinical features of simple partial seizures.
" Fisher RS, van Emde Boas W, Blume W, et al. Epileptic seizures and
epilepsy: definitions proposed by the International League Against
Epilepsy (ILAE) and the International Bureau for Epilepsy (IBE). Epilepsia
2005;46(4):470472.
New definitions for seizures and epilepsy.
" Gil-Nagel A, Risinger MW. Ictal semiology in hippocampal versus
extrahippocampal temporal lobe epilepsy. Brain 1997;120(pt 1):183192.
" Holmes GL, McKeever M, Adamson M. Absence seizures in children: clinical
and electroencephalographic features. Ann Neurol 1987;21(3):268273.
" Jallon P, Latour P. Epidemiology of idiopathic generalized epilepsies.
Epilepsia 2005;46(suppl 9):1014.
45
" Janszky J, Schulz R, Ebner A. Simple partial seizures (isolated auras) in
medial temporal lobe epilepsy. Seizure 2004;13(4):247249.
" Kim DW, Lee SK, Yun CH, et al. Parietal lobe epilepsy: the semiology, yield
of diagnostic workup, and surgical outcome. Epilepsia 2004;45(6):641649.
" Leppik IE. Classification of the myoclonic epilepsies. Epilepsia
2003;44(suppl 11):26.
" Loddenkemper T, Kellinghaus C, Wyllie E, et al. A proposal for a
five-dimensional patient-oriented epilepsy classification. Epileptic Disord
2005;7(4):308316.
Description of the Cleveland Clinic semiologic classification system.
" Loiseau P, Duche B, Loiseau J. Classification of epilepsies and epileptic
syndromes in two different samples of patients. Epilepsia 1991;32(3):
303309.
" Maillard L, Vignal JP, Gavaret M, et al. Semiologic and electrophysiologic
correlations in temporal lobe seizure subtypes. Epilepsia 2004;45(12):
15901599.
" Markand ON. Lennox-Gastaut syndrome (childhood epileptic
encephalopathy). J Clin Neurophysiol 2003;20(6):426441.
" Martinez-Juarez IE, Alonso ME, Medina MT, et al. Juvenile myoclonic
epilepsy subsyndromes: family studies and long-term follow-up. Brain
2006;129(pt 5):12691280.
" Niaz FE, Abou-Khalil B, Fakhoury T. The generalized tonic-clonic seizure in
partial versus generalized epilepsy: semiologic differences. Epilepsia
1999;40(11):16641666.
" Nordli DR Jr. Idiopathic generalized epilepsies recognized by the
International League Against Epilepsy. Epilepsia 2005;46(suppl 9):4856.
" Ottman R, Lee JH, Hauser WA, et al. Reliability of seizure classification using
a semistructured interview. Neurology 1993;43(12):25262530.
Structured questionnaire to improve interrater reliability in seizure classification.
" Ottman R, Winawer MR, Kalachikov S, et al. LGl1 mutations in autosomal
dominant partial epilepsy with auditory features. Neurology
2004;62(7):11201126.
Genetics and clinical features of autosomal dominant partial epilepsy with auditory features.
" Penry JK, Porter RJ, Dreifuss RE. Simultaneous recording of absence
seizures with video tape and electroencephalography. A study of 374
seizures in 48 patients. Brain 1975;98(3):427440.
" Picot MC, Crespel A, Tricot M, et al. Validity of diagnosis using the French
translation of the semi-structured interview for seizure classification.
Epilepsia 1999;40(11):16491656.
" Reutens DC, Berkovic SF. Idiopathic generalized epilepsy of adolescence:
are the syndromes clinically distinct? Neurology 1995;45(8):14691476.
Discussion of overlap of idiopathic generalized epilepsies.
46
"
" Rugg-Gunn FJ, Harrison NA, Duncan JS. Evaluation of the accuracy of
seizure descriptions by the relatives of patients with epilepsy. Epilepsy
Res 2001;43(3):193199.
Study of ability of witnesses to identify important localizing and lateralizing features of
videotaped seizures.
" Salanova V, Morris HH, Van Ness P, et al. Frontal lobe seizures: electroclinical
syndromes. Epilepsia 1995;36(1):1624.
" Scheffer IE, Berkovic SF. Generalized epilepsy with febrile seizures plus.
A genetic disorder with heterogeneous clinical phenotypes. Brain
1997;120(pt 3):479490.
" Shields WD. Diagnosis of infantile spasms, Lennox-Gastaut syndrome,
and progressive myoclonic epilepsy. Epilepsia 2004;45(suppl 5):24.
" Shulman MB. The frontal lobes, epilepsy, and behavior. Epilepsy Behav
2000;1(6):384395.
" Sullivan JE, Dlugos DJ. Idiopathic generalized epilepsy. Curr Treat Options
Neurol 2004;6(3):231242.
" Taylor I, Scheffer IE, Berkovic SF. Occipital epilepsies: identification
of specific and newly recognized syndromes. Brain 2003;126(pt 4):753769.
" Theodore WH, Porter RJ, Albert P, et al. The secondarily generalized
tonic-clonic seizure: a videotape analysis. Neurology 1994;44(8):14031407.
Review of clinical features of secondarily generalized partial seizures.
" Theodore WH, Porter RJ, Penry JK. Complex partial seizures: clinical
characteristics and differential diagnosis. Neurology 1983;33(9):11151121.
" Uthman BM, Reichl A. Progressive myoclonic epilepsies. Curr Treat Options
Neurol 2002;4(1):317.
Update on clinical features, EEG features, diagnostic evaluation, genetics, and treatment of
progressive myoclonus epilepsy.
" Wieser HG, ILAE Commission on Neurosurgery of Epilepsy. ILAE commission
report. Mesial temporal lobe epilepsy with hippocampal sclerosis. Epilepsia
2004;45(6):695714.
Proposed new epilepsy syndrome.
" Winawer MR, Marini C, Grinton BE, et al. Familial clustering of seizure types
within the idiopathic generalized epilepsies. Neurology 2005;65(4):523528.
Discussion of utility of seizure type versus epilepsy syndrome in genetic studies.
47

Classification of Epileptic Seizures

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