Accepted: 11 December 2017
DOI: 10.1111/epi.13996
CRITICAL REVIEW AND INVITED COMMENTARY
Generalized nonmotor (absence) seizures—What do absence,
generalized, and nonmotor mean?
Iris Unterberger1 | Eugen Trinka2 | Peter W. Kaplan3 | Gerald Walser1 |
Gerhard Luef1 | Gerhard Bauer1
1
Department of Neurology, Innsbruck
Medical University, Innsbruck, Austria
2
Department of Neurology, Christian-
Doppler-Klinik, Paracelsus Medical
University of Salzburg, Salzburg, Austria
3
Johns Hopkins Bayview Neurology,
Baltimore, MD, USA
Correspondence
Iris Unterberger, Department of
Neurology, Innsbruck Medical University,
Innsbruck, Austria.
Email: iris.unterberger@icloud.com
1 | INTRODUCTION
The operational classification of seizure types by the Inter-
national League Against Epilepsy (ILAE) differentiates
Epilepsia. 2018;1–7.
Summary
Objective: Clinical absences are now classified as “generalized nonmotor (ab-
sence) seizures” by the International League Against Epilepsy (ILAE). The aim of
this paper is to critically review the concept of absences and to put the accompa-
nying focal and motor symptoms into the context of the emerging pathophysio-
logical knowledge.
Methods: For this narrative review we performed an extensive literature search
on the term “absence,” and analyzed the plethora of symptoms observed in clini-
cal absences.
Results: Arising from the localization and the involved cortical networks, motor
symptoms may include bilateral mild eyelid fluttering and mild myoclonic jerks
of extremities. These motor symptoms may also occur unilaterally, analogous to a
focal motor seizure with Jacksonian march. Furthermore, electroencephalography
(EEG) abnormalities may exhibit initial frontal focal spikes and consistent asym-
metries. Electroclinical characteristics support the cortical focus theory of absence
seizures. Simultaneous EEG/functional magnetic resonance imaging (fMRI) mea-
surements document cortical deactivation and thalamic activation. Cortical deacti-
vation is related to slow waves and disturbances of consciousness of varying
degrees. Motor symptoms correspond to the spike component of the 3/s spike-
and-wave-discharges. Thalamic activation can be interpreted as a response to
overcome cortical deactivation. Furthermore, arousal reaction during drowsiness
or sleep triggers spikes in an abnormally excitable cortex. An initial disturbance
in arousal mechanisms (“dyshormia”) might be responsible for the start of this
abnormal sequence.
Significance: The classification as “generalized nonfocal and nonmotor (absence)
seizure” does not covey the complex semiology of a patient’s clinical events.
KEYWORDS
absence seizures, generalized epilepsy, motor seizures, nonmotor seizures
seizures with focal and generalized/bilateral onset.1 Gener-
alized seizures are divided into motor seizures and nonmo-
tor (absence) seizures. However, what does “absence”
mean? Are typical and atypical absence seizures nonmotor?
wileyonlinelibrary.com/journal/epi Wiley Periodicals, Inc. | 1
© 2018 International League Against Epilepsy
2
| UNTERBERGER
Furthermore, is the electroclinical onset of absences indeed
nonfocal? Here we critically discuss the larger implications
and more complex symptomatology of absence seizures.
2 | WHAT DOES “ABSENCE” MEAN?
According to the Oxford English Dictionary the term “ab-
sence” denotes a state of being absent or away from any
place. In the 18th century, the term was also used in a
sense of absence of mind, such as “inattention to what is
going on or failure to receive impression of what is pre-
sent.”2 With absence seizures, the term is mostly used for
absence of consciousness, which itself is a composite of
arousal (alertness) and awareness (knowledge of self and
environment).3,4 Unconscious subjects are unaware per def-
inition, but unawareness is not implicitly associated with
loss of consciousness as is the case in comatose conditions.
Coma is defined as “a state of unarousable psychologic
unresponsiveness in which subjects lie with eyes closed.”4
Coma results from a dysfunction of the Ascending Reticu-
lar Activating System (ARAS) or disruptions of connec-
tions of widespread cortical areas with the thalamus and
globus pallidus.5 The centrencephalic6 as well as the corti-
coreticular hypothesis of absences7 assume primary
involvement of the reticular system. However, during
absence seizures, patients are not comatose, their eyes are
open, and they retain an upright position, unless in rare
cases, when atonic components prevail.8,9 Furthermore,
aside from coma, the differential diagnoses include sleep,
physiological behavioral changes resulting from impaired
attentiveness due to preoccupation with other activities,
inability to react properly to exogenous stimuli in conse-
quence of neurological problems such as dementia, chronic
disturbances of consciousness such as the unreactive wake-
fulness (apallic) syndrome, and psychiatric conditions with
hallucinations, fugue states, stupor, and other neurologic/
psychiatric conditions.
Hence, how can one interpret the nature of the distur-
bances of consciousness during 3/s spike and wave dis-
charge (SWD) absence seizures? Most authors have stated
that consciousness is not invariably lost during SWD—
absences. Such a conscious/unconscious continuum ranges
from a “light slowing of responses and slight confusion in
thinking”9 to the complete loss of consciousness.10 L€uders
et al.11 differentiated 5 types of disturbances of conscious-
ness in epileptic seizures, and considered 1 type (“dialep-
sis”) typical for 3/s SWD absences. Dialepsis was
described as “. . .unresponsiveness to external stimuli,
absence of any impediment of the arousal system . . . and
total amnesia for the event.” In contrast, with absence sei-
zures, the degree of memory deficit may vary from reten-
tion of memory to partial or complete amnesia.12 Most
ET AL.
Key Points
• The medical term absence has been used to
denote a state of absence of consciousness,
which itself is a composite of alertness and
awareness
• The ambiguity of the term has not been solved
by the new terminology classifying, absences as
“generalized nonmotor (absence) seizures”
• Absence seizures encompass not only a “blank
stare” but also a variety of motor features, which
also can be asymmetric, regional, or focal
• Clinical absences represent bilateral network sei-
zures with focal or regional (asymmetric) onset
and mild, but recognizable motor signs
authors agree, that different aspects of consciousness and
awareness are selectively impaired.12–16 For example, some
absence seizures result in pure prefrontal behavioral
changes including suspension of working memory.17,18
However, the prefrontal disturbances represent only 1 fea-
ture of cortical seizures, and the nature and degree of alter-
ations of consciousness depend on the localization and
number of impaired cortical areas.19
To the best of our knowledge, there is no report on sys-
tematic correlations of clinical signs in absence seizures
and the relevant involved cortical networks.
3 | ARE ABSENCE SEIZURES
NONMOTOR AND NONFOCAL FROM
ONSET?
As correctly stressed by Fisher et al.,1 absences are differ-
ent from seizures observed in focal epilepsies with a pro-
ven or suspected structural/metabolic etiology.
Aside from behavioral changes, clinical signs may
include abnormal motor activities. Motor activities and
focal signs in absence seizures have been reported for
many years. Janz20 described the incidence and distribution
of mild bilateral myoclonic jerks during “pyknoleptic petit
mal” (ie, absence seizures in childhood absence epilepsy
[CAE]).
In 1981, the classification of epileptic seizures9 defined
absence seizures as impairment of consciousness with mild
clonic, atonic, tonic, and autonomic components. Blank
staring represents a cardinal symptom during absences,
which could be interpreted as a frontal negative motor sign
but has not yet been experimentally established. Bilateral
myoclonic jerks mostly involve the oculomotor system in
the form of eyelid fluttering. Absence seizures with
UNTERBERGER ET AL.
pronounced eyelid myoclonus are considered a distinct
generalized idiopathic epilepsy syndrome.17 Atonic rhyth-
mical nodding represents a sign of a negative motor activ-
ity in absences.9 Complex automatisms may also be
observed20 and were recently considered a reactive phe-
nomenon to internal and external stimuli during prolonged
absence seizures.21 The ictal nature of a subtype of oral
automatisms can be deduced from the simultaneous onset
of spikes with synchronous rhythmicity (Appendix Case 1).
In these cases, an ictal involvement of the oroalimentary
cortical areas is likely. Other focal motor symptoms include
circling,22 ictal head version,20,23–25 horizontal nystag-
mus,26 unilateral repetitive myoclonus,25 or even a motor
sequence comparable to a Jacksonian march.25 The most
remarkable study on the seizure semiology of absences was
performed by Stefan et al. 35 years ago.25 For this docu-
mentation, patients were investigated in standard conditions
prospectively in a sitting position wearing only a short-
sleeved shirt and shorts. Video documentation of behavior
was performed with a total view of the body and additional
close-up view of the face. In this study, a careful analysis
of 528 absences revealed that in addition to myoclonic,
atonic motor signs and automatisms also frequent tonic
movements occur during absences. Ictal motor signs during
absences present in a complex motor pattern, or with iden-
tical time delay from spike wave onset as fragments of the
whole motor pattern, or even as single elements. An exam-
ple of such a motor pattern and with temporal sequence of
the single ictal elements is ocular, then face movement, fol-
lowed by tonic increase in upper and lower extremities
with finally also unilateral dorsal extension of the big toe.
This observation led to the term “craniocaudal march” of
absence signs.27,28 The manifestation of the different motor
elements after ocular movements was dependent on the
duration of the SWDs.25 The knowledge of the Gestalt of
the complex motor program facilitates the clear allocation
of even single-occurring elements or fragments. More
recently, Kessler et al.29 analyzed videos of electrographic
absence seizures (n = 1.932) in 416 patients with CAE.
The authors described pause/stare as the most common
semiological feature (99.3%) and confirmed the high per-
centage of motor automatisms (86.1%) and eye involve-
ment (76.5%).
Electroencephalography (EEG) recordings in patients
with typical and atypical absence seizures exhibit a number
of localizing features. A frontal amplitude maximum of
spikes and waves had already been described in earlier
papers23,30,31 and recently confirmed by Seneviratne et al.32
Besides having frontal amplitude maxima, 3/s SWDs might
begin with unilateral frontal spikes in 36.9%23 or in
21.5%32 of patients, which may have changing lateraliza-
tion.23 Matthes33 called these spikes “pseudofocal,” sug-
gesting that they are different from those due to structural
| 3
cortical lesions. Early observations were summarized by
Bauer23 and Dalby34 and are corroborated by later studies
in absence epilepsies35–37 as well as in other idiopathic
generalized epilepsy syndromes.38–40 Constant initial unilat-
eral frontal spikes at the onset of absences clearly support
the cortical focus theory of bilateral 3/s SWDs.30,39,41,42
Focal abnormalities have also been demonstrated using
magnetoencephalography (MEG).43–45 Furthermore, Sakurai
et al.45 showed that cortical regions that constitute a default
mode network are strongly involved in the generalized
spike wave process in some patients with juvenile absence
epilepsy (JAE). Combined MEG/EEG recordings demon-
strated initial involvement of frontal areas, often combined
with activity in the periinsular and subcortical/thalamic
areas during bilateral spikes discharges.46
In addition,, regional gray matter changes in frontal
regions have been reported in volumetric MRI studies of
patients with IGE.47–49 Simultaneous registration of EEG
and fMRI have added further localizing aspects. During
SWDs, blood oxygen level–dependent contrast imaging
(BOLD) responses are activated or deactivated in different
areas. Activation reflects increased, and deactivation may
reflect decreased, neuronal activity.50 BOLD signals are
found in several regions with different characteristics but
without constant asymmetries. Most studies report deactiva-
tions in different parts of cortical networks, especially in
the cortical default mode network (DMN), and activations
in thalamic structures.51–56 Archer et al.57 found deactiva-
tion in cortical regions but no changes in the thalamus. Ini-
tial cortical activations followed by profound deactivations
are reported by Bai et al.58 and Benuzzi et al.59 In the
thalamo-cortical-striatal network, activations are observed
prior to EEG discharges.53,58–61 These initial activations
might also explain shortened reaction times immediately
before the onset of SWDs.62
In summation, patients exhibit not only an impairment
of consciousness during 3/s SWD seizures, but they also
have a number of motor and focal signs. These variable
electroclinical signs and symptoms are cortically mediated.
Berg et al.63 discussed the apparent anomaly of focal signs
in generalized epileptic seizures: “Generalized epileptic sei-
zures are conceptualized as originating at some point
within, and rapidly engaging bilaterally distributed net-
works. Although individual seizure onsets can appear local-
ized, the location and lateralization are not consistent from
one seizure to another.”
4 | ABSENCE SEIZURES AND
EPILEPSY SYNDROMES
Absence seizures occur with different epilepsy syndromes.
In CAE and in JAE, absence seizures are part of the
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| UNTERBERGER
syndromic definition. In juvenile myoclonic epilepsy
(JME), absences are frequently but not invariably observed.
Panayiotopoulos et al.64 found separable clinical and EEG
symptoms within idiopathic generalized epilepsy subsyn-
dromes. Betting et al.49 confirmed differences by voxel-
based morphometry and Pugnaghi et al.55 by BOLD
changes. No syndrome-specific differences could be estab-
lished with pure electroclinical parameters except for statis-
tically shorter absences in JME.15,21,65
Absence seizures cannot be taken as equivalent to hav-
ing an idiopathic generalized epilepsy. Atypical absence
seizures occur in Lennox-Gastaut syndrome (LGS) in up to
60% of patients.66 Atypical absence seizures are included
in the newest classification of seizures.1 Two per second
slow spike-and-waves (so called “petit mal variant” after
Gibbs and Gibbs30) represents ictal EEG activities easily
differentiated from typical 3/s SWD pattern. In a pure
semiological sense, the loss of consciousness in frontal
lobe67 as well as in temporal lobe epilepsies (“fausses
absences d’origine temporale”68) can resemble that of
absence seizures. Atypical absences in LGS and absence-
like seizures in focal epilepsies can be distinguished from
typical absence seizures (TAS) in idiopathic generalized
epilepsies by EEG and—less accurately—by accompanying
neurological symptoms.
5 | RELATION OF EEG AND
CLINICAL SYMPTOMS
Duration of SWDs longer than 5 seconds is considered the
minimum for the manifestation of clinical symptoms,
although shorter discharge durations may exhibit inconspic-
uous symptoms, such as slight impairment in cognitive or
motor tasks.69 The depth of disturbances of consciousness
depends on voltage and distribution of the SWDs,6,65,70,71
whereas the duration had no effect.70,72 Amplitudes of
BOLD changes in fMRI/EEG investigations are linearly
related to SWD duration.55 Guo et al.19 demonstrated a
relationship between the number of involved areas and
behavioral symptoms.
In recent years, there has been ongoing controversy
about the pathophysiology of absence seizures—cortical
versus subcortical—and on the cortical focus theory. All
clinical symptoms are cortically mediated, whether trig-
gered by subcortical or cortical pacemakers, or not.6
5.1 | Is there any clue that motor and
consciousness features correspond to different
components of the SWD pattern?
To our knowledge, experimental and clinical evidence at
this time cannot confirm the hypothesis, but there are
ET AL.
several clinical indications that motor symptoms are
directly connected with the spikes. First, motor signs occur
synchronous to the spikes.20,72 Furthermore, types of motor
symptoms correlate with regions most involved by spikes,
that is, the frontal eye field and frontal motor areas respon-
sible for eyelid fluttering and mild myoclonic jerks.20,73
Finally, exaggerated jerks in JME are time-locked to poly-
spikes.20
Absence symptoms exhibit no overt rhythmic fluctua-
tions; they manifest as a continuous suppression of cortical
function. Slow waves have been interpreted as a response
that attempts to suppress the development of poly-spikes
and more massive motor symptoms, an inhibitory “brake
wave” so to speak (“Bremswelle” after Jung and Toen-
nies).74 In this interpretation, slow waves are an inhibitory
phenomenon. Fromm75 considered “staring spells” as inhi-
bitory seizures due to the inhibition of cortical activity.
Penfield and Jasper6 explained the loss of function as a
“‘line busy’ effect.” Cortical BOLD deactivations support
the concept of cortical inhibition. Recent studies support
such inhibitory theories.76,77 Differences in the degree and
symptomatology of behavioral as well as motor signs can
be explained by the involvement of different cortical areas
—corroborated by EEG and fMRI results.
6 | SUMMARIZING HYPOTHESIS
AND CLASSIFICATION
The generation of absences and SWDs can be summarized
as follows. Arousal signals trigger thalamic activation and
focal or bilateral synchronous spikes in a regionally or
multiregionally electrically hyperexcitable cortex.78 This
triggering mechanism of SWDs was called “dyshormia” by
Niedermeyer.79 Spikes are related to mild motor symptoms.
Inhibitory slow waves (brake waves) prevent the develop-
ment of poly-spikes, massive myoclonus, and eventually
generalized tonic–clonic seizures. These inhibitory slow
waves correlate with cortical BOLD deactivation and clini-
cal impairment of consciousness. Ongoing arousal stimuli
—as evidenced by activated BOLD responses in thalamic
areas—can be interpreted as an effort to overcome cortical
inhibition, and to restore normal cortical functions. Alterna-
tively, there may be triggering of recurrent spikes until the
SWD sequence disintegrates due to overwhelming inhibi-
tory influences.
All attempts at classifying natural phenomena usually
involve inevitable elements of abstraction, and interpreta-
tion with loss of clinical detail. By necessity the endeavor
to find a simple term for a complex clinical phenomenon
involving disturbance of consciousness and motor signs,
carries a high risk of oversimplification. To characterize
and classify the multifaced semiology of absence seizures
UNTERBERGER ET AL.
as “generalized onset, nonmotor seizures”1 is an oversim-
plification, which does not fully take into consideration our
present knowledge that absences represent bilateral network
seizures with focal or regional (asymmetric) onset and
mild, but clearly recognizable unilateral, or bilateral motor
signs. Thus we suggest the use of the term absence seizure
without any further descriptor. The term absence speaks
for itself, and any added term would weaken the stringent
concept of absences as it stands.
DISCLOSURE
None of the authors has any conflict of interest to disclose.
We confirm that we have read the Journal0 s position on
issues involved in ethical publication and affirm that this
report is consistent with those guidelines.
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APPENDIX 1: ILLUSTRATIVE CASE
REPORT
Case 1. Male, 10 years. Mild cognitive impairment, no
other neurologic abnormalities. The patient was referred to
our EEG laboratory for attacks with “lip smacking.” Sev-
eral attacks per day occurred, lasting for 10-20 seconds
and were characterized by unresponsiveness, memory loss,
and stereotyped rhythmic lip smacking. The EEG exhibited
typical 3/s SWDs without any asymmetries. During EEG
registration, the observation revealed the start of smacking
with the first spike and a rhythmical repetition synchronous
to the spike-component of the SWD pattern. With the stop
of discharges, clinical symptoms disappeared. The conclud-
ing diagnosis was CAE with absence seizures characterized
by ictal automatisms.

How to cite this article: Unterberger I, Trinka E,

Kaplan PW, Walser G, Luef G, Bauer G.
Generalized nonmotor (absence) seizures—What do
absence, generalized, and nonmotor mean? Epilepsia.
2018;00:1–7. https://doi.org/10.1111/epi.13996