Epilepsia, 53(Suppl.
3):1–51, 2012
doi: 10.1111/j.1528-1167.2012.03593.x
EEGS IN NONCONVULSIVE STATUS EPILEPTICUS
Electroencephalographic criteria for nonconvulsive
status epilepticus: Synopsis and comprehensive survey
*yRaoul Sutter and yPeter W. Kaplan
*Division of Neurosciences Critical Care, Departments of Anesthesiology and Critical Care Medicine,
The Johns Hopkins University School of Medicine, Baltimore, Maryland, U.S.A.; and yDepartment of Neurology,
The Johns Hopkins Bayview Medical Center, Baltimore, Maryland, U.S.A.
SUMMARY
There have been many attempts at defining the
electroencephalography (EEG) characteristics of
nonconvulsive status epilepticus (NCSE) without a
universally accepted definition. This lack of con-
sensus arises because the EEG expression of NCSE
does not exist in isolation, but reflects status epi-
lepticus under the variety of pathologic conditions
that occur with age, cerebral development,
encephalopathy, and epilepsy syndrome. Current
NCSE definitions include ‘‘boundary conditions,’’
in which electroencephalographic seizure activity
occurs without apparent clinical seizures. Further-
more, what appears to one interpreter as status epi-
lepticus, is not to another reader, reflecting the ‘‘art’’
of EEG interpretation. Seizures and epilepsy syn-
dromes have undergone an evolution that has
moved beyond a classification of focal or general-
ized conditions into a syndromic approach. It
seems appropriate to make similar changes in the
EEG analysis of the syndromes of NCSE. In effect,
the literature on epilepsy classification has pro-
gressed to incorporate the different NCSE types
with clinical descriptions, but the specific EEG evi-
dence for these types is found largely in individual
reports, and often by description only. NCSE clas-
sification of EEG patterns should derive from the
aggregate of published EEG patterns in the respec-
tive clinical subtype, supported by an analysis of
these EEG studies. The analysis that follows pre-
Address correspondence to Peter W. Kaplan, Department of Neurol-
ogy, Johns Hopkins Bayview Medical Center, 301 Mason Lord Drive,
Suite 2100, Baltimore, MD 21224, U.S.A. E-mail: pkaplan@jhmi.edu or
Raoul Sutter, Division of Neurosciences Critical Care, Department of
Anesthesiology, Critical Care Medicine and Neurology, Johns Hopkins
University School of Medicine, Baltimore, Maryland, U.S.A. E-mail:
rsutter3@jhmi.edu until July 2013; from August 2013 forward:
Department of Neurology and Intensive Care Unit, University Hospital
Basel, Petersgraben 4, 4031 Basel, Switzerland. E-mail sutterr@uhbs.ch
Wiley Periodicals, Inc.
ª 2012 International League Against Epilepsy
1
sents clinical descriptions and EEG patterns of
NCSE in the neonatal period, infancy, childhood,
adulthood, and late adulthood from a syndromic
perspective based on age, encephalopathy, cere-
bral development, etiology, and syndrome. Pro-
ceeding from the proposed classification of status
epilepticus syndromes in ‘‘Status epilepticus: its
clinical features and treatment in children and
adults’’ (published in 1994 by Cambridge Univer-
sity Press, New York), we have performed a sys-
tematic search for reports presenting EEG
patterns of NCSE using the online medical search
engine PubMed for 22 different search strategies.
EEG patterns were reviewed by two board-certi-
fied epileptologists who reached consensus
regarding presence of NCSE. From a total of 4,328
search results, 123 cases with corresponding EEG
patterns could be allocated to underlying epilepsy
syndromes. Typical characteristic, prominent
electrographic patterns, and sequential arrange-
ments are elucidated for the different NCSE syn-
dromes. This compendium of patterns by NCSE
syndrome classification with illustration of EEGs,
and delineation of electroencephalographic fea-
tures helps define the characteristics and semio-
logic borderlines among the types of NCSE.
KEY WORDS: Epilepsy, Nonconvulsive status
epilepticus, Electroencephalographic criteria,
Diagnosis criteria, Clinical syndromes, Electroen-
cephalographic patterns, Neonatal, Infants, Child-
hood, Adults, Late life.
Nonconvulsive status epilepticus (NCSE) has been
defined as a state of ongoing (or nonrecovery between)
seizures without convulsions, usually for more than
30 min (Shorvon, 1994; Kaplan, 1996). When diagnosing
NCSE, the clinician faces challenges in correlating sug-
gestive clinical features with electroencephalography
(EEG) patterns to arrive at a diagnosis of NCSE. The con-
2
R. Sutter and P. W. Kaplan
firmation of NCSE is largely based on the EEG, given the
nonspecific and pleomorphic clinical manifestations.
Proposed EEG criteria for NCSE are directed mainly at
forms of NCSE seen in adults (Young et al., 1996;
Kaplan, 2007). This approach has not been used in pedi-
atric patients, as the EEG appearance of NCSE in early
life is fundamentally influenced by age, cerebral
maturity, presence of encephalopathy, and underlying
epilepsy syndromes, and NCSE does not lend itself
easily to a set of EEG criteria. Several different epileptic
encephalopathies occurring in neonatals, infants, and
children may appear as continuous or subcontinuous
epileptiform activity, such as in West syndrome, with
hypsarrhythmia (Riikonen, 2005), and in other forms of
malignant epilepsies of childhood, such as in Ohtahara
(Ohtahara & Yamatogi, 2006) and Dravet syndrome
(Dravet et al., 2005). In other less-specific cases,
encephalopathy can be due to numerous etiologies,
including peripartum anoxia, metabolic disorders, infec-
tion, or developmental disturbances, in which NCSE
may present less-specific features.
For diagnosis, the art of and training for EEG interpretation
are key to the identification of ‘‘seizures’’ or ‘‘seizure activ-
ity,’’ since many epileptiform morphologies may not alone
represent seizures. Some nonseizure, epileptiform exam-
ples include ‘‘interictal’’ periodic discharges that may be
seen focally, in a generalized pattern, independently/bilat-
erally, or when brought out by stimulation—periodic
lateralized epileptiform discharges (PLEDs), generalized
periodic epileptiform discharges (GPEDs), bilateral
independent periodic lateralized epileptiform discharges
(BIPLEDs), and stimulus-induced rhythmic, periodic, or
ictal discharges (SIRPIDs), respectively. These discharges
have been described as lying along an ictal–interictal con-
tinuum ranging from the interictal isolated epileptiform
discharge (Chong & Hirsch, 2005), through clustering, or
an increased frequency of periodic discharges, to seizures
and status epilepticus. There has been an ongoing effort
reflected in three international colloquia to define EEG
criteria of NCSE (Walker et al., 2005; Shorvon et al.,
2007; Trinka & Shorvon, 2009). One challenge to this cor-
ralling of EEG characteristics under one roof has been the
recognition that just as there are many epilepsies, there are
many types of status epilepticus and hence NCSE. Over
the last 20 some years, with the increasing recognition of
NCSE—initially identified in ambulatory confused
patients, and in mildly confused hospitalized patients—there
has been increasing identification in lethargic and
comatose patients in intensive care units.
EEG definitions for NCSE have been attempted by
reducing the EEG analysis and diagnosis to one of patterns
with particular characteristics. These were judged to reflect
special, diagnostically important characteristics in fre-
quency, amplitude, morphology, and evolution domains.
Such efforts have led to criteria that may loosely fit all types
Epilepsia, 53(Suppl. 3):1–51, 2012
doi: 10.1111/j.1528-1167.2012.03593.x
of NCSE. However, they largely and reliably are applicable
to the more obvious cases of NCSE, and avoid the more
contentious middle-ground that may lie further down the ic-
tal–interictal continuum. What is needed to supplement
EEG definitions for NCSE is an approach contoured to the
specific settings and syndromes in which these states exist,
and culled from peer-reviewed literature that established
status in these cases with EEG documentation. These tai-
lored characterizations would help differentiate more
clearly seizures or status epilepticus from nonseizures,
leaving less of an uncertain middle-ground, and would
reside in a syndromic context for the various NCSEs.
Previously published criteria include descriptions of the
morphologies, discharge frequencies, evolution patterns,
and secondary criteria for NCSE (Young et al., 1996; Cla-
assen et al., 2004; Kaplan, 2007). Included are characteristics
of EEG evolution, rhythmicity, and response to antiepileptic
drugs (AEDs). However, the determination of status
epilepticus on EEG is inevitably subjective. In most publi-
cations on these entities, authors make the ‘‘leap’’ to a
diagnosis of status epilepticus without providing objective
criteria of frequency, amplitude, morphology, and evolu-
tion of EEG characteristics, and often without showing the
EEG. Illustrative figures of the EEG patterns are often
absent, may be ambiguous, or may show only the most evi-
dent cases.
With these caveats in mind, this compendium provides
the various EEG patterns seen in NCSE from a syndromic
perspective, and expands on acute symptomatic forms
based on etiology.
Material and Methods
Setting and design
This observational study was performed at the depart-
ment of Neurology, Johns Hopkins Bayview Medical
Center in Baltimore, Maryland, U.S.A. It is a compendium
of EEG patterns for NCSE in the neonatal period, infancy,
childhood, adulthood, and late life.
Data and case collection
A systematic search for case reports, case series, or
reviews presenting EEG patterns of NCSE in a syndromic
context was performed using the online medical search
engine PubMed (http://www.ncbi.nlm.nih.gov/pubmed) for
22 search strategies (Table 1) according to the proposed
revised classification of status epilepticus syndromes by
Shorvon (1994) (Table 2). Cases that present figures of EEG
patterns meeting the criteria for NCSE, as defined below, in
association with underlying epilepsy or ‘‘boundary’’ syn-
dromes as classified in Table 1 were selected by critical
review of two board-certified epileptologists (RS and
PWK). For all syndromes, one representative EEG figure
and the corresponding clinical and EEG description was
selected. For all the other identified cases, clinical and
 3
EEG Criteria for NCSE

neuroanatomic location of seizure activity. It denotes a

Table 1. Search strategies used for data range of conditions in which EEG seizure activity is
acquisition in PubMed prolonged and results in typical clinical nonconvulsive
Search syndromes.
Life period Search terms results (n) The following criteria were used for the diagnosis of
Neonatal and infantile ‘‘Nonconvulsive status 287 NCSE in early life as proposed in the section ‘‘Diagnosis
epilepsy syndromes epilepticus [title]’’ of NCSE in children’’ of the Oxford conference report in
‘‘West syndrome [title]’’ 378 2005 (Walker et al., 2005). Therefore, a continuous or vir-
‘‘Ohtahara syndrome [title]’’ 39
‘‘Dravet syndrome [title]’’ 106
tually continuous dysrhythmia or paroxysmal activity on
EEG is necessary. Furthermore, a continuous, abnormal
Childhood epilepsy ‘‘Panayiotopoulos 68 electrical dysrhythmia may occur on EEG and be difficult
syndromes syndrome [title]’’
‘‘Ring chromosome 15
to equate with the clinical state. Such electrical status that
20 syndrome [title]’’ occurs every time the child goes to sleep is seen in Lan-
‘‘Angelman syndrome [title]’’ 465 dau-Kleffner syndrome and some cases of Lennox-Gas-
‘‘Rett syndrome [title]’’ 1,325 taut syndrome. These continuous dysrhythmias may be
‘‘Myoclonic astatic epilepsy [title]’’ 36 acute or chronic.
‘‘Electrical Status epilepticus [title]’’ 52
‘‘Landau-Kleffner syndrome [title]’’ 190
The diagnosis of NCSE ideally must consist of a combi-
nation of clinical and EEG features. Therefore, the follow-
Epilepsy syndromes in ‘‘Lennox-Gastaut syndrome [title]’’ 274 ing four clinical and electroencephalographic criteria for
childhood and ‘‘Absence status epilepticus [title]’’ 22
adulthood ‘‘Tonic status epilepticus [title]’’ 7
the diagnosis of NCSE in early life were used:
1 Clear clinical change in behavior (manifested as
Epilepsy syndromes in ‘‘Limbic status epilepticus [title]’’ 33 changes in cognition, memory, arousal affect, ataxia,
adulthood and ‘‘Complex partial status 91
late adulthood epilepticus [title]’’
motor learning, and motor behavior) that lasted at least
‘‘Late-onset nonconvulsive 52 30 min. The word ‘‘clear’’ in the context of NCSE
status epilepticus’’ implies that an adequate description of behavior before
‘‘Late-life nonconvulsive 3 the onset of NCSE was available for comparison and
status epilepticus’’ the time of onset could have been defined, given that
‘‘Coma nonconvulsive 367
status epilepticus’’
the onset can be gradual and the duration of the NCSE
‘‘Epileptic psychosis [title]’’ 47 prolonged.
‘‘Drug-induced status epilepticus’’ 65 2 There must have been confirmation by clinical or neu-
‘‘Metabolic status epilepticus’’ 406 ropsychological examination of a clinical change.
3 Continuous or virtually continuous paroxysmal epi-
sodes must have been present on EEG.
EEG descriptions are provided. The quality of images var- 4 Continuous major seizures either tonic or clonic must
ied depending on the resolutions of the image sources. have been absent.
‘‘Boundary syndromes’’ were defined as conditions in All of the above criteria had to be fulfilled for the
which it was not clear to what extent the symptoms were diagnosis of NCSE in early life. A clinical response to
due to NCSE (Shorvon, 1994). We classified ‘‘boundary anticonvulsant medication such as intravenous/oral ben-
syndromes’’ into the three following categories: (1) myo- zodiazepine with simultaneous improvement in the
clonic status epilepticus in coma in the context of acute EEG and clinical symptoms added further support to
severe brain injury; (2) epileptic psychosis and behavior the diagnosis if positive, but did not exclude the diag-
disturbance; and (3) confusional states with epileptiform nosis if negative as proposed by Livingston & Brown
EEG changes (drug-induced or metabolic). In each, (1987).
patients had clinical nonconvulsive symptoms with ongo- For NCSE in adults and late adulthood, the definition
ing seizure activity on the EEG interpreted as NCSE. from the Oxford conference on NCSE (Walker et al., 2005)
Consensus on whether the EEG patterns and clinical was used as follows: (1) The diagnosis of NCSE was depen-
syndromic descriptions were consistent with NCSE was dent primarily on the presence of electrographic seizure
reached after a second critical review. The EEG criteria activity. This allowed the inclusion, within the rubric of
for NCSE are presented below. NCSE, of a range of ‘‘boundary conditions’’ in which such
activity occurred but in which there were no obvious clini-
Criteria for NCSE cal ‘‘seizures.’’ (2) Electrographic seizure activity can take
NCSE in early life can be viewed as an epileptic various forms, some of which clearly denote NCSE (clear-
response influenced and configured by cerebral develop- cut criteria) and some of which are less easy to interpret and
ment and integrity, the presence or absence of encephalop- probably denote NCSE only in some cases (equivocal crite-
athy, the underlying epilepsy syndrome, and the ria). The six ‘‘clear-cut’’ criteria included:
Epilepsia, 53(Suppl. 3):1–51, 2012
doi: 10.1111/j.1528-1167.2012.03593.x
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R. Sutter and P. W. Kaplan

Table 2. NCSE etiology or clinical context, forms, and response to treatment

Etiology or clinical Response to
Syndrome context Clinical form treatment or prognosis
NCSE in the neonatal and infantile epilepsy syndromes
West syndrome Various Infantile spasms with periods of Poor
NCSE with no clinical signs of
ongoing epileptic activity
Ohtahara syndrome Various Tonic spasms Poor
Severe myoclonic epilepsy of infancy (Dravet syndrome) Genetic Nonspecific Poor
NCSE in other forms of neonatal or infantile epilepsy Various Nonspecific Various
NCSE in childhood
Early onset benign childhood occipital epilepsy Idiopathic Autonomic status epilepticus Excellent
(Panayiotopoulos syndrome)
NCSE in other forms of childhood epileptic Various, usually Atypical absence and other Generally poor
encephalopathies, syndromes, and etiologies, (e.g., ring genetic or nonspecific forms
chromosome 20, Angelman syndrome, myoclonic–astatic cryptogenic
epilepsy, other childhood myoclonic encephalopathies)
Electrical status epilepticus in slow-wave sleep Various, usually No clinical signs but ongoing NCSE usually remits
cryptogenic electrographic activity in sleep but may leave
cognitive deficits
Landau-Kleffner syndrome Various, usually Clinical correlate of NCSE usually remits
cryptogenic electrographic activity is but may leave
severe speech disturbance cognitive deficits
NCSE in adulthood (and childhood) with epileptic
encephalopathy
NCSE in Lennox-Gastaut syndrome Various, often Atypical absence status Poor
cryptogenic epilepticus and tonic status
epilepticus
NCSE in other forms of disrupted cerebral development Various, often Various Variable
(cryptogenic or symptomatic) cryptogenic
NCSE in adulthood (and childhood) without epileptic
encephalopathy
Typical absence status epilepticus Idiopathic Generalized absence Excellent
generalized
epilepsy
Complex partial status epilepticus (limbic and Various - Complex partial Good
nonlimbic origin) symptomatic or
cryptogenic
NCSE in the postictal phase of TCSE Various Confusional state with psychiatric Good
features
Subtle status epilepticus Various Coma with small irregular Variable
myoclonic jerks
Aura continua Various - Simple partial (sensory, special Good
symptomatic or sensory, cognitive)
cryptogenic

NCSE in late adulthood

De novo absence status epilepticus Psychotropic drug Generalized absence Excellent
withdrawal or
idiopathic
generalized
epilepsy
Boundary syndromes
Coma with epileptiform EEG changes
Epileptic behavioral disturbance or psychosis
Drug-induced or metabolic confusional state with
epileptiform EEG changes

Adapted from the revised classifications of status epilepticus in children and adults according to Shorvon (1994).
NCSE, nonconvulsive status epilepticus; EEG, electroencephalography.

Epilepsia, 53(Suppl. 3):1–51, 2012

doi: 10.1111/j.1528-1167.2012.03593.x

1 Frequent or continuous focal electrographic seizures,
with ictal patterns that wax and wane with change in
amplitude, frequency, and/or spatial distribution.
2 Frequent or continuous generalized spike-wave dis-
charges in patients without a previous history of epilep-
tic encephalopathy or epilepsy syndrome.
3 Frequent or continuous generalized spike-wave dis-
charges, which showed significant changes in intensity
or frequency (usually a faster frequency) when compared
to baseline EEG, in patients with an epileptic encepha-
lopathy or epilepsy syndrome.
4 PLEDs or BIPEDs that occurred in patients in coma in
the aftermath of a generalized tonic–clonic status epi-
lepticus (subtle status epilepticus).
5 EEG patterns that were less easy to interpret included:
Frequent or continuous EEG abnormalities (spikes,
sharp-waves, rhythmic slow activity, PLEDs, BIPEDs,
GPEDs, triphasic waves) in patients whose EEGs
showed no previous similar abnormalities, in the
context of acute cerebral damage (e.g., anoxic brain
damage, infection, trauma).
6 Frequent or continuous generalized EEG abnormalities
in patients with epileptic encephalopathies in whom
similar interictal EEG patterns were seen, but in whom
clinical symptoms were suggestive of NCSE.
Categories 3 and 6 reflect the problem of deciding the
significance of spike-wave discharges in the setting of epi-
leptic encephalopathy (e.g., Lennox-Gastaut syndrome) in
which the ictal and interictal EEG patterns may be very
similar. The differentiation of the two is problematic. Cat-
egory 5 reflects the difficulty of differentiating patterns of
epileptic discharges that may lie along an ictal–interictal
continuum.
Results
From a total of 4,328 search results, 125 cases with
clinical descriptions and EEG patterns met the criteria
for NCSE. For each syndrome, one representative EEG
pattern is presented and reports from all identified EEG
patterns are compounded and indexed separately
(Figs. 1–43). We note that the quality of the EEG fig-
ures may be limited. This arises from the literature-
based source of the images.
A synthesis of the significant EEG morphologies and
evolutions of the individual NCSE syndromes arranged
according to the classification of NCSE syndromes of
Shorvon (1) is provided in Table 3.
Discussion
To our knowledge, this is the first study analyzing
and assembling the clinical and EEG patterns of
5
EEG Criteria for NCSE
NCSE from a syndromic perspective. An unambigu-
ous definition of NCSE that adequately covers all
types including ‘‘boundary syndromes’’ remains elu-
sive. There are several conditions in which there is
good evidence of ongoing electrographic epileptiform
activity but in which the clinical symptoms are not
conventionally considered to be epileptic. The defini-
tion proposed and accepted during the Oxford confer-
ence on NCSE (Walker et al., 2005) defines NCSE
as a range of conditions in which electrographic sei-
zure activity is prolonged and results in nonconvul-
sive clinical symptoms. This derives from an earlier
definition (Shorvon, 1994). Some points are worth
emphasizing. (1) The definition is dependent primarily
on the presence of electrographic seizure activity.
This allows the inclusion, within the rubric of NCSE,
of a range of ‘‘boundary conditions’’ as described
above. In addition, (2) electrographic seizure activity
can take various forms. Some segments of EEG pat-
terns that were presented in the reviewed reports did
not qualify for NCSE, although the clinical descrip-
tions were suggestive of status and were excluded
from this study. The reasons for exclusion were
absence of epileptic elements, such as lack of rhyth-
mic activity, lack of progression, and no waxing and
waning of ictal patterns (i.e., no change in amplitude,
frequency, and/or spatial distribution). This may be
explained by the fact that the published EEG excerpts
mostly show no more than 10–20 s of recording,
whereas the cited changes sometimes may develop
over much more than just a few seconds. On study
of the illustrated electrographic NCSE patterns, partic-
ular elements were identified that were seen in most
syndromes, but were not always part of the current
suggested criteria for NCSE (Table 3). Because these
elements may contribute substantially to recognition
and diagnosis of the corresponding NCSE syndromes,
we suggest adding them to the current criteria.
Summary
This study identifies and provides the various EEG pat-
terns seen in NCSE from a syndromic perspective. In addi-
tion, it explores borderline patterns and associations with
seizures, and it provides clinical and illustrative electroen-
cephalographic descriptions that enable the clinicians to
approach and categorize NCSE within the context of spe-
cific syndromes with clinical features and subtypes, rather
than using previous distinctions into complex partial
(focal) and absence (generalized) subtypes. It is hoped that
this compendium will help in moderating semiologic bor-
derline disputes on NCSE.
Epilepsia, 53(Suppl. 3):1–51, 2012
doi: 10.1111/j.1528-1167.2012.03593.x
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R. Sutter and P. W. Kaplan
Table 3. Prominent electrographic elements and EEG patterns for NCSE syndromes
Syndrome
NCSE in the neonatal and infantile
epilepsy syndromes
West syndrome
Ohtahara syndrome
Severe myoclonic epilepsy
of infancy (Dravet syndrome)
NCSE in other forms of neonatal or
infantile epilepsy
NCSE in childhood
Early-onset benign childhood occipital
epilepsy (Panayiotopoulos syndrome)
NCSE in other forms of childhood
epileptic encephalopathies, syndromes,
and etiologies
Electrical status epilepticus in
slow-wave sleep
Landau–Kleffner syndrome
NCSE in adulthood (and childhood)
with epileptic encephalopathy
NCSE in Lennox–Gastaut syndrome
NCSE in other forms of disrupted cerebral Various forms as defined from the Oxford conference on NCSE (Walker et al., 2005) (Figs 16 and 17)
development (cryptogenic or symptomatic)
Epilepsia, 53(Suppl. 3):1–51, 2012
doi: 10.1111/j.1528-1167.2012.03593.x
Prominent elements or sequential arrangements
Generalized continuous or waxing and waning high-voltage polymorphic slow wave
discharges (>200 lV) with interspersed multifocal, irregular spikes and sharp waves
usually followed by voltage attenuation and irregular 2–4 Hz slow wave discharges.
There may be periods of suppression of background activity or a continuous
burst-suppression pattern (Fig. 1A–F)
Continuous burst-suppression pattern with repetitive bursts of high-voltage slow waves
(200 to >400 lV) interspersed with multifocal, irregular spikes followed by periods of near
isoelectric suppression (Fig. 2A,B)
Continuous or waxing and waning, periodic or pseudoperiodic frontal or frontotemporal spikes,
which may be followed by slow waves in the awake tracing. Spikes tend to be triphasic instead
of biphasic and subclinical discharges are slower than in Lennox–Gastaut syndrome.
There are no polyspikes in sleep, and discharges that mimic tonic discharges show no
recruiting pattern. No clinical tonic or electromyographic features on video EEG awake and
sleep records. Usually background activity is slow and/or suppressed (Fig. 3)
Various forms as defined from the Oxford conference on NCSE (Walker et al., 2005) (Figs. 4 and 5).
Waxing and waning low-voltage fast and rhythmic epileptic activity of 1–2.5 Hz predominantly
in the occipital regions, increasing in voltage (>200 lV) and decreasing in frequency with
rapid bilateral frontotemporal spreading that is followed by rhythmic spike-and-wave discharges
with occipital predominance. Usually background activity is slow and desynchronized (Fig. 6A–E)
Ring chromosome 20 syndrome: Generalized continuous or waxing and waning repetitive irregular
spikes with frontal predominance, followed by rhythmic or irregular high-amplitude slow waves
at 3–4 Hz with interspersed multifocal, irregular spikes. Usually the frequency of the
spike-and-wave discharges increases, and the slow waves can get polymorphic. Usually
background activity is slow and/or suppressed (Fig. 7A–C).
Angelman syndrome: Generalized continuous or waxing and waning rhythmic sharp wave
discharges with frontocentral predominance. Usually background activity is slow and/or
suppressed (Fig. 8A,B).
Rett syndrome: Continuous or waxing and waning unilateral, multifocal or generalized
spikes usually during 50% of slow wave sleep. Usually background activity is disorganized and
desynchronized (Fig. 9A–D).
Myoclonic-astatic epilepsy syndrome: Generalized continuous or waxing and waning slow wave
discharges of 2–3 Hz with interspersed multifocal, irregular spikes, polyspikes, and slow wave
complexes. Electromyographic channels show multifocal, erratic myoclonic jerks on a
background of mild tonic contraction. The ictal activity then may blend into a burst-suppression
pattern (Fig. 10)
Generalized continuous rhythmic or irregular spike-and-wave discharges of up to 3 Hz during
slow-wave sleep with a frontocentral, or posterotemporal predominance. Usually background
activity is slow and/or suppressed (Fig. 12)
Focal, multifocal or generalized continuous or nearly continuous repetitive high-voltage spike
or spike-and-wave discharges, which are activated in slow-wave sleep. The epileptic activity
usually involves the dominant temporal area. Usually background activity is slow and/or
suppressed (Fig. 13)
Atypical absence status epilepticus: Generalized continuous or waxing and waning irregular
2–2.5 Hz spike and polyspike-slow-wave discharges predominantly frontotemporal and
usually with normal background activity (Fig. 14A,B).
Tonic status epilepticus: Generalized repetitive runs of polyspikes at high frequencies
of 16–20 Hz, beginning with low-voltage and increasing high-voltage (100 lV) and a
10 Hz recruiting rhythm of high-voltage from onset. The runs of polyspikes can be interspersed
with slow waves. Background activity may be disorganized and desynchronized (Fig. 15)
Continued

Syndrome
NCSE in adulthood (and childhood) without
epileptic encephalopathy
Typical absence status epilepticus
Complex partial status epilepticus
(limbic and nonlimbic origin)
NCSE in the postictal phase of TCSE
Subtle status epilepticus
Aura continua
NCSE in late adult life
De novo absence status epilepticus
Boundary syndromes
Coma with epileptiform EEG changes
Epileptic behavioral disturbance or
psychosis
Drug-induced or metabolic confusional
state with epileptiform EEG changes
NCSE, nonconvulsive status epilepticus; PLEDSs, periodic lateralized epileptiform discharges; GPEDs, generalized periodic epi-
leptiform discharges.
7
EEG Criteria for NCSE
Table 3. Continued.
Prominent elements or sequential arrangements
Generalized continuous or waxing and waning rhythmic 3–4 Hz spike and polyspike-slow-wave
discharges predominantly anterior and usually with normal background activity (Fig. 18)
Limbic: Continuous or waxing and waning periodic or pseudoperiodic sharp wave discharges
interspersed with low-voltage spikes and polyspike-and-waves with uni- or bilateral frontocentral
predominance and usually generalized slow and/or suppressed background activity. Waxing and
waning rhythmic delta activity, only (Fig. 19).
Nonlimbic: Continuous or waxing and waning periodic or pseudoperiodic sharp waves and
spike-and-wave discharges with unilateral, focal onset and frequent seizure progression into
generalized sharp wave and slow, and/or suppressed wave discharges usually with frontal
predominance and generalized slow background activity (Fig. 20)
Generalized or partial continuous or waxing and waning periodic epileptic discharges that resemble
PLEDs or GPEDs with a frontal predominance and usually generalized slow background
activity (Fig. 21A,B)
Generalized continuous or waxing and waning periodic epileptic discharges resembling GPEDs
that may be interrupted by short periods with nearly isoelectric suppression of 3–5 s.
Usually background activity is slow and/or suppressed (Fig. 22)
Unilateral continuous or waxing and waning rhythmic spike-and-wave or high-voltage slow-wave
discharges (>200 lV) usually with generalized slow and/or suppressed background
activity (Figs. 23–30)
Generalized continuous or waxing and waning rhythmic 3–4 Hz spike, polyspike-slow-wave
discharges predominantly anterior and usually with normal background activity (Fig. 31)
Generalized waxing and waning periodic epileptic discharges that resemble GPEDs with periods of
nearly isoelectric suppression. Usually background activity is slow and/or suppressed (Fig. 34)
Various forms as defined from the Oxford conference on NCSE (Walker et al., 2005) (Figs. 36–38)
Various forms as defined from the Oxford conference on NCSE (Walker et al., 2005) (Figs. 39–43)
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R. Sutter and P. W. Kaplan

Electroencephalographic Illustrations
and Clinical Descriptions
Index Page
1. NCSE occurring in the neonatal and infantile epilepsy syndromes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10
1a. NCSE in West syndrome (Blitz-Nick-Salaam, infantile spasms) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10
1b. NCSE in Ohtahara syndrome (early infantile epileptic encephalopathy with suppression-bursts). . . . . . . . . . . . . . 12
1c. NCSE in severe myoclonic encephalopathy of infancy (Dravet syndrome) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
1d. NCSE in other forms of neonatal or infantile epilepsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14
(i) Ring chromosome 14 syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14
(ii) Systemic lupus erythematosus with encephalopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14
2. NCSE occurring only in childhood . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14
2a. NCSE in early onset benign childhood occipital epilepsy (Panayiotopoulos syndrome) . . . . . . . . . . . . . . . . . . . . . . 14
2b. NCSE in other forms of childhood epileptic encephalopathies, syndromes, and etiologies. . . . . . . . . . . . . . . . . . . . 16
(i) Ring chromosome 20 syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16
(ii) Angelman syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17
(iii) Rett syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18
(iv) Myoclonic–astatic epilepsy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18
(v) NCSE in other myoclonic epilepsies in childhood (i.e., Lafora body disease) . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18
2c. NCSE in electrical status epilepticus in slow-wave sleep (ESES) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20
2d. NCSE in Landau-Kleffner syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
3. NCSE occurring in both childhood and adulthood . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
With epileptic encephalopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
3a. NCSE in the Lennox-Gastaut syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
(i) Atypical absence status epilepticus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
(ii) Tonic status epilepticus. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22
3b. Other forms of NCSE in patients with learning disability or disturbed cerebral development (cryptogenic or
symptomatic) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25
Without epileptic encephalopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26
3c. Typical absence status epilepticus in idiopathic generalized epilepsy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26
3d. Complex partial status epilepticus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26
(i) Limbic status epilepticus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26
(ii) Nonlimbic complex partial status epilepticus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28
3e. NCSE in the postictal phase of tonic–clonic status epilepticus (TCSE) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30
3f. Subtle status epilepticus (myoclonic status after convulsive status epilepticus). . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30
3g. Aura continua . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30
(i) Sensory symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30
(ii) Special sensory symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32
(iii) Autonomic symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32
(iv) Cognitive symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34
4. NCSE in late adulthood . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35
4a. De novo absence status epilepticus of late onset . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35
4b. Other forms of NCSE in late adulthood . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 37

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EEG Criteria for NCSE

5. Boundary syndromes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39
5a. Coma with epileptiform EEG changes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39
(i) Cryptogenic encephalitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39
(ii) Hypoxic encephalopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39
5b. Epileptic behavioral disturbance or psychosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41
5c. Drug-induced or metabolic confusional state with epileptiform EEG changes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45
(i) Drug-induced confusional state with epileptiform EEG changes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45
(ii) Metabolic confusional state with epileptiform EEG changes. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 49

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R. Sutter and P. W. Kaplan

8 months, Figure 1C,D at the age of 1 year and 6 months,

NCSE Occurring in the
Neonatal and Infantile Epilepsy
Syndromes
1a. NCSE in West syndrome (Blitz-Nick-Salaam,
infantile spasms)
Case 1
Clinical/EEG description (Saito et al., 2010): A girl
known for having West syndrome since the age of
3 months. EEG during wakefulness (Fig. 1A,C–E) and
sleep (Fig. 1B,F). Figure 1A,B were recorded at the age of
and Figure 1E,F at the age of 1 year and 8 months. Wide-
spread high-voltage slow waves, spikes, and sharp waves
are observed at the age of 8 months, particularly during
sleep (Fig. 1A,B). In Figure 1C, diffuse epileptiform dis-
charges occupy the tracing, when the patient showed pro-
longed unresponsiveness without convulsive motions.
The mother’s voice calling the name of patient (Fig. 1D;
arrow) elicits a right-sided predominantly tonic seizure
lasting several seconds. The epileptiform discharges are
relatively localized to the right posterior areas in
Figure 1E,F.

Figure 1.
(A–F) NCSE in West syndrome (Saito et al., 2010). Calibration: Sensitivity not known.
Epilepsia ILAE

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Case 2
Clinical/EEG description (Coulter, 1986): A 3-year
6-month-old boy with known West syndrome showed
repetitive flexion of the neck and hips every 2–3 s dur-
ing status epilepticus. He raised his arms, and then
relaxed until the next spasm occurred. On some occa-
sions his eyes were deviated to the right and the right
leg jerked more than the left, but flexion spasms of the
head and trunk were always present. Diffuse high-volt-
age bursts of polymorphic slow waves with interspersed
spikes followed by voltage attenuation; single or serial
sharp waves followed by a high-voltage slow waves
with interspersed spikes and voltage attenuation; and
sharp and slow waves followed by irregular 2–4 Hz
Figure 2.
(A,B) NCSE in Ohtahara
syndrome (Saneto & Sotero
de Menezes, 2007).
Calibration: 1 s per
horizontal unit; 100 lV per
B
vertical unit.
Epilepsia ILAE
11
EEG Criteria for NCSE
activity. Occasionally lateralized it was also at times
seen diffusely. On many occasions, the voltage attenua-
tion of one spasm was terminated by the onset of the
next spasm. Scattered multifocal spikes, diffuse spikes
and slow waves, and bursts of diffuse voltage attenua-
tion persisted after status epilepticus.
Although EEG resembled a burst-suppression pattern at
these times, it could be distinguished because of the docu-
mented clinical spasms that accompanied the periods of
voltage attenuation. On other occasions, an individual
spasm was followed by a few seconds of disorganized
slowing and multifocal spikes (hypsarrhythmia) before
the next clinical spasm, which clearly distinguished the
EEG from a burst-suppression pattern.
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R. Sutter and P. W. Kaplan

1b. NCSE in Ohtahara syndrome (early infantile consisting of a brief arrest of respiration. Tonic
epileptic encephalopathy with suppression-bursts) spasms were continuous on awakening and during
wakefulness. In wakefulness there was a burst-suppres-
Case 1
sion pattern with asymmetric discharge, more evident
Clinical/EEG description (Saneto & Sotero de Mene-
over the left side. There was tonic contraction of both
zes, 2007): All EEG recordings from the first 5 years of a
deltoid muscles for the whole discharge and discontin-
girl with Ohtahara syndrome showed a burst-suppression
ued at the end of it. The discharge was spikier over
pattern consisting of high-amplitude bursts, 400–
the left hemi-megalencephalic hemisphere.
2,000 lV, slow waves intermixed with multifocal spikes
followed by near isoelectric suppression during wake and
Case 3
sleep states (Fig. 2A). A representative of burst-suppres-
Clinical/EEG description (Fusco et al., 2001): A new-
sion pattern is shown when the patient was approximately
born girl showed persistent burst-suppression pattern.
1 year old. This epoch (Fig. 2A) shows high-amplitude
Each burst was associated with a complex tonic contrac-
bursts of slow delta waves intermixed with multifocal
tion involving the trunk, the arms, and the neck. Repetitive
spikes followed by near isoelectric suppression. A repre-
and at times periodic tonic seizures were associated with
sentative of burst-suppression pattern is presented when
bursts of high-voltage generalized polymorphic poly-
the patient was approximately 5 years old (Fig. 2B). This
spikes with frontocentral dominance. Between the bursts
epoch shows high-amplitude bursts of slow delta waves
there was suppression.
with reduced multifocal spikes compared with the EEG
pattern seen at a younger age. This high-amplitude slow-
Case 4
ing is again followed by near isoelectric suppression as
Clinical/EEG description (Al-Futaisi et al., 2005): A
seen in the EEG from a younger age.
girl with Ohtahara syndrome had suppression periods
>40 s between high-amplitude 1-mV spike bursts lasting
Case 2 up to 5 s. Low-amplitude (30-lV) slow rhythmic (0.3–
Clinical/EEG description (Fusco et al., 2001): A 0.7 Hz) sharp and slow waves gradually built up over the
53-day-old female infant had alternation between right temporocentroparietal regions (T6, C4, and P4) dur-
wakefulness with eyes open and a state of apparent ing the period of suppression period. These rhythmic slow
sleep with eyes closed, regular respiration, and poor sharp waves and slow waves over the right hemisphere
response to external stimuli. During sleep there was a persisted during the entire EEG without clinical signs.
subtle sign concurrent with an EEG burst discharge, High-amplitude bursts were off-scale at this sensitivity.

Figure 3.
NCSE in Dravet syndrome (Nabbout et al., 2008). Calibration: 1 s per horizontal unit; 100 lV per vertical unit.
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1c. NCSE in severe myoclonic encephalopathy of
infancy (Dravet syndrome)
Case 1
Clinical/EEG description (Nabbout et al., 2008): An
EEG pattern in the adolescent course. It combined fron-
tal spikes (as couplet or triplet), which may have been
followed by slow waves on the awake tracing. Sleep
may have been activated 5–10 s of subclinical dis-
charges of 8–9 Hz (Fig. 3). In Dravet syndrome, (1)
spikes tend to be triphasic instead of biphasic, (2) sub-
clinical discharges are slower (at 8–9 Hz) than in Len-
nox-Gastaut syndrome, (3) there are no polyspikes in
sleep, and (4) discharges that mimic tonic discharges
show no recruiting pattern and no clinical tonic or elec-
tromyography features on video-EEG awaking and
sleeping records.
There were no polyspikes in sleep, and discharges that
mimic tonic discharges show no recruiting pattern and no
Figure 4.
NCSE in ring chromosome 14 syndrome (Giovannini et al., 2010). Calibration: 1 s per horizontal unit; 50 lV per
vertical unit.
Epilepsia ILAE
13
EEG Criteria for NCSE
clinical tonic or electromyography features on video-EEG
awaking and sleeping records.
Case 2
Clinical/EEG description (Moseley et al., 2011): A
10-month-old boy developed continuous right facial
twitching, left upper extremity tonic posturing, and
accompanying oxygen desaturations. EEG revealed con-
tinuous generalized polyphasic periodic as well as right
frontotemporal and left temporal epileptiform discharges.
Case 3
Clinical/EEG description (Wakai et al., 1996): lctal
EEG of a 1-year 7-month-old boy disclosed persisting irreg-
ular spike-and-wave complexes in the left hemisphere, pre-
dominantly in the occipital and occipitotemporal areas.
After administration of diazepam and midazolam, frequency
of the spike-and-wave complexes gradually decreased,
although the occipital spikes remained.
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R. Sutter and P. W. Kaplan

1d. NCSE in other forms of neonatal or infantile
epilepsy
(i) Ring chromosome 14 syndrome
Case 1
Clinical/EEG description (Giovannini et al., 2010):
At the age of 15 months this boy had afebrile repeti-
tive seizures during sleep with staring, loss of con-
sciousness, breathing difficulties, and generalized
hypertonus, followed by four-limb clonias with a right
mild predominance, resembling status epilepticus. EEG
during seizure revealed bifrontal delta waves followed
by an abrupt, 3-s flattening of background activity
after which rhythmic anterior triphasic slow waves
with intermingled spikes appeared. This was followed
by left frontotemporal fast rhythmic spikes with rapid
diffusion to the right frontotemporal regions. This
activity rapidly generalized with prolonged irregular
spike-waves, mostly 2–2.5 complexes/s, and stopped
abruptly after 2 min with a diffuse flattening, followed
by the reappearance of background rhythms. EEG
recording showed bilateral frontal high-voltage contin-
uous delta activity (Fig. 4; above) concomitant with a
behavior characterized by confusion, hypotonia,
alternating gestural automatisms, and vegetative symp-
toms. Continuous delta activity was resolved after
intravenous bolus of phenytoin (Fig. 4; below).
(ii) Systemic lupus erythematosus with encephalopathy
Case 1
Clinical/EEG description (Korff & Nordli, 2007): An
11-year-old girl with systemic lupus erythematosus,
seizures, and encephalopathy. EEG revealed PLEDs (pari-
etal and posterior temporal irregular pseudorhythmic
polyspike-and-wave discharges) with left-hemisphere pre-
dominance, but with a more diffuse manifestation (Fig. 5).
2. NCSE Occurring Only in
Childhood
2a. NCSE in early onset benign childhood occipital
epilepsy (Panayiotopoulos syndrome)
Case 1
Clinical/EEG description (Specchio et al., 2010):
Ictal recording (video-EEG) of a 7-year-old girl
(Fig. 6A). The ictal discharge starts in sleep over the left
occipital area with low-voltage fast activity, increasing

Figure 5.
NCSE in systemic lupus erythematosus with encephalopathy (Korff & Nordli, 2007). Calibration: 1 s between gray
vertical lines; 100 lV between horizontal lines.
Epilepsia ILAE

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Figure 6.
(A–E) NCSE in Panayiotopoulos syndrome (Specchio et al., 2010). Calibration: Sensitivity not known.
Epilepsia ILAE
in amplitude and decreasing in frequency, and quickly
involving temporal and parietal areas. This epileptic
activity persists over the right occipital area as indepen-
dent spike-and-wave activity lasting 22 s. After 50 s,
eyes are open and tachycardia is evident. (Fig. 6B) At
3 min, discharge is bilateral, and there are more pro-
nounced slow waves over left posterior area (Fig. 6C).
Next there is eye deviation and vomiting with EEG
characterized by 2.5-Hz spike-and-waves over the left
occipital region and delta waves over the frontal and
temporal areas (Fig. 6D,E). After 7 min, the patient has
complete loss of consciousness (Fig. 6D), after which
the seizure ends (Fig. 6E); slow waves are evident and
involve mainly the left hemisphere.
Case 2
Clinical/EEG description (Specchio et al., 2010):
Ictal recording of a boy 4 years 6 months of age. Sei-
zure onset is characterized by subclinical low-voltage
fast activity over the right occipital region during
non–rapid eye movement (non-REM) phase II sleep.
This activity persists over 3 min and child opens his
eyes; rhythmic spikes are also evident over the right
posterior region. After 4 min, wider epileptic activity,
increasing in amplitude and decreasing in frequency,
is evident over the right occipital area. The child is
unresponsive and the ictal discharges are more diffuse.
15
EEG Criteria for NCSE
After 11 min, over the occipital regions, high-voltage
spikes-and-waves are evident (1–2 Hz), and on the
bilateral frontal areas, there are high-amplitude rhyth-
mic sharp waves synchronous with the occipital activ-
ity. At this stage, both tachycardia and tachypnea are
evident on electrocardiography and airflow traces,
respectively. The patient also has some episodes of
retching. After 15 min, diazepam is administered intra-
venously and the epileptic activity becomes less rhyth-
mic. After 19 min, the seizure gradually ends with
slow sharp waves on the posterior regions of lower
voltage on the right side.
Case 3
Clinical/EEG description (Specchio et al., 2010):
Ictal EEG of a child showed right temporooccipital
spikes with delta waves over the left frontal area and
vertex during chewing automatisms and left eye devia-
tion. After 8 min, the activity persisted and tachycardia
was evident (150 beats/min). This was followed by
left-sided eyelid clonic jerks with persistent spike-and-
wave discharges, more pronounced over the right pos-
terior areas, which became more rhythmic a few min-
utes later during abdominal clonic jerks. Seizure ended
after midazolam was administered intravenously, and
delta waves have involved the right hemisphere with
higher amplitude over temporooccipital traces.
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R. Sutter and P. W. Kaplan

Figure 7.
(A–C) NCSE in ring chromosome 20 syndrome (Inoue et al., 1997). Calibration: 1 s per horizontal unit; 50 lV per
vertical unit (as shown in Fig. 13C).
Epilepsia ILAE

The seizure lasted 39 min. Verbal response was

2b. NCSE in other forms of childhood epileptic
encephalopathies, syndromes, and etiologies
(i) Ring chromosome 20 syndrome
Case 1
Clinical/EEG description (Inoue et al., 1997): A
14-year-old girl with repetitive spikes in the right
frontal region (Fig. 7A), followed by 3–4 Hz slow
waves and frontal dominant bilateral 3-Hz spike-and-
wave complexes (Fig. 7B). Spike-and-wave complexes
gradually lost the spike component with increasing
frequency and became polymorphous (Fig. 7C).
impaired to various degrees, ranging from simple
slowness, perseveration, and inappropriate utterance to
muteness.
Case 2
Clinical/EEG description (Inoue et al., 1997): A 21-
year-old woman evinced frontal dominant irregular high-
voltage slow waves with occasional spikes that lasted
40 min. The patient looked ‘‘indifferent and weary.’’
Verbal responses were short and often inappropriate.
Complex mental action such as calculation or thinking
was impossible.

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Case 3
Clinical/EEG description (Inoue et al., 1997): A 31-
year-old woman with seizures at the age of 7 years that
lasted up to 120 min. Frontal dominant slow-wave rhythm
gradually increasing in amplitude with slow waves and
spikes. The patient’s mental state fluctuated. She was mute
and motionless when high-voltage slow waves became
faster or were accompanied by spikes.
Case 4
Clinical/EEG description (Inoue et al., 1997): A 25-
year-old man with first seizures at the age of 11 years.
Irregular slow waves over the anterior region were
replaced by 3–4 Hz repetitive spikes or spike-and-
wave complexes, which then decreased in frequency.
The amplitude of the spikes and slow waves were
higher on the right side. He was mute and his move-
ments were slow.
Case 5
Clinical/EEG description (Jacobs et al., 2008): A
4-year-old boy with up to 30 seizures a day of four recog-
nizable types: (1) prolonged atypical absences; (2) tonic
Figure 8.
(A,B) NCSE in Angelman
syndrome (Weber, 2010).
Calibration: 1 s every four
vertical lines; sensitivity not B
known.
Epilepsia ILAE
17
EEG Criteria for NCSE
posturing of the arms, extension of the neck and head turn-
ing to the left; (3) nocturnal hypermotor seizures with
agitation; and (4) focal seizures with terror, visual halluci-
nations, and impaired consciousness. EEG changes during
prolonged absences consisted of generalized suppression,
2–3 Hz rhythmic slow waves and 1 Hz spike-and-slow-
wave activity over both hemispheres with some focality.
Variable EEG patterns with rhythmic slow waves and
spike and slow waves recorded focally, followed by low-
voltage fast activity during the tonic phase of the seizure.
(ii) Angelman syndrome
Case 1
Clinical/EEG description (Weber, 2010): A 7-year-
old boy with developmental delay was diagnosed for the
first time at the age of 6 months. At the age of 30 months
he had convulsive status epilepticus and at the age of 5
years he had atypical absences. He showed a change in his
behavior with reduced activity and eye contact. His readi-
ness to laugh was prominently reduced. EEG revealed
continuous epileptic discharge with high-voltage sharp
waves (Fig. 8A,B).
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R. Sutter and P. W. Kaplan
(iii) Rett syndrome
Case 1
Clinical/EEG description (Nissenkorn et al., 2010):
These patients with Rett syndrome showed evolution of
EEG epileptic pattern in Rett syndrome according to age:
Left central spikes during sleep in a 2-year-old girl
(Fig. 9A). Multifocal spikes and disorganized background
activity in a 3-year 6-month-old girl (Fig. 9B). Multifocal
and generalized epileptic activity during 50% of slow-
wave sleep in a 6-year-old patient (Fig. 9C). Generalized
epileptic activity during sleep that fulfills the criteria for
ESES (electric status epilepticus during slow sleep) at the
age of 7 years (Fig. 9D).
(iv) Myoclonic–astatic epilepsy
Case 1
Clinical/EEG description (Guerrini & Aicardi, 2003):
A 4-year 6-month-old boy with myoclonic status epilepti-
cus after carbamazepine. The child was unresponsive and
in a sort of ‘‘stupor’’ accompanied by multifocal and gen-
eralized myoclonic jerks. EEG showed diffuse slow waves
with intermingled multifocal, irregular spikes, and wave
complexes (Fig. 10; left). Electromyographic channels
showed multifocal, erratic myoclonic jerks on a back-
ground of mild tonic contraction. Figure 10 (right) shows
polygraphic recording performed 24 h after withdrawal of
A B
C D
Figure 9.
(A–D) NCSE in Rett syndrome (Nissenkorn et al., 2010). Calibration: 1 s per horizontal unit; 100 lV per vertical
unit.
Epilepsia ILAE
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carbamazepine in the same boy who was awake and
responsive. There was rhythmic background activity and
no epileptiform activity.
Case 2
Clinical/EEG description (Kobayashi et al., 2007):
EEG from a boy during a series of spasms with a burst-
suppression pattern during sleep with early myoclonic
encephalopathy. The ictal activity of spasms blends with
the postseries burst-suppression pattern. Myoclonus
occurs in association with EEG bursts during sleep.
(v) NCSE in other myoclonic epilepsies in childhood (i.e.,
Lafora body disease)
Case 1
Clinical/EEG description (Fernandez-Torre et al.,
2011): A 19-year-old woman with progressive myoclonus
epilepsy of Lafora type. A tactile stimulus (Fig. 11; black
arrow) elicited a burst of epileptiform discharges in keep-
ing with the definition of stimulus-induced rhythmic, peri-
odic, ictal discharges (SIRPIDs).
Case 2
Clinical/EEG description (Corkill & Hardie, 1999): A
15-year-old boy with eyelid myoclonia during eye closure.
Frequent multifocal, spontaneous, and action myoclonic
 19
EEG Criteria for NCSE

Figure 10.
NCSE in myoclonic–astatic epilepsy (Guerrini & Aicardi, 2003). Calibration: 1 s per horizontal unit; 100 lV per
vertical unit. Mas, masseter; Orb, oris and OO, orbicularis oris; Delt, deltoid; W Ext, wrist extensors; W Flex, wrist
flexors; APB, abductor pollicis brevis; Quad, quadriceps; L, left; R, right; AV, common average reference.
Epilepsia ILAE

Figure 11.
NCSE in other childhood myoclonic encephalopathies; Lafora body disease (Fernandez-Torre et al., 2011).
Calibration: 1 s per horizontal unit; 100 lV per vertical unit.
Epilepsia ILAE

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R. Sutter and P. W. Kaplan
jerks affected the upper limbs. EEG showed absence status
epilepticus with generalized polyspike-and-wave activity
with photosensitivity (at 10 Hz).
Case 3
Clinical/EEG description (Dhamija et al., 2011): A
10-month-old boy with myoclonic jerks. During myo-
clonic status epilepticus, EEG recording showed continu-
ous generalized epileptiform discharges associated with
body jerking, consistent with myoclonic status epilepticus.
EEG showed 1–2 Hz generalized spike-and-wave dis-
charges corresponding with myoclonic jerks. A longitudi-
nal bipolar EEG montage revealed maximal discharges
over bicentral parietooccipital regions.
2c. NCSE in electrical status epilepticus in slow-wave
sleep (ESES)
Case 1
Clinical/EEG description (Van Hirtum-Das et al.,
2006): A 5-year-old girl with receptive and expressive
language problems. In sleep: spike and sharp wave dis-
charges over the vertex, left frontoparietal, and right
central head regions independently (Fig. 12; before
Figure 12.
NCSE in ESES (Van Hirtum-Das et al., 2006). Calibration: 1 s between vertical lines; 100 lV per vertical unit.
Epilepsia ILAE
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subpial transection). These discharges occupied >85% of
stage III sleep.
Case 2
Clinical/EEG description (Tassinari et al., 2000): A
7-year-old boy during slow-wave sleep. EEG showed
continuous, diffuse 2.5-Hz spike-and-wave activity, of
higher amplitude on the left side. During REM sleep,
diffuse spike-and-wave discharges disappeared,
whereas focal left frontotemporal spikes without con-
tralateral diffusion reappeared.
Case 3
Clinical/EEG description (Coutelier et al., 2008): An
8-year-old girl with subtle seizures with eyelid myoclonia.
Sleep-EEG showed nearly continuous and bitemporal
spike-and-wave complexes, while in frontocentral areas,
only slow waves were recorded.
Case 4
Clinical/EEG description (Zhang et al., 2010): A
5-year-old boy had frequent epileptiform discharges while
he was awake. During sleep the EEG revealed continuous
spike-and-wave discharges.

2d. NCSE in Landau-Kleffner syndrome
Case 1
Clinical/EEG description (Nickels & Wirrell, 2008):
A 6-year-old boy with Landau-Kleffner syndrome had
markedly increased frequency of left centrotemporal peri-
odic spike and slow-wave complexes during sleep
(Fig. 13).
3. NCSE Occurring in Both
Childhood and Adulthood
With epileptic encephalopathy
3a. NCSE in the Lennox-Gastaut syndrome
(i) Atypical absence status epilepticus
Case 1
Clinical/EEG description (Livingston & Brown,
1987): A 7-year-old girl with prolonged and recurrent
febrile seizures and drop attacks. EEG showed rhythmic
and polymorphic diffuse delta activity of 1.5–2 Hz with-
out response to benzodiazepines (Fig. 14A,B).
Figure 13.
NCSE in Landau-Kleffner syndrome (Nickels & Wirrell, 2008). Calibration: 1 s between vertical lines; 30 lV/mm.
Epilepsia ILAE
21
EEG Criteria for NCSE
Case 2
Clinical/EEG description (Dravet et al., 1986): An 8-year-
old girl with constant confusion, isolated or generalized myo-
clonias, and drop attacks. Absence status with subcontinuous
slow waves, spike-and-waves and polyspike-and-waves.
Case 3
Clinical/EEG description (Ohtsuka et al., 1999): A 1-
year 10-month-old girl. At 3 years and 3 months of age,
she began to have atypical absences and brief head-nod-
ding seizures in addition to complex partial seizures.
These atypical absences and brief head-nodding seizures
appeared in a cluster and evolved into NCSE, during
which she became unresponsive with frequent head nod-
ding. EEG revealed multifocal spike-and-waves, which
were mainly bilateral midtemporal. The patient’s head
nodded repeatedly in a sitting position. There was a
decrease in response to external stimuli. Although the
electromyogram was not recorded on this EEG, the
patient’s mother activated the marker at the time of the
head-nodding seizures. On another occasion, no dis-
charges were detected by the electromyogram at the time
of the head-nodding seizures, which were associated with
diffuse spike-wave bursts.
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Figure 14.
(A,B) NCSE as atypical absence status epilepticus (Livingston & Brown, 1987). Calibration: 1 s per horizontal unit;
100 lV per vertical unit.
Epilepsia ILAE
Case 4
Clinical/EEG description (Ohtsuka et al., 1999): A 7-
year 3-month old girl with left-frontal spike-and-waves.
The patient had frequent head-nodding seizures in a sitting
position with a decrease in response to external stimuli.
Electromyogram showed that discharges from bilateral
trapezius muscles suddenly disappeared at the time of a
brief atonic seizure, which was time-locked by a diffuse
spike-and-wave burst.
Case 5
Clinical/EEG description (Ohtsuka et al., 1999): A 2-
year 2-month-old boy with frequent head-nodding sei-
zures in a sitting position with a decrease in response to
external stimuli. EEG showed relatively synchronous, dif-
fuse, 1.5–2 Hz slow spike-and-wave bursts (left-side dom-
inant) when the patient showed a decrease in response to
external stimuli.
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Case 6
Clinical/EEG description (Bauer & Trinka, 2010):
A 33-year-old woman with late onset Lennox-Gastaut
syndrome (onset at age 16 years). EEG 3 days after
start of an atypical absence status (obtundation and
occasional twitches) showed continuous generalized
sharp and slow waves at 2 Hz, dominant over the ante-
rior regions. The pattern also can be read as triphasic
waves.
(ii) Tonic status epilepticus
Case 1
Clinical/EEG description (Dravet et al., 1986): Tonic
status in a girl aged 11 years 7 months. Diffuse, fast
rhythms followed by a burst of high-voltage polyspikes
intermixed with slow waves (Fig. 15 top). Clinically: short
apnea, followed by superficial polypnea and tachycardia.

Figure 15.
Tonic status epilepticus (Dravet et al., 1986). Calibration: 1 s per horizontal unit; 100 lV per vertical unit.
Epilepsia ILAE
At the end, intensive muscular activity recorded on the left
muscles. Figure 15; bottom: A long seizure (55 s) involv-
ing principally the left hemisphere, with only anterior
rapid rhythms and slow waves on the right. No clinical
expression.
Case 2
Clinical/EEG description (Tassinari et al., 1972): A
child with state of confusion with frequent myoclonic
jerks, either massive, segmental or discrete. The EEG
showed nearly continuous diffuse spike-and-waves of
high amplitude and variable frequency, most marked
anteriorly. This was followed by absence status with
confusion and discrete myoclonic jerks. EEG showed
diffuse spike and polyspike-and-wave discharges. Spike
and waves persisted between seizures with confusion.
Before injection: Discharges of subcontinuous general-
ized spike and waves and polyspikes-and-waves, some-
times accompanied by repeated clonias recorded from
the right deltoid. A few seconds after intravenous injec-
tion of 10 mg of diazepam tonic seizures appeared with
an EEG characterized by rhythmic spikes, accompanied
by intense tonic contraction.
23
EEG Criteria for NCSE
Case 3
Clinical/EEG description (Tassinari et al., 1972):
A patient with apnea and bradycardia without motor mani-
festations, and on EEG fast spikes lasting 4–6 s. The
whole episode lasted 45 min. Before activation—bursts of
angular diffuse theta waves. After generalized tonic–clo-
nic seizures following intravenous injection of 500 mg of
pentylenetetrazol the EEG is characterized by diffuse,
continuous slow spike-and-waves appearing immediately
after the postcritical depression that followed the grand
mal seizure.
Case 4
Clinical/EEG description (Bittencourt & Richens,
1981): Several week-long episodes of minor status
occurred, in which this girl looked vacant, with occasional
twitching of the face and hands. She then had tonic sei-
zures with sleep or drowsiness. EEGs showed a slow
spike-and-wave abnormality, sometimes with atypical
absences and bursts of repetitive spikes. From the data
summarized it can be concluded that diazepam injected as
a bolus caused tonic status epilepticus with concomitant
bursts of repetitive spikes on the EEG.
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Figure 16.
(A,B) NCSE in learning disability or disturbed cerebral development (Dirik et al., 2006). Calibration: 1 s between
vertical prominent lines; sensitivity not known.
Epilepsia ILAE

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EEG record showed atypical spike-and-wave bursts,
the period varying between 2 and 3 Hz. Bursts of
repetitive spikes at 16–20 Hz and amplitudes of up to
100 lV were more prominent anteriorly and series of
bursts of repetitive spikes, intercalated with large
amplitude slow waves.
3b. Other forms of NCSE in patients with learning
disability or disturbed cerebral development
(cryptogenic or symptomatic)
Case 1
Clinical/EEG description (Dirik et al., 2006): An 18-
month-old boy with global neurodevelopmental delay and
Figure 17.
(A,B) NCSE in learning disability or disturbed cerebral development (Shin et al., 2011). Calibration in A: 1 s between
prominent vertical lines; 30 mV/mm. Calibration in B: 1 s between prominent vertical lines; 7 mV/mm.
Epilepsia ILAE
25
EEG Criteria for NCSE
decreased alertness. In Figure 16A EEG during sleep state
demonstrates generalized slow-wave discharges and some
spikes between slow-wave discharges. Figure 16B shows
disappearance of epileptiform activity 24 h after
intravenous valproic acid treatment; however, there is still
some slowing.
Case 2
Clinical/EEG description (Shin et al., 2011): A 7-
year-old girl with cerebral palsy and extensive bilateral
polymicrogyria. She has generalized 1–2 Hz spike and
waves over several hours while she is taking valproate,
levetiracetam, and clobazam (Fig. 17A). Figure 17B
shows slowing before complete remission.
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R. Sutter and P. W. Kaplan
Without epileptic encephalopathy
3c. Typical absence status epilepticus in idiopathic
generalized epilepsy
Case 1
Clinical/EEG description (Cascino, 1993): A patient with
typical absence status epilepticus. EEG showed generalized
continuous 3-Hz spike-and-wave activity that is anterior pre-
dominant during typical absence status epilepticus (Fig. 18).
Case 2
Clinical/EEG description (Akman, 2010): In this
patient with typical absence status epilepticus EEG
showed irregular diffuse spike-and-wave discharges that
are sometimes synchronous with 3 Hz.
Case 3
Clinical/EEG description (Genton et al., 2008):
Patient appeared confused, slow, drowsy. EEG showed
almost continuous generalized spike-and-wave and poly-
spike-and-wave discharges at 3 Hz. Intravenous injection
of diazepam induced a transient effect, with patient
regaining full consciousness, and partially recalling the
event; concomitantly EEG showed a normal background
activity with only rare generalized polyspike-and-wave
discharges. After 15 min, SE reappeared with EEG show-
ing pseudo rhythmic bursts of spike-and-wave and polys-
pike-wave discharges.
Case 4
Clinical/EEG description (Genton et al., 2008): An
adult patient with absence status characterized by mild
impairment of consciousness and by motor impersistence.
EEG shows discontinuous short bursts of slow polyspike-
and-waves. When asked to close his eyes, the patient
rhythmically opened them concomitantly with the spike
component of the polyspike-and-wave complexes on the
EEG, despite the reiteration of the order.
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Case 5
Clinical/EEG description (Korff & Nordli, 2007): A
6-year-old child with unresponsiveness and subtle twitch-
ing of the corner of the mouth. EEG showed continuous
rhythmic generalized spike-and-wave discharges with
frontal predominance.
Case 6
Clinical/EEG description (Fernandez-Torre et al.,
2011): A 27-year-old woman known for having GTCS
with mutism and no other precipitating factors in her per-
sonal history. No further description available. EEG
showed presence of continuous polyspike-and-wave and
spike-and-wave complexes in keeping with the diagnosis
of typical absence status epilepticus.
3d. Complex partial status epilepticus
(i) Limbic status epilepticus
Case 1
Clinical/EEG description (Nahab et al., 2008): A 57-
year-old woman with a 2-month history of refractory sim-
ple partial seizures. She had right upper extremity clonic
movements that progressed to a GTCS. These developed
into frequent episodes of clonic right face, arm, and occa-
sionally leg movements without alteration of awareness.
Epilepsia partialis continua of unclear etiology was diag-
nosed. Seizure onset was seen over the left frontocentral
region. Frequent epileptiform discharges over the left
frontocentral region correlating with clinical seizure activ-
ity (Fig. 19).
Case 2
Clinical/EEG description (Kirkpatrick et al., 2011): A
19-year-old woman with behavioral problems, emotional
lability with religious verbalizations. She displayed
personality changes and bizarre behaviors, responding to
Figure 18.
Typical absence status
epilepticus in idiopathic
generalized epilepsy
(Cascino, 1993).
Calibration: 1 s per
horizontal unit; 100 lV per
vertical unit.
Epilepsia ILAE

Figure 19.
Limbic status epilepticus (Nahab et al., 2008). Calibration: 1 s between vertical lines; sensitivity not known.
Epilepsia ILAE
hallucinations and reacting aggressively. EEG showed
generalized rhythmic delta activity. The evolution of the
EEG pattern is difficult to appreciate based on one 10-s
epoch.
Case 3
Clinical/EEG description (Bayreuther et al., 2009):
This 25-year-old woman had unusual headache, auditory
hallucinations, and extreme anxiety, consistent with
panic attacks. She had fluctuating consciousness with
temporal and spatial disorientation and recurrent chew-
ing movements. Over a 4-week period she developed
complex abnormal movements, with stereotypic epi-
sodes of brief repetitive dystonic posturing of the left
hemiface and left upper limb, severe orofacial dyskine-
sias leading to injury of the lips, bruxism, hypersaliva-
tion, oromandibular dystonia with tongue protrusion,
and episodes of opisthotonus. During the first EEG, the
patient was confused with oroalimentary automatisms.
EEG showed right hemispheric 1-Hz rhythmic activity.
After intravenous diazepam, the patient became respon-
sive and EEG improved markedly with reappearance of
the alpha rhythm.
27
EEG Criteria for NCSE
Case 4
Clinical/EEG description (Espay et al., 2006): A
68-year-old woman with anti-Hu antibodies, rapidly
evolving impairment in consciousness, and EEG evidence
of lateralized pseudoperiodic sharp-wave discharges.
Ataxia and sensory neuropathy developed within the first
two weeks. EEG on admission showed predominantly
right pseudoperiodic sharp waves, spike-and-wave
complexes, and subsequent pseudoperiodic complexes.
Background was poorly organized with intermixed,
almost continuous, irregular and generalized sharp waves.
Case 5
Clinical/EEG description (Kaplan et al., 2012): A
51-year-old woman had a 10-month history of rapid
progressive dementia, partial seizures, sudden dystonic
movements, and hyponatremia. EEG showed waxing and
waning generalized 2–4 Hz rhythmic delta activity. After
5 mg of midazolam the patient began interacting.
Case 6
Clinical/EEG description (Kaplan et al., 2012): A 21-
year-old woman began having brief delusional thoughts
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R. Sutter and P. W. Kaplan
Figure 20.
Nonlimbic complex partial status epilepticus (Akman, 2010). Calibration: 1 s between prominent vertical lines;
sensitivity not known.
Epilepsia ILAE
with hallucinations, altering ‘‘between dreams and
reality,’’ that became persistent. Clinical examination
revealed unresponsiveness to voice, touch, or command,
and a right gaze preference with increased tone of the four
limbs without spontaneous movements. EEG showed
anterior rhythmic 2.5–3 Hz delta waves. Background was
suppressed, runs lasted >4 s, and there was no change with
noxious stimuli, indicating that this was not frontal inter-
mittent rhythmic delta activity (FIRDA).
(ii) Nonlimbic complex partial status epilepticus
Case 1
Clinical/EEG description (Akman, 2010): A 2-year-
old child with left-sided pachygyria presented with inter-
mittent brief habitual seizures. EEG shows continuous
focal seizure lateralized to the left hemisphere (Fig. 20).
Case 2
Clinical/EEG description (Stayman & Abou-Khalil,
2011): A 51-year-old woman with evolution of bizarre
behavior such as placing her cell phone in the microwave,
placing a glass tumbler into the blender while attempting
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to make herself a cocktail, and perseverative behaviors
such as repeating ‘‘channel 168’’ while manipulating the
television remote. She had deficits in recall, attention, and
judgment. EEG revealed irregular frontal delta activity
bilaterally.
Case 3
Clinical/EEG description (Sensoy et al., 2009): An 11-
month-old female infant was unconscious and responsive
to only painful stimuli at admission. Left central facial
paralysis was detected and she had no swallow reflex or gag
reflex. Brain magnetic resonance imaging (MRI) showed
lesions in both frontotemporal lobes. EEG showed persis-
tent epileptic activity at the second hospitalization.
Case 4
Clinical/EEG description (Cascino, 1993): A patient
with complex partial status epilepticus with a simple par-
tial onset. Simple partial onset with focal epileptiform dis-
charge was seen in the right superior frontal region (F4
electrode). With seizure progression there was a loss of
consciousness and the development of generalized back-
ground slowing.
 29
EEG Criteria for NCSE

Figure 21.
(A,B) NCSE in the postictal phase of TCSE (Langheinrich et al., 2005). Calibration: 1 s per horizontal unit; 100 lV
per vertical unit.
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R. Sutter and P. W. Kaplan
Case 5
Clinical/EEG description (Kikumoto et al., 2009):
Patient at the time of diagnosis of complex partial status epi-
lepticus. Ictal EEG showed 1.5–3 Hz irregular slow spike
and waves and polyspike and waves mixed with 14–16 Hz
fast activity, observed predominantly in the left hemisphere.
3e. NCSE in the postictal phase of tonic–clonic status
epilepticus (TCSE)
Case 1
Clinical/EEG description (Langheinrich et al., 2005):
A 39-year-old woman in a stuporous state over 6 days
after tonic–clonic status epilepticus. On day 6 the woman
was found to be drowsy, feverish (temperature 39.0C),
not eating or drinking, slow to obey simple commands,
and having attacks every few minutes; she had slight stiff-
ening of the whole body with rolling up of the eyes
lasting <10 s. The EEG (Fig. 21A) showed widespread
bursts of spike and slow-wave activity with a frontal
preponderance.
Case 2
Clinical/EEG description (Shorvon & Trinka, 2010):
A 24-year-old woman after a GTCS with prolonged mild
fluctuating impairment of consciousness. EEG showed
polyspike-and-waves, periodic generalized polyspikes-
and-waves. There was preserved alpha rhythm seen
between the generalized polyspikes-and-waves.
Case 3
Clinical/EEG description (Shorvon & Trinka, 2010):
A 50-year-old man after a GTCS with prolonged postictal
coma. EEG revealed periodic lateralized epileptiform
activity over the left frontocentral leads.
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Case 4
Clinical/EEG description (Korff & Nordli, 2007): A 10-
year-old girl in convulsive status epilepticus. EEG obtained
after convulsions resolved but while still unresponsive
showed periodic discharges of nonconvulsive status epilepti-
cus following convulsive status epilepticus. There were right
hemisphere periodic sharp waves with a slow repetition rate.
Case 5
Clinical/EEG description (Korff & Nordli, 2007): A
12-year-old girl with prolonged unresponsiveness after
convulsive status. EEG showed bilateral periodic lateral-
ized epileptiform discharges (rhythmic 1 Hz spike-and-
wave discharges).
3f. Subtle status epilepticus (myoclonic status after
convulsive status epilepticus)
Case 1
Clinical/EEG description (Arzimanoglou & Resnick,
2011): A 39-year-old woman with viral encephalitis and sec-
ondary generalized convulsive status epilepticus, then coma-
tose with mild bilateral facial twitching. EEG showed subtle
status epilepticus with generalized periodic discharges inter-
rupted by short generalized flat periods (Fig. 22).
3g. Aura continua
(i) Sensory symptoms
Case 1
Clinical/EEG description (Manford & Shorvon,
1992): 23-year-old man with ‘‘butterfly sensations,’’ often
felt in the abdomen but not specifically localized,
sometimes persisting after the seizure. During prolonged
Figure 22.
Subtle status epilepticus
(Arzimanoglou & Resnick,
2011). Calibration: 1 s per
horizontal unit; 100 lV per
vertical unit.
Epilepsia ILAE
 31
EEG Criteria for NCSE

Figure 23.
Aura continua with sensory
symptoms (‘‘butterfly
sensations’’) (Manford &
Shorvon, 1992). Calibration:
1 s per horizontal unit;
100 lV per vertical unit.
Epilepsia ILAE

Figure 24.
(A,B) Aura continua with
special sensory symptoms
(anxiety) (Brigo et al., 2011).
Calibration: 1 s between B
vertical lines; sensitivity
100 lV/mm.
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R. Sutter and P. W. Kaplan
epigastric sensation, there was rhythmical slow activ-
ity, predominantly in the right frontocentral leads (Fig. 23).
(ii) Special sensory symptoms
Case 1
Clinical/EEG description (Brigo et al., 2011): A 60-
year-old with prolonged fear and anxiety. EEG showed
continuous, rhythmic 2.5-Hz spike-and-wave activity lat-
eralized to the right, with maximal amplitude in the pari-
etotemporal region (Fig. 24A).
(iii) Autonomic symptoms
Case 1
Clinical/EEG description (Panayiotopoulos, 2004):
A child with Panayiotopoulos syndrome. EEG (Fig. 25A)
B vertical unit. Calibration in
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showed high-amplitude spike-and-waves recorded from
the bifrontal regions before onset of the electrical dis-
charge, which was also purely bifrontal. Marked tachycar-
dia appeared 13 min from the onset of the electrical
discharge, when this had become diffuse. There was
tachycardia and ictus emeticus.
Case 2
Clinical/EEG description (Koutroumanidis et al.,
2005): A 5-year-old child with emetic symptoms and other
autonomic phenomena. EEG showed bifrontal spikes that
increased during sleep, with additional independent
interictal left centrotemporal spikes. The seizure started
from stage II sleep with fast activity over the bifrontal areas.
After 12 min from onset he had change in heart rate from
65–120 beats/min. Level of cognition fluctuated between
reduced awareness and complete unresponsiveness.
Figure 25.
(A,B) Aura continua with
autonomic symptoms
(visceral, tachycardia)
(Panayiotopoulos, 2004).
Calibration in A: 2 s per
horizontal unit; 200 lV per
B: 1 s per horizontal unit;
100 lV per vertical unit.
Epilepsia ILAE
 33
EEG Criteria for NCSE

Figure 26.
Aura continua with cognitive symptoms (isolated alexia) (Kutluay et al., 2007). Calibration: 1 s per horizontal unit;
sensitivity not known.
Epilepsia ILAE

Figure 27.
Aura continua with cognitive
symptoms (altered memory
task, alexia, and acalculia in
ictal neuropsychological
assessment) (Profitlich et al.,
2008). Calibration: 1 s per
horizontal unit; 100 lV per
vertical unit.
Epilepsia ILAE

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doi: 10.1111/j.1528-1167.2012.03593.x
34
R. Sutter and P. W. Kaplan

(iv) Cognitive symptoms
Case 1
Clinical/EEG description (Kutluay et al., 2007): A 57-
year-old man with fluctuations in mental status and an
inability to read. During the seizures he was unable to
answer questions and had nystagmoid movements of the
eyes to the right. Brain MRI revealed fluid-attenuated
inversion recovery (FLAIR) hyperintensities over the left
occipitotemporal region. EEG showed ictal activity over
the left temporooccipital region with a maximal field at
the O1, T3, and T5 electrode sites and intermittent focal
slowing over the left posterior head region. Four electro-
graphic seizures came from the left temporooccipital
region lasting 150–220 s. Ictal pattern started with peri-
odic sharp waves from the left temporooccipital region,
followed by rhythmic 8–9 Hz discharges.

Figure 28.
Aura continua with
cognitive symptoms (altered
vigilance, comprehension,
writing, ideomotor praxis,
motor and memory tasks, as
well as altered
visuoconstructive tasks and
old memory in ictal
neuropsychological
assessment) (Profitlich
et al., 2008). Calibration: 1 s
per horizontal unit; 150 lV
per vertical unit.
Epilepsia ILAE

Figure 29.
Aura continua with
cognitive symptoms (altered
mood and affect in ictal
neuropsychological
assessment) (Profitlich
et al., 2008). Calibration: 1 s
per horizontal unit; 70 lV
per vertical unit.
Epilepsia ILAE

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Case 2
Clinical/EEG description (Profitlich et al., 2008): A
42-year-old man with altered memory. Speech revealed
the use of neologisms and perseverations. Counting, nam-
ing, and fluency were reduced. He had alexia, acalculia,
and apraxia. EEG revealed bilateral generalized spike and
spike-and-wave activity with frontal emphasis (Fig. 27).
Case 3
Clinical/EEG description (Profitlich et al., 2008): A
31-year-old woman with reduced psychomotor speeds,
learning, and memory. Ictal neuropsychological test
showed altered vigilance, comprehension, writing, ideo-
motor praxis, motor and memory tasks, as well as altered
visuoconstructive tasks and old memory. EEG showed
cryptogenic generalized bifrontal spike activity with right
emphasis (Fig. 28).
Case 4
Clinical/EEG description (Profitlich et al., 2008): A
49-year-old man with altered mood and affect. EEG
Figure 30.
Aura continua with cognitive symptoms (isolated aphasia respectively speech arrest) (DeToledo et al., 2000).
Calibration: Sensitivity not known.
Epilepsia ILAE
35
EEG Criteria for NCSE
revealed cryptogenic generalized high-amplitude rhyth-
mic 10–12 Hz alpha activity (Fig. 29).
Case 5
Clinical/EEG description (DeToledo et al., 2000): A
69-year-old woman with episodes of inability to talk,
without any other motor or cognitive impairment. Epi-
sodes lasted as long as 24 h. During aphasia EEG showed
3.5-Hz paroxysmal discharges (continuous spikes), maxi-
mal over the right frontal and central regions (Fig. 30).
4. NCSE in Late Adulthood
4a. De novo absence status epilepticus of late onset
Case 1
Clinical/EEG description (Szucs et al., 2008): A 55-
year-old woman with strange states lasting for hours or
days when she could not care for herself, was disoriented,
could not perform everyday activities, and sometimes
became paranoid and aggressive. At admission, she was
extremely slow, smiling, vague, and confused. EEG
Epilepsia, 53(Suppl. 3):1–51, 2012
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36
R. Sutter and P. W. Kaplan
revealed continuous, generalized, 3–4 Hz spike-and-wave
pattern during her slow, disoriented state (Fig. 31).
Case 2
Clinical/EEG description (Genton et al., 2008): EEG
of a 53-year-old confused, slow, and drowsy man showed
almost continuous generalized spike-and-wave and poly-
spike-and-wave discharges at 3 Hz. Intravenous injection
of diazepam induced a transient effect, with patient
regaining full consciousness and partially recalling the
event. After 15 min, status epilepticus reappeared with
EEG showing pseudorhythmic bursts of spike-wave and
polyspike-and-wave discharges.
Case 3
Clinical/EEG description (Szucs et al., 2008): A
56-year-old housewife with sparse, generalized tonic–
clonic convulsions as well as frequent ‘‘small
seizures’’ with absence-like features. Her movements
and speech were unusually slow; she had small,
episodic jerks of the jaw; and she seemed to be absent,
disoriented, and strange. EEG showed continuous
Figure 31.
De novo absence status epilepticus of late onset (Szucs et al., 2008). Calibration: 1 s between prominent vertical
lines; sensitivity not known.
Epilepsia ILAE
Epilepsia, 53(Suppl. 3):1–51, 2012
doi: 10.1111/j.1528-1167.2012.03593.x
2.5–3.5 Hz spike-and-wave pattern, which disap-
peared after intravenous injection of diazepam. Her
psychomotor activity improved and she became ori-
ented and responsive.
Case 4
Clinical/EEG description (Szucs et al., 2008): A 63-
year-old woman with tonic–clonic seizures followed by
stupor, with no verbal or metacommunicative contact; she
stared in a rigid, vague way. She sat or lay and remained in
forced positions in a catatonic manner. EEG showed con-
tinuous, generalized, 2.5–4 Hz spike-, polyspike-and-
wave pattern during patient’s stuporous state.
Case 5
Clinical/EEG description (Pro et al., 2011): A 72-year-
old woman with acute onset of altered consciousness for 5 h.
The episodes were characterized by acute onset of mental
confusion and ideomotor slowing, apraxia, and speech
arrest, without motor abnormalities or incontinence. EEG
showed generalized spike and polyspike-and-wave dis-
charges at 3–3.5 Hz of higher voltage in the anterior regions.

Case 6
Clinical/EEG description (Fernandez-Torre et al.,
2011): A 74-year-old woman with slowness in mental
activity. She was confused and disoriented to person,
time, and place. There were no automatisms or
myoclonic jerks. EEG showed nearly continuous gener-
alized spike-and-wave and polyspike-and-wave dis-
charges. At that time, the woman was perplexed and
confused.
Case 7
Clinical/EEG description (Fernandez-Torre et al.,
2011): A 79-year-old woman with abnormal behavior
who was confused and disoriented. When she was coma-
Figure 32.
(A,B) NCSE in late adulthood with opercular origin (Thomas et al., 1995). Calibration in A: 3 s per horizontal unit;
100 lV per vertical unit. Calibration in B: 1 s per horizontal unit; 75 lV per vertical unit.
Epilepsia ILAE
37
EEG Criteria for NCSE
tose, EEG revealed continuous ongoing seizure activity
constituted by synchronous high-voltage epileptiform dis-
charges with brief periods of flattening. Focal sharp waves
localized in the left centroparietal area and vertex after the
injection of 10 mg of diazepam.
4b. Other forms of NCSE in late adulthood
Case 1
Clinical/EEG description (Thomas et al., 1995): A 47-
year-old man with rhythmic bilateral clonic twitching of
the lower part of the face, lasting up to 5 h. Opening of the
mouth when seizure developed provoked a brief arrest of
the myoclonus, whereas closing the mouth did not modify
Epilepsia, 53(Suppl. 3):1–51, 2012
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38
R. Sutter and P. W. Kaplan
the seizures (Fig. 32B; MO = mouth open, MC = mouth
closed). EEG revealed opercular myoclonic–anarthric sta-
tus epilepticus. Myoclonias were recorded as muscular
potentials over the right temporal area (associated left
facial palsy) and produced 5–10 mm amplitude quivering
of the inferior jaw with no evidence of associated cortical
epileptiform EEG activity. During recording, eyes and
mouth were opened.
Case 2
Clinical/EEG description (Thomas et al., 1995): A
33-year-old right-handed man with sudden expressive
aphasia lasting 5 h. Ten months later, he had perma-
nent rhythmic lingual movements. EEG showed
continuous slow periodic myoclonias occurring with
3.5–4 s frequency involving the tongue and the hypo-
B Calibration: 1 s per
Epilepsia, 53(Suppl. 3):1–51, 2012
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pharyngeal area. Top trace showed occurrence of
rhythmic, 2-Hz clonic seizure with a transient postictal
silent period. Myoclonus was preceded on surface
EEG by a biphasic component localized over the left
inferofrontal (F3-F7) and left centrotemporal (C3-T3)
areas (arrowheads). Back-averaging of 50 consecutive
traces showed that the biphasic component preceded
myoclonia onset a )160 msec delay.
Case 3
Clinical/EEG description (Thomas et al., 1995): A
62-year-old man developed severe permanent dysarthria
with buccofacial apraxia associated with a bilateral lower
central facial palsy. There was no impairment of compre-
hension or vigilance. Brain MRI showed a right opercular
tumor. EEG revealed opercular myoclonic–anarthric
Figure 33.
(A,B) NCSE in late
adulthood with sporadic
Jakob-Creutzfeldt disease
(Espinosa et al., 2010).
horizontal unit; 140 lV per
vertical unit.
Epilepsia ILAE

status epilepticus due to opercular oligodendroglioma
with recurrent left hemifacial somatomotor seizures. Sta-
tus epilepticus was characterized by bilateral middle-
amplitude slow myoclonias involving lips, chin, and soft
palate with no evidence of associated cortical epileptiform
EEG abnormality.
Case 4
Clinical/EEG description (Espinosa et al., 2010): A
64-year-old woman with sporadic Jakob-Creutzfeldt
disease. Autopsy revealed spongiform changes in corti-
cal and subcortical sections. Interictal EEG showed
poorly organized background, generalized slowing, and
periodic epileptiform discharges at a frequency of
1–1.5 Hz greater over the right temporal region
(Fig. 33A). The ictal EEG revealed bilateral synchro-
nous high-amplitude, periodic sharp wave discharges
that evolve in morphology and frequency (Fig. 33B),
followed by intermittent brief diffuse suppression with-
out changes in behavior.
Case 5
Clinical/EEG description (Bauer & Trinka, 2010): A
65-year-old woman with Jakob-Creutzfeldt disease. After
intravenous injection of 4 mg clonazepam, no change of
continuously repeated and rhythmic 1–2 Hz ‘‘triphasic’’
waves, no spikes.
Figure 34.
Coma with epileptiform EEG changes in cryptogenic encephalitis (Fernandez-Torre et al., 2011). Calibration: 1 s per
horizontal unit; 100 lV per vertical unit.
Epilepsia ILAE
39
EEG Criteria for NCSE
5. Boundary Syndromes
5a. Coma with epileptiform EEG changes
(i) Cryptogenic encephalitis
Case 1
Clinical/EEG description (Fernandez-Torre et al.,
2011): A 39-year-old woman in deep coma after crypto-
genic encephalitis showed continuous epileptiform
discharges consisting of rhythmic sharp waves and
sharp-and-slow-wave complexes at 2.5–3 Hz (Fig. 34).
(ii) Hypoxic encephalopathy
Case 1
Clinical/EEG description (Holtkamp & Meierkord,
2011): A 56-year-old comatose man after global cerebral
hypoxia following ventricular fibrillation had dominating
GPEDs at 2 Hz showing maximum amplitudes in bifrontal
regions (Fig. 35A). Between the bursts the EEG activity
was extremely flat. Five minutes after intravenous admin-
istration of 2 mg lorazepam, GPEDs disappeared and
there was widespread low-amplitude activity at the end of
the trace (Fig. 35B). The changes after administration of
benzodiazepines were not associated with any clinical
improvement.
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R. Sutter and P. W. Kaplan

Case 2 rhythm and hypotension was unresponsive with absent

Clinical/EEG description (Lowenstein & Aminoff,
1992): A 72-year-old man after cardiac arrest with repeti-
tive motor activity of the mouth and extremities. EEG
showed continuous spike-and-wave activity.
Case 3
Clinical/EEG description (Lowenstein & Aminoff,
1992): A 31-year-old man after cardiac arrest with
frequent, stereotyped episodes in which there were clonic
movements on the left side of the face and right lower
extremity. EEG revealed mixed frequency background
activity with episodic accentuation and occasional sharp
transients.
Case 4
Clinical/EEG description (Lowenstein & Aminoff,
1992): A 79-year-old man with idioventricular cardiac
pupillary light responses, oculocephalic reflex, corneal
reflex, and deep tendon reflexes. No clinical seizures were
observed. Twenty-four hours after admission he showed
episodes of repetitive sharp waves, slightly more marked
on the left.
Case 6
Clinical/EEG description (Bauer & Trinka, 2010): A
76-year-old comatose woman after status asthmaticus had
continuous and very regular generalized 2–3 Hz spike-
and-wave activities 8 h after onset of status asthmaticus.
Case 7
Clinical/EEG description (Bauer & Trinka, 2010): A
63-year-old man with coma after cardiac arrest showed
periodic multiple spikes on a nearly flat background activ-
ity on day 2.

Figure 35.
(A,B) Coma with
epileptiform EEG changes in
hypoxic encephalopathy
(Holtkamp & Meierkord,
B
2011). Calibration: 1 s per
horizontal unit; 100 lV per
vertical unit.
Epilepsia ILAE

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Case 8
Clinical/EEG description (Bauer & Trinka, 2010): A
49-year-old woman in a coma was on respirator due to
intoxication with sedative drugs. She had no brainstem
reflexes and EEG showed a burst-suppression pattern.
Case 9
Clinical/EEG description (Bauer & Trinka, 2010): A
58-year-old man with coma after cardiac arrest showed a
burst-suppression pattern containing spikes with short
interval on day 3 after the initial event.
Case 10
Clinical/EEG description (Bauer & Trinka, 2010): A
53-year-old man in a coma after traumatic brain injury.
EEG showed BIPLEDs with a unilateral burst-suppression
pattern over the right hemisphere.
5b. Epileptic behavioral disturbance or psychosis
Case 1
Clinical/EEG description (Chiara et al., 2011): A
76-year-old woman with dementia looked distracted and
vacant, and replied to questions after a brief pause and
with inadequate answers. She had trouble speaking, and
Figure 36.
Epileptic behavioral disturbance in association with dementia (Chiara et al., 2011). Calibration: 1 s between vertical
lines; sensitivity not known.
Epilepsia ILAE
41
EEG Criteria for NCSE
was uncooperative, disoriented, had a fixed gaze, and
was unresponsive to any stimuli. She had oroalimentary
automatisms and jerking of the right foot. EEG showed
theta–delta activity with sporadic low-voltage spikes
prevalent in the bilateral frontotemporal regions
(Fig. 36).
Case 2
Clinical/EEG description (Valko et al., 2009): A 63-
year-old right-handed woman who was confused, agitated,
dysarthric, and incoherent in her thoughts had widespread
right hemispheric attenuation and periodic frontally
accentuated focal epileptic discharges consistent with the
diagnosis of a NCSE. Periodic 1.5-Hz epileptic discharges
were seen with right frontal predominance (Fig. 37A).
Case 3
Clinical/EEG description (Sethi et al., 2010): A 93-
year-old woman with acute right temporooccipital stroke.
Video-EEG recording revealed frequent brief right hemi-
spheric focal seizures, every 5–10 min. There was 9–
10 Hz rhythmic activity in the right posterior temporal
leads evolving to spread across the entire right hemisphere
with intermixed sharp waves and spike-and-wave dis-
charges (Fig. 38A–D).
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R. Sutter and P. W. Kaplan

Figure 37.
(A,B) Epileptic behavioral disturbance in association with cerebral autosomal dominant arteriopathy with
subcortical infarcts and leukoencephalopathy (CADASIL) (Valko et al., 2009). Calibration: 1 s per horizontal unit;
50 lV per vertical unit.
Epilepsia ILAE

Epilepsia, 53(Suppl. 3):1–51, 2012

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 43
EEG Criteria for NCSE

Figure 38.
(A–D) Epileptic behavioral disturbance with frequent throat clearing (Sethi et al., 2010). Calibration: 1 s per
horizontal unit; 50 lV per vertical unit.
Epilepsia ILAE

Epilepsia, 53(Suppl. 3):1–51, 2012

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44
R. Sutter and P. W. Kaplan

Figure 38b.
Continued.
Epilepsia ILAE

Epilepsia, 53(Suppl. 3):1–51, 2012

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 45
EEG Criteria for NCSE

5c. Drug-induced or metabolic confusional state with
epileptiform EEG changes
(i) Drug-induced confusion state with epileptiform EEG
changes
Case 1
Clinical/EEG description (Anzellotti et al., 2011): A
64-year-old woman on cefixime followed by confusion
had continuous generalized spike or double-spike and
slow-wave discharges, which were occurring at a fre-
quency of 3–3.5 Hz and amplitudes of 100–120 lV
(Fig. 39A), with sporadic inscription of polyspikes at a
frequency of 14–15 Hz and amplitudes of 90–100 lV.
Case 2
Clinical/EEG description (Thabet et al., 2009): A 15-
year-old girl on hemodialysis and cefepime who had

Figure 39.
(A,B) Drug-induced confusional state with epileptiform EEG changes by cefixime (Anzellotti et al., 2011).
Calibration: 1 s between vertical lines; sensitivity 7 lV/mm.
Epilepsia ILAE

Epilepsia, 53(Suppl. 3):1–51, 2012

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R. Sutter and P. W. Kaplan

myoclonic arm jerks and become unresponsive. EEG tive than usual. Subcontinuous EEG abnormalities during
revealed generalized spike and sharp wave activity com- NCSE with high-amplitude 3–4 Hz sharp waves with
patible with NCSE (Fig. 40A). Repeated EEG after cefe- irregular runs of atypical spike-and-wave complexes over
pime withdrawal was normal (Fig. 40B). the anterior regions of both hemispheres.

Case 3 Case 4
Clinical/EEG description (Piccinelli et al., 2000): A Clinical/EEG description (Knake et al., 1999): A 32-
12-year-old boy on tiagabine became less active and reac- year-old woman on tiagabine with mental clouding of

Figure 40.
(A,B) Drug-induced confusional state with epileptiform EEG changes by cefepime (Thabet et al., 2009).
Calibration: 1 s between prominent vertical lines; sensitivity 15 lV/mm.
Epilepsia ILAE

Epilepsia, 53(Suppl. 3):1–51, 2012

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Figure 41.
(A,B) Drug-induced confusional state with epileptiform EEG changes by tiagabine (Piccinelli et al., 2000). Calibration:
1 s per horizontal unit; 100 lV per vertical unit.
Epilepsia ILAE
>4 hours. She had never experienced absences lasting for
more than a minute before treatment with tiagabine. Dur-
ing status epilepticus for >6 h EEG showed intermittent
generalized slowing and spike-and-wave complexes.
Case 5
Clinical/EEG description (Imperiale et al., 2003): A
30-year-old woman who developed NCSE during tiaga-
bine adjunctive therapy. EEG during tiagabine-associated
NCSE episode showed diffuse slowing of background
47
EEG Criteria for NCSE
rhythm and frequent spike-and-wave discharges on right
temporal derivations spreading to contralateral regions.
After intravenous injection of 4-mg lorazepam, EEG
revealed clear improvement of background activity with
appearance of left temporal spike-wave discharges associ-
ated with rare contralateral sharp waves.
Case 6
Clinical/EEG description (Mangano et al., 2003): A
4-year-old patient who developed NCSE following
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R. Sutter and P. W. Kaplan

Figure 42.
(A,B) Drug-induced confusional state with epileptiform EEG changes by topiramate (Brandt et al., 2010).
Calibration: 1 s per horizontal unit; 10 lV per vertical unit.
Epilepsia ILAE

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 49
EEG Criteria for NCSE

A B

Figure 43.
(A,B) Metabolic confusional state with epileptiform EEG changes in citrullinemia and ammonemia in adult-onset type
II citrullinemia (CTLN2) (Funabe et al., 2009). Calibration: 1 s per horizontal unit; 50 lV per vertical unit.
Epilepsia ILAE

tiagabine as add-on treatment for refractory partial sei-
zures. Ictal EEG, while patient was confused and unre-
sponsive, showed generalized subcontinuous spike-and-
sharp-wave discharges during tiagabine.
Case 7
Clinical/EEG description (Brandt et al., 2010): A 21-
year-old man with idiopathic generalized epilepsy who
ingested about 8,000 mg of topiramate concentration of
144.6 lg/ml. EEG showed a nearly continuously general-
ized seizure pattern with changing maximum, most fre-
quently left frontal respectively left frontotemporal with
wide potential field (Fig. 42A,B after intravenous injec-
tion of lorazepam).
(ii) Metabolic confusional state with epileptiform EEG
changes
Case 1
Clinical/EEG description (Funabe et al., 2009): A
47-year-old woman with repeated unconsciousness
and abnormal behavior. The high plasma ammonia
level was not always associated with neurobehavioral
symptoms (unconsciousness, disorientation, abnormal
behavior, and epilepsy), but paroxysmal EEG dis-
charges were invariably associated with these symp-
toms. Intravenous injection of diazepam improved
neurobehavioral symptoms and EEG discharges
(Fig. 43A: before treatment; Fig. 43B: after treatment
of citrullinemia).

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R. Sutter and P. W. Kaplan
Disclosure
None of the authors has any conflicts of interest to disclose regarding
this research activity. Raoul Sutter is supported by the Research Funds of
the University of Basel, the Scientific Society of Basel, and the Gottfried
Julia Bangerter-Rhyner Foundation. We confirm that we have read the
Journal’s position on issues involved in ethical publication and affirm
that this report is consistent with those guidelines.
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