Epilepsia, 53(Suppl.

4):127138, 2012
doi: 10.1111/j.1528-1167.2012.03622.x

SEIZURES IN SPECIAL AND SEVERE SITUATIONS

Causes of status epilepticus

Eugen Trinka, Julia Hofler, and Alexander Zerbs
Department of Neurology, Christian Doppler Klinik, Paracelsus Medical University Salzburg, Salzburg, Austria

SUMMARY
Status epilepticus (SE) is the most extreme form
of epilepsy. It describes a prolonged seizure that
may occur in patients with previous epilepsy or in
acute disorders of the central nervous system. It is
one of the most common neurologic emergencies,
with an incidence of up to 41 per 100,000 per year
and an estimated mortality is 20%. The three
major determinants of prognosis are the duration
of SE, patient age, and the underlying cause. Common and easily recognized causes of SE include
cerebrovascular disorders, brain trauma, infections, and low antiepileptic drug levels in patients

Status epilepticus (SE) is a term used to describe a prolonged and self-sustaining seizure that may have overt,
subtle, or almost no behavioral manifestations. It may be
regarded as the most extreme form of epilepsy, or as an
expression of an acute and often life-threatening brain disorder, such as stroke, encephalitis, or trauma. Mortality
associated with SE is up to 20% (Shorvon, 1994; Logroscino
et al., 1997, 2005). Less than 50% of people in SE have
had previous seizures or epilepsy (DeLorenzo et al., 1996;
Hesdorffer et al., 1998; Coeytaux et al., 2000; Knake
et al., 2001; Vignatelli et al., 2003). SE is one of the most
common neurologic emergency, with an overall annual
incidence of 1041 per 100,000 (DeLorenzo et al., 1996;
Hesdorffer et al., 1998; Coeytaux et al., 2000; Knake
et al., 2001; Vignatelli et al., 2003). Up to 287,000 patients
per year are affected in Europe. Not all forms of status are
life-threatening and, given the variety of its clinical presentations, the management must be tailored according to
the type of SE and the underlying cause. Three major
factors determine an increased risk of mortality and morbidity associated with SE: (1) certain etiologies, (2) age

Address correspondence to Eugen Trinka, Department of Neurology,

Christian Doppler Klinik, Paracelsus Medical University, Ignaz Harrer
Strae 79, 5020 Salzburg, Austria. E-mail: e.trinka@salk.at
Wiley Periodicals, Inc.
2012 International League Against Epilepsy

with epilepsy. Less common causes present a clinical and diagnostic challenge, but are major determinants of prognosis. Among them, inflammatory
causes and inborn errors of metabolism have
gained wide interest; recent insights into these
causes have contributed to a better understanding
of the pathophysiology of SE and its appropriate
treatment. This review focuses on the different
etiologies of SE and emphasizes the importance of
prompt recognition and treatment of the underlying causes.
KEY WORDS: Status epilepticus etiology, Treatment, Prognosis.

>60 years, and (3) long duration of SE (Towne et al.,

1994; DeLorenzo et al., 1996; Wu et al., 2002; Rossetti
et al., 2006). This review focuses on the various causes of
SE, since etiology is increasingly recognized as one of the
most important factors for prognosis and outcome. Therefore, identifying and treating the underlying cause of SE is
at least as important as prompt and effective treatment
with early termination of seizures.

Definition and Classification

of Status Epilepticus
Status epilepticus (SE) has been recognized for centuries (Shorvon, 1994; Wolf et al., 2009). Henri Gastaut
(1970) recognized SE as a prolonged seizure with as many
forms as there were types of epileptic seizures. Therefore,
SE classification mirrored exactly the seizure classification. In the International League against Epilepsy (ILAE)
1981 Classification, SE was defined as a seizure that persists for a sufficient length of time, or repeated frequently
enough that recovery between attacks do [does] not
occur (Commission on Classification, ILAE, 1981).
Generalized tonicclonic seizures usually do not last
longer than 23 min (Theodore et al., 1994); the risk of a
seizure becoming self-sustaining increases as the duration
reaches 5 min or more (Lowenstein et al., 1999). For the
purpose of this review, we use a clinical classification

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E. Trinka et al.
along two taxonomic criteria: the presence (or absence) of
motor symptoms and the impairment (or retention) of consciousness. Therefore, one can distinguish (A) SE types
with prominent motor symptoms, such as convulsive SE,
and (B) SE types without prominent motor symptoms,
summarized as nonconvulsive SE (NCSE). NCSE can
occur with or without coma; this has important etiologic
implications and determines its treatment. A third category (C) comprises the boundary syndromes, including
epileptic encephalopathies and acute forms of coma with
status-like electroencephalography (EEG) patterns.
Table 1 briefly outlines this tentative classification. For a
more detailed discussion of SE classification, refer to:
(Shorvon, 1994; Walker et al., 2005; Bauer & Trinka,
2006, 2009; Berg et al., 2010). However, the definitions
and classifications are in flux and an ILAE Task force is
currently developing a new draft classification of SE, following the concepts of the ILAEs new classification proposal of seizures and syndromes (Berg et al., 2010).

Causes of Convulsive Status

Epilepticus in Adults
Most population-based studies have used a traditional
30-min duration of SE, and so the numbers given are the
lowest estimates. Using the 5-min definition, determining
the time from onset to starting emergency treatment, the
incidence in clinical practice is much higher than in the
epidemiologic studies. Convulsive SE comprises 3770%
of all forms of status, and its annual incidence is up to 40
per 100,000 (Waterhouse, 2008). In adults with preexisting epilepsy, the most common etiologies are low antiepileptic drug (AED) levels (accounting for at least one
fourth of SE [Fig. 1]), remote symptomatic etiologies, and

Table 1. Proposed classification of seizure

types according to their semiology, along two
taxonomic criteria: motor symptoms and
impairment of consciousness
With prominent motor symptoms
Convulsive SE (syn.: tonicclonic SE)
Myoclonic SE (prominent epileptic myoclonic jerks)
Focal motor (including EPC)
Tonic SE
Hyperkinetic SE
Without prominent motor symptoms (i.e., NCSE)
NCSE with coma
NCSE without coma
Generalized
Focal
Boundary syndromes
Epileptic encephalopathy
Acute forms of coma with status-like EEG pattern
Epileptic behavioral disturbance and psychosis
Confusional states, or delirium with epileptiform EEG changes

Epilepsia, 53(Suppl. 4):127138, 2012

doi: 10.1111/j.1528-1167.2012.03622.x

Figure 1.
Etiology of status epilepticus in adults, with associated
mortality for each category. Based on data from
DeLorenzo et al., 1995. AED, antiepileptic drugs; CNS,
central nervous system.
Epilepsia ILAE

stroke (DeLorenzo et al., 1995, 1996). This subgroup with

epilepsy and low AED levels has a good prognosis, with a
low mortality of 4.08.6% (Towne et al., 1994; DeLorenzo
et al., 1995). Overall, acute symptomatic causes are the
most common etiology, accounting for 4863% of all SE
cases (Hesdorffer et al., 1998; Coeytaux et al., 2000;
Knake et al., 2001). Stroke is the leading cause among the
acute symptomatic cases, accounting for 1422% of SE in
adults (DeLorenzo et al., 1995; Knake et al., 2001). In
older adults, remote stroke is a major cause. Knake et al.
(2001) found that remote stroke caused 36% of SE in
patients older than 56 years. In the Richmond Virginia
Status Epilepticus Study, 41% of adults and 61% of the
elderly had acute or remote ischemic and hemorrhagic
strokes as cause of status (DeLorenzo et al., 1995).
In the context of epilepsy, SE may develop in those with
a previous diagnosis of epilepsy or de novo, as its initial
manifestation. Approximately 15% of patients with epilepsy have had at least one episode of status during their
lifetime. Most often, the SE is due to the epilepsy itself,
triggered by medication nonadherence, resulting in subtherapeutic AED levels (Aminoff & Simon, 1980) or by
inappropriate drug treatment (Thomas et al., 2006a,b).
The clinical features of SE in these patients depend on the
underlying epilepsy syndrome. In the context of idiopathic
generalized epilepsy, status is most often nonconvulsive
(Shorvon & Walker, 2005); in the context of juvenile
myoclonic epilepsy it may be myoclonic (Thomas et al.,
2006a,b; Larch et al., 2009). Myoclonic status may also
develop in progressive myoclonic epilepsy, LennoxGastaut syndrome, or epilepsy with myoclonic absences.

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Causes of Status Epilepticus
However, the precise incidence of convulsive status or
myoclonic status in these syndromes is not known.
Approximately 12% of patients who eventually develop
epilepsy have presented with SE as their first clinical manifestation (Hauser, 1990; Hesdorffer et al., 1998). In these
patients, SE may be an intrinsic manifestation of disease,
sometimes with recurrent episodes, or the epilepsy may be
the consequence of a prolonged SE, with neuronal death
and alteration of networks causing recurrent seizures after
the initial event. During a 10-year follow-up, epilepsy
developed in 42% of patients who had acute symptomatic
SE and in 14% of patients who had acute symptomatic
seizures (Hesdorffer et al., 1998). The development of
subsequent epilepsy is more likely if the status is refractory to treatment (Holtkamp et al., 2005), supporting the
hypothesis that SE contributes to epileptogenesis by
enhancing hyperexcitable networks.

Nonconvulsive Status
Epilepticus
NCSE may be defined as an enduring epileptic condition with reduced or altered consciousness, behavioral and
vegetative abnormalities, or merely subjective symptoms
without major convulsive movements (Drislane, 2000).
This umbrella term includes a wide spectrum of disorders,
ranging from benign conditions, such as absence status in
idiopathic generalized epilepsy, to severe life-threatening
conditions, such as subtle SE or coma with generalized
epileptiform discharges (coma-GEDs). Therefore, it is
important to subdivide this category according to the
degree of unresponsiveness or to the depth of coma
(Fig. 2). Consciousness, which notoriously resists definition, becomes a taxonomic criterion for subdividing
NCSE (Bauer & Trinka, 2009). Consciousness itself can
be categorized into quantitative and qualitative consciousness. The quantitative element depends upon the patients
level of consciousness and arousability, which in turn
depends upon the integrity of the ponto-mesodiencephalic
reticular pathways and the thalamocortical projections.
Qualitative consciousness, on the other hand, depends
upon the content of consciousness, experience, emotions,
and sensations, known only to patients themselves. It
reflects the inner monologue and it is essential for any
meaningful interaction with the environment. Qualitative
consciousness is associated with awareness, enabling the
patient either to focus on and interact with the environment or to engage in an inner monologue. Table 2
describes the different types of NCSE.
The incidence of NCSE, based on epidemiologic studies, ranges from 40.0 to 66.9% (DeLorenzo et al., 1995;
Hesdorffer et al., 1998; Coeytaux et al., 2000; Knake
et al., 2001; Vignatelli et al., 2003). Like convulsive status, NCSE may occur in acute central nervous system
(CNS) disorders or may be a part of certain electroclinical

Figure 2.
Relationship between depth and coma (x-axis), prognosis (x-axis), degree of structural brain damage (red yaxis), and epileptic brain dysfunction (blue y-axis) due
to status epilepticus. Clinical entities depicted in the
upper part of the graph are arranged along the x-axis
without distinct positions, in recognition that large
border zones and overlaps between the conditions
may exist. With permission from Bauer & Trinka, 2009.
AS, absence status epilepticus; EPC, epilepsia partialis
continua; GEDs, generalized epileptiform discharges;
IGE, idiopathic generalized epilepsy; LEDs, lateralized
epileptiform discharges; NCSE, nonconvulsive status
epilepticus.
Epilepsia ILAE

Table 2. Categories of NCSE, classified

according to the degree of disturbed
consciousness
NCSE with coma
NCSE without coma
Generalized
Typical absence status
Atypical absence status
Myoclonic absence status
Focal
Aura continua
With vegetative symptoms
With sensory symptoms
With visual symptoms
With olfactory symptoms
With gustatory symptoms
With emotional symptoms
Aphasic SE
SE with dyscognitive symptoms

syndromes. We do not know the precise incidence of

NCSE in the epilepsy population. In our own audit (1975
to 2003) at the University Hospital Innsbruck, 220 patients
with epilepsy (140 women, median age 45 years [range 2
89]) had at least one episode of NCSE either during their
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130
E. Trinka et al.
Table 3. Types of NCSE in 220 patients with
epilepsy and a history of NCSE (Bauer &
Trinka, data on file)
Generalized (106 = 48.2%)
Typical absence SE
Atypical absence SE
Localized/lateralized (100 = 45.5%)
Focal simple (58 = 26.4%)
Aura continua
Vegetative
Aphasic
Pure frontal
Psychosis
Focal complex (42 = 19.1%)
Continuous
Discontinuous
Unclassified
Total

57
49

25.9
22.3

11
8
27
9
3

5.0
3.6
12.3
4.1
1.4

30
12
14
220

13.7
5.5
6.3
100.1

disease or at their initial presentation (Bauer G, Trinka E,

unpublished data). The cause of NCSE was remote symptomatic in 47%, idiopathic in 45%, and remained

unknown (cryptogenic) in 48%. Forty-six percent had

focal SE, 21% had a generalized NCSE in the context of
idiopathic (or genetic) generalized epilepsies, and 22%
had atypical absence status in the context of Lennox-Gastaut syndrome. Table 3 details their different types of status epilepticus.

Causes of Absence Status

Epilepticus
Absence status (AS) is best described as a confusional
state of variable intensity, ranging from simple cognitive
slowing to catatonic stupor, lasting for hours to days or
weeks (Andermann & Robb, 1972). The EEG shows
bilateral rhythmic, synchronous, and mostly symmetric
paroxysmal activity, which can be continuous or discontinuous (Bauer & Trinka, 2010; Fig. 3). It is important to
recognize that AS is a heterogeneous condition, which
may occur in patients with preexisting idiopathic generalized epilepsy (typical AS) or during the course of
chronic symptomatic generalized epilepsy, such as Lennox-Gastaut syndrome (atypical AS). Of interest, some

Figure 3.
Typical absence status epilepticus in an older patient (V.J., female, 74 years. tc 0.3 F30). This patient had scattered
generalized tonicclonic seizures since age of 54 years. Her EEG showed periodically repeated generalized 3/s spike
and wave, with no major diffuse slow activity between periods (note the reduced time calibration). EEG kindly
provided by Prof. Dr. G. Bauer, Innsbruck. The patient displayed discontinuous psychic functions, and was amnesic
for the abnormal condition. Recovery was immediate after intravenous diazepam.
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Causes of Status Epilepticus
patients develop AS later in life, occasionally de novo
(Andermann & Robb, 1972; Thomas et al., 1992), or it
may occur as a late exacerbation of an idiopathic generalized epilepsy syndrome (Bauer et al., 2007). There may
also be a fourth group of AS, comprising absences with
very clear focal characteristics. This group may be considered as a transitional form between AS and dyscognitive
focal SE of frontal origin (Bauer et al., 2006; Thomas,
2011).
Many cases of AS have nonspecific precipitating factors. The most important are antiepileptic drug (AED)
withdrawal or impaired adherence, alcohol, sleep deprivation, and sleepwake cycle disturbance. Other nonspecific
factors include stress, fatigue, fever, mild head trauma, or
metabolic derangement after surgery (Thomas & Zifkin,
2008; Thomas & Gelisse, 2009).
Several idiopathic generalized epilepsy syndromes may
be aggravated by inappropriate AEDs: carbamazepine,
phenytoin, tiagabine, or other c-aminobutyric acid
(GABA)ergic medications (Snead & Hosey, 1985; Knake
et al., 1999; Thomas et al., 2006a,b; Trinka et al., 2002).
Thomas et al. reported 14 patients with idiopathic generalized epilepsy treated with either carbamazepine alone or
with other potentially aggravating drugs, for example,
phenytoin, vigabatrin, or gabapentin. Ten of these cases
developed AS; in half, the AS was atypical. All their
patients had a clear seizure aggravation, with development
of new seizure types before AS developed (Thomas et al.,
2006a,b). The prognosis was invariably good with full seizure control in all patients after switching to appropriate
drugs.
In late-onset AS, other drug-related factors play an
important clinical role (Thomas et al., 1992; Thomas &
Andermann, 1994). Most importantly, psychotropic medicationor its withdrawalmay provoke late-onset AS
(Fernandez-Torre, 2001). In addition, many other drugs,
for example, theophylline, baclofen, metformin, and
cimetidine, may exacerbate absences later in life (for a
review see Thomas & Snead, 2007).
There are metabolic and toxic factors in many patients,
although the precise incidence is not known. Examples
include hyponatremia, hypoglycemia decompensated
chronic renal failure, hepatic failure, and hypocalcemia
(for review see Thomas & Snead, 2007). There have been
several cases of AS following the use of contrast-enhancing products during myelography or carotid angiography
(Pritchard & ONeil, 1984; Vollmer et al., 1985; Coeytaux
et al., 2000). Some cases of transient global amnesia after
angiography may in fact also be epileptic in nature (Bauer
et al., 2005).
AS probably occurs most frequently in the context of
electroclinical syndromes. Aside from childhood
absence epilepsy, juvenile absence epilepsy, and juvenile myoclonic epilepsy, there are other syndromes associated with AS as a key clinical feature: AS may occur

in eyelid myoclonia with absences (Jeavons syndrome)

(Yang et al., 2008), idiopathic generalized epilepsy with
phantom absences (Panayiotopoulos et al., 2001), perioral myoclonias with absences (Agathonikou et al., 1998),
or absences status epilepsy (Genton et al., 2008). The
prognosis in these syndromes (not yet fully accepted in
the ILAE classification) is generally good, and patients
respond well to appropriate AEDsvalproate in most
cases. However, this is not true of AS associated with
ring chromosome 20, where prolonged confusional
states are notoriously resistant to AEDs and there have
been lethal cases (Jacobs et al., 2008). The EEG shows
bilateral high-voltage slow waves, sometimes with intermingled spikes and frontal accentuation. There is no
typical clinical picture, except for mild cognitive impairment (Fig. 4).

Causes of Focal Nonconvulsive

Status Epilepticus
Focal NCSE (as with AS) encompasses a wide range of
clinical symptoms. Previously the term complex focal or
complex partial SE was used, which may be replaced in
future by dyscognitive SE. These forms usually have
structural abnormalities and clinical focal signs that must
be identified to guide appropriate drug treatment (Fig. 2).
Most of the published literature concerns patients with
temporal or extratemporal lobe epilepsy of remote symptomatic cause, whose NCSE is either as the presenting
symptom or develops during the course of the disease
(Tomson et al., 1992, Kaplan et al., 1996; Scholtes et al.,
1996). As with AS, there are often nonspecific risk factors,
but more often status occurs in these patients without specific provoking factors. In a review of 70 patients with
focal NCSE of frontal origin, more than one fourth had no
history of epilepsy. Forty-five percent had a focal frontal

Figure 4.
Clinical course of convulsive status epilepticus.
Epilepsia ILAE
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E. Trinka et al.
lesion. The etiology was most often a brain tumor (benign
or malignant), or a posttraumatic or a postsurgical lesion
(Thomas & Zifkin, 2008).
Rare causes of focal NCSE include intravenous contrast
media, or drugs such as ciprofloxacin, lithium intoxication, theophylline, vigabatrin, tiagabine, or crack cocaine
(Thomas et al., 2006b). Mesial temporal focal NCSE can
also be the presenting symptom of the increasingly recognized nonparaneoplastic limbic encephalitis, related to
voltage-dependent potassium channels (VGKC/LGI1)
(Irani et al., 2010), NMDA receptors (Vincent & Bien,
2008), or antibodies against glutamic acid decarboxylase
(Malter et al., 2010). Some of them lead to a specific seizure type (e.g., faciobrachial dystonic seizures associated
with anti-VGKC/LGI1 antibodies), preceding the onset of
the limbic encephalitis (Irani et al., 2011a,b). It is important to note that paraneoplastic limbic encephalitis may
also present with focal NCSE (Dalmau et al., 2008), indicating the need for a comprehensive search and rigorous
treatment of any underlying primary neoplasm. We do not
yet know the full spectrum of this new group of immunemediated encephalopathies (Table 4).
The outcome for focal NCSE is less favorable than that
for AS. Long-lasting focal status, especially of temporal
lobe origin, may cause brain edema identifiable on magnetic resonance imaging (MRI) (Bauer et al., 2006). These
patients may also have severe and prolonged amnesia
(Engel et al., 1978; Treiman et al., 1981). However, many
patients presenting with focal NCSE have acute or remote
symptomatic epileptogenic lesions, making it difficult to
disentangle the dysfunction due to the epileptic activity
from the effect of the structural lesion (Hilkens & De
Weerd, 1995; Kaplan, 1996; Trinka et al., 2002). However, focal NCSE occurring with an acute lesion most
likely contributes substantially to the associated neurologic dysfunction (Hilkens & De Weerd, 1995; Bauer &
Trinka, 2010). Therefore, patients with focal NCSE need
prompt and vigorous treatment, tailored to the underlying
cause.

Causes of Comatose
Nonconvulsive Status
Epilepticus
Comatose forms of NCSE need further attention. It is
important from the clinical standpoint (1) to clarify
whether the coma is caused by the epileptic seizure or status, or by the brain disorder itself, (2) to assess the contribution of epileptic activity to the depth of coma, (3) to
consider whether treatment improves prognosis in these
patients or not, and (4) to implement an appropriate treatment in these critically ill patients. Unlike in other forms
of NCSE, the cause of the comatose patients SE can
sometimes only be identified from the history, the temporal pattern of coma, and the neurologic signs. The epileptic
etiology is most often confirmed only by the presence of
continuous or discontinuous epileptiform discharges on
the EEG (Lowenstein & Aminoff, 1992; Jordan, 1999;
Brenner, 2005). A frequently used, but not universally
accepted, classification of patients in comatose status uses
the dichotomy of generalized versus focal or lateralized
EEG discharges (Brenner, 2004). The EEG is absolutely
necessary to make this distinction. In this review we used
the terms coma with generalized epileptiform discharges (or coma-GED) and coma with lateralized epileptiform discharges (or coma-LED) (Bauer & Trinka,
2009). The etiology in these patients is almost always
acute symptomatic, encompassing a wide variety of

Table 5. Etiologic factors and EEG pattern in

comatose NSCE
coma-GED

Table 4. Immunologic disorders causing

status epilepticus
Paraneoplastic encephalitis
Hashimoto encephalopathy
Anti-NMDA-receptor encephalitis
Anti-VGKC-receptor encephalitis
Rasmussen encephalitis
Cerebral lupus
Adult-onset Still disease
Anti-GAD antibody associated encephalitis
Goodpasture syndrome
Thrombotic thrombocytopenic purpura
Antibody-negative limbic encephalitis

NMDA, N-methyl-D-aspartate; GAD, glutamate acid decarboxylase.

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coma LED

Etiology

EEG pattern

Diffuse primary or
secondary brain
disturbances (anoxic,
toxic, metabolic,
infectious, degenerative)
Space-occupying lesions
with brainstem
compression (direct or
due to tentorial
herniation)a
Known epilepsies?
Focal brain lesions (in
most cases acutely
acquired) In rare cases
diffuse abnormalities
(aminophylline,
intoxiation, some forms
of diabetic coma)
Known epilepsies?

Continuous generalized
spiking
Periodic spiking
Burst suppression pattern
in different variations
Other generalized
periodic abnormalities
Bilateral triphasic waves

Continuous focal spiking

PLEDs and Bi-PLEDS
Unilateral burst
suppression pattern
unilateral triphasic waves

Bi-PLED, bilateral periodic epileptiform discharges; GEDs,

generalized epileptiform discharges; LED, lateralized epileptiform discharges; PLEDs, periodic epileptiform discharges.
a
Might also present as coma-LED.

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Causes of Status Epilepticus
Table 6. Causes of epilepsia partialis continua (according to Tan et al., 2010)
EPC type 1 (static cause)

Infancy

Hemimegalencephaly

Mitochondrial disease
(Alper disease)

Childhood

Focal cortical dysplasia

Sturge-Weber
syndrome focal cortical
dysplasia
Tuberous sclerosis

Rasmussen syndrome

Adults

Diagnostic test

EPC type 2 (progressive

cause)

Age period

Repeated skin
examination

Neurocysticerosis

Immunoelectrotransfer
blot assay

(Tick-borne) encephalitis

Cerebrospinal fluid study

serologic test for virus
Serum glucose, urinary
ketones

Gliomatosis cerebri
Other foreign tissue
lesions Nonketotic
(ketotic) hyperglycemia
Cerebrovascular
disorders (stroke;
intracranial bleeding,
cerebral venous
thrombosis, vasculitis)
Nonketotic (ketotic)
hyperglycemia
Focal cortical dysplasia

Paraneoplastic limbic
encephalopathy

Neoplasms
Tuberculous meningitis
(tuberculoma)
(Tick-borne) encephalitis

Autoimmune thyroid
encephalopathy

Behcet disease

Sjogren syndrome

Multiple sclerosis

HIV encephalopathy

Serum glucose

Diagnostic test
Serum and cerebrospinal
fluid lactate, muscle
biopsy biopsy,
mitochondrial DNA
(mutation of POLG1)
Cerebrospinal fluid
oligoclonal banding,
immunoglobulin G index

Mitochondrial
encephalomyopathy, lactic
acidosis and stroke-like episodes
(MELAS)
Delayed type of measles
encephalitis (complication of
measles in
immunocompromised children)

Serum and cerebrospinal

fluid lactate, muscle
biopsy, mitochondrial
DNA
Immunosuppresive
treatment, contact with
measles

Adult-onset Rasmussens
syndrome

Cerebrospinal fluid
oligoclonal banding,
immunoglobulin G index

Creutzfeldt-Jakob disease

14-3-3 protein in
cerebrospinal fiuid
Serum and cerebrospinal
fluid lactate, muscle
biopsy, mitochondrial
DNA
Skin or rectal mucosal
biopsy

Myoclonus epilepsy with ragged

red fibers (MERRFs)

Cerebrospinal fluid study,

chest computed
tomography, anti-Hu
test

Kufs disease

Cerebrospinal fluid study,

chest XR, tuberculin skin test
Cerebrospinal fluid
study, serologic test for
virus
Thyroid function tests,
antithyroglobulin
antibody,
antimicrosomal antibody
Neuroimaging, recurrent
oral and genital
ulceration, skin lesions,
HLA-B5 positivity
Hypergammaglobulinemia
positive antinuclear
antibody, anti-SSA, SSB,
rheumatoid factor
Cerebrospinal fluid
oligoclonal banding
Immunoglobulin G index
Serologic test for HIV

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134
E. Trinka et al.
systemic diseases or CNS disorders. Table 5 shows the etiologic factors and EEG patterns found in coma-GED and
coma-LED. Unlike in AS and focal NCSE, these patients
are often resistant to treatment, and the prognosis depends
entirely on the course of the underlying disorder.
Advanced convulsive SE becomes oligosymptomatic with
fewer motor symptoms; deep coma is the leading symptom, hence the term subtle status (Treiman et al., 1984).
The causes are identical to convulsive SE.

Causes of Epilepsia Partialis

Continua
Epilepsia partialis continua (EPC) is a special type of
focal status epilepticus (Table 1), first described by
Kojewnikov in 1894; he considered it as a peculiar form
of cortical epilepsy. EPC is characterized by spontaneous regular or irregular clonic muscle twitching of cerebral cortical origin, sometime aggravated by action or
sensory stimuli, confined to one part of the body, and
continuing for a period hours days, or weeks (Obeso
et al., 1985). EPC, also called Kojewnikov syndrome,
occurred in patients with Russian springsummer tickborne encephalitis; EPC typically develops 23 weeks
after the end of the acute illness (Zemskaya et al., 1991).
EPC was later recognized to occur in several neurologic

Table 7. Mitochondrial diseases causing

status epilepticus
Alpers disease
Occipital lobe epilepsy/mitochondrial spinocerebellar ataxia and
epilepsy (MSCAE)
Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes
(MELAS)
Leigh syndrome
Myoclonic encephalopathy with ragged red fibers (MERRF)
Neuropathy, ataxia, and retinitis pigmentosa (NARP)

disorders, with age of onset ranging from infancy to adulthood, and even in the elderly. The prevalence, based on
the EPC case registry of the British Neurological Surveillance Unit, is <1 per million (Cockerell et al., 1996).
The reported causes of EPC are extremely diverse, and
include metabolic disorders (nonketotic hyperglycemia or
mitochondrial encephalopathy), cerebrovascular disorders, inflammation (especially CreutzfeldtJakob disease,
progressive multifocal leukoencephalopathy from HIV
infection, multiple sclerosis, neurocysticercosis and others), neoplasms (astrocytoma, hemangioma, lymphoma
and metastasis), and cortical dysplasia (focal cortical
dysplasia and hemimegalencephaly) (Oguni et al., 1991;
Veggiotti et al., 1995; Lee et al., 2000; Placidi et al., 2001;
Pandian et al., 2002; Huang et al., 2005; Wieser & Chauvel,
2005; Aydin-Ozdemir et al., 2006; Kinirons et al., 2006;
Sinha & Satischandra, 2007; Bien & Elger, 2008; Yeh &
Wu, 2008; Mameniskiene et al., 2011). The most common
etiologies are cerebrovascular disorders (2428%), inflammatory causes (1519%), neoplasms (516%), and metabolic disorders (614%). Despite full neurologic work-up,
1928% of EPC cases have no identifiable cause (Thomas
et al., 1977; Cockerell et al., 1996; Sinha & Satischandra,
2007). Table 6 gives an overview of the causes of EPC.
Bancaud divided EPC into two types: type 1, caused by
focal static lesions of the sensorimotor cortex, and type 2,
caused by progressive cerebral lesions with neurologic
and intellectual deterioration (Bancaud, 1992). Type 2
most often reflects mitochondrial diseases and inflammatory causes, such as Rasmussen syndrome or Creutzfeldt
Jakob disease.

Uncommon Causes of Status

Epilepticus
The uncommon causes of SE deserve consideration. In
many cases of drug-resistant SE, the underlying disorder
remains untreated, because rare causes may be overlooked. We do not know the frequency of these rare

Table 8. Uncommon infectious disease causing status epilepticus

Atypical bacterial infections
Bartonella/cat-scratch disease
Coxiella burnett (Q fever)
Neurosyphilis
Scrub typhus
Shigellosis
Mycoplasma pneumonia
Chlamydophilapsittaci

Epilepsia, 53(Suppl. 4):127138, 2012

doi: 10.1111/j.1528-1167.2012.03622.x

Viral infections

Prion disease

Other infections

HIV and HIV-related infections

West Nile encephalitis
JC virus (progressive multifocal
leukoencephalopathy)
Parvovirus B19
Varicella encephalitis
Subacute sclerosing panencephalitis
Measles encephalitis
Rubella encephalitis
Rous sarcoma virus
(RSV) associated SE
Polioencephalomyelitis
St. Louis encephalitis

Creutzfeldt-Jakob disease

Paracoccidioidomycosis
Paragonimiasis
Mucormycosis

135
Causes of Status Epilepticus
Table 9. Status epilepticus due to genetic diseases
Chromosomal aberrations

Inborn errors of
metabolism

Malformations of
cortical development

Neurocutaneous
syndromes
Sturge-Weber
syndrome
Tuberous sclerosis

Ring chromosome 20

Porphyria

Angelman syndrome

Menkes disease

Focal cortical
dysplasias
Hemimegalencephaly

Wolf-Hirschhorn syndrome

Wilsons disease

Polymicrogyria

Fragile X syndrome
X-linked mental retardation
syndrome
Ring chromosome 17

Alexanders disease
Gobalamin C/D
deficiency
Ornithine transcarbamylase
(OTC) deficiency
Hyperprolinemia
Maple-syrup urine disease
3-Methylcrotonyl CoA
carboxylase deficiency
Lysinuric protein intolerance
Hydroxyglutaric aciduria
Metachromatic
leukodystrophy
Kufs disease
Late infantile ceroid
lipofuscinosis
Beta-ureidopropionase
deficiency

Heterotopias
Schizencephaly

3-Hydroxyaxyl
CoA dehydrogenase
deficiency
Carnitine
palmitoyltransferase
Succinic semildehyde
dehydrogenase deficiency

causes, and the available literature is mostly confined to

single case reports or small case series. This overview is
based on a systematic search of all available English literature between 1990 and 2008 (Tan et al., 2010). The
authors identified 181 causes of SE after reviewing 513
articles. The causes fell into five categorical groups:
1.
2.
3.
4.
5.

Immunologically mediated disorders (Table 4).

Mitochondrial diseases (Table 7).
Uncommon infective disorders (Table 8).
Genetic disorders (Table 9).
Drugs or toxins.

Table 10 lists other uncommon causes of SE. It cannot

be overemphasized that the knowledge of the range of
conditions is important to clinical practice; the underlying
disorder must be treated to achieve full seizure control.
The tables are derived from the article by Tan et al. (2010)
and reflect the current state of knowledge on these causes
of SE.

Others
Retts syndrome
Dravet syndrome and
SCN1A gene mutation
spectrum
Migrating partial seizures
in infancy
Pyeridoxine dependency
Familial hemiplegic
migraine
Laforas disease
Dentato-rubro-pallido-luysian
atrophy
Infantile-onset
spinocerebellar ataxia
Wrinkly-skin syndrome
Neurocutaneous
melanomatosis
Neuroserpin mutation
Wolfram syndrome
Autosomal recessive
hyperekplexia
Cockayne syndrome
Cerebral autosomal
dominant arterio-pathy
with subcortical infarcts
and leuko
encephalopathy
(CADASIL)
Jeavons syndrome
Robinow syndrome
LYK5 mutation
MECP2 mutation
Malignant hyperpyrexia

Therapeutic Considerations
Given the wide variety of clinical presentations of SE,
ranging from life-threatening conditions to seemingly
harmless ones, it is important to tailor the treatment to the
type of status and to the underlying disorder. The clinical
presentation of SE determines the aggressiveness treatment. All treatment concepts on convulsive SE are based
on a staged approach (Fig. 4). In the early phase of convulsive SE, large randomized controlled trials support the use
of intravenous benzodiazepines. Alternatively, the intramuscular route is effective in the prehospital setting
(Silbergleit et al., 2012). In stage two, AEDs were used,
but it must be emphasized that there are no clinical trials
to inform the best drug treatment at this stage (Cock and
ESETT Group, 2011). Phenytoin, levetiracetam, and valproic acid are most often used (Shorvon et al,. 2008). The
newer AED lacosamide has also gained acceptance in the
community, but data on its effectiveness in convulsive
Epilepsia, 53(Suppl. 4):127138, 2012
doi: 10.1111/j.1528-1167.2012.03622.x

136
E. Trinka et al.
Table 10. Other causes of status epilepticus
Iatrogenic

Other medical conditions

Electroconvulsive therapy
Temporal lobectomy and
other neurosurgery
Insertion of intracranial electrode
Ventriculoperitoneal shunt
Blood transfusion
Carotid angioplasty and stenting

Multiple sclerosis
Hypertension-induced posterior
reversible encephalopathy
syndrome
Panayiotopoulos syndrome
Thyroid disease
Pyridoxine-dependent seizure
Neuroleptic malignant syndrome
Ulcerative colitis
Behcet syndrome
Celiac disease
Cobalamin deficiency
Folinic acid responsive seizures
Renal artery stenosis
Pituitary apoplexy
Renal artery dissection
Hypomelanosis of Ito
Cerebral palsy
Hemophagocytic
lymphohistiocytosis
Anhidrotic ectodermal dysplasia
Methemoglobinemia

Figure 6.
Treatment algorithm for superrefractory status
epilepticus. Modified after Shorvon & Ferlisi, 2011.
Epilepsia ILAE

ning of status (Shorvon et al., 2008). There is general

agreement that AS, dyscognitive status and other forms of
focal nonconvulsive SE do not require the same aggressive treatment as convulsive SE. Most patients in AS
respond promptly to a benzodiazepine or valproic acid. In
focal NCSE the potential risks of treatment, especially
intubation and sedation, must be weighed against the benefits of seizure control in preventing neuronal damage and
long-term consequences.

Disclosure of Conflict
of Interest

Figure 5.
Staged approach to the treatment of convulsive status
epilepticus. *There is currently limited evidence for the
use of lacosamide in SE (see Hofler et al., 2011) Modified after Trinka, 2007; Shorvon et al., 2008.
Epilepsia ILAE
status are limited (Hfler et al., 2011; Trinka, 2011). From
stage three onward, intensive care unit treatment and general anesthesia are the mainstays of drug treatment. Again,
there are no randomized controlled trials informing the
effectiveness of individual drugs in this stage. Midazolam
seems to be better tolerated than pentobarbital/thiopental
and propofol (Shorvon & Ferlisi, 2012). A fourth stage of
status was recently introduced (Shorvon & Trinka, 2011),
and a treatment protocol was proposed by Shorvon and
Ferlisi (2011). Needless to say, all general measures for
intensive care treatment have to be applied at the beginEpilepsia, 53(Suppl. 4):127138, 2012
doi: 10.1111/j.1528-1167.2012.03622.x

AZ has no conflict of interest to declare. JH has received speakers

honoraria from UCB and travel grants from UCB, Eisai, and Gerot. ET
has acted as a paid consultant to Eisai, Medtronics, Bial, and UCB. He
has received research funding from UCB, Biogen-Idec, and Sanofi-Aventis, and speakers honoraria from Bial, Cyberonics, Desitin Pharma, Eisai,
Gerot, Bhringer, Sanofi, Medis, and UCB. We confirm that we have
read the Journals position on issues involved in ethical publication and
affirm that this report is consistent with those guidelines.

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