NCSE is characterized clinically by subtle manifestations such as clouding of

consciousness, pseudodementia, or ataxia, no overt motor (convulsive)

manifestations, and electrically continuous or repetitive seizure patterns. It is
often difficult to notice, and controversial in its definition and classification (6, 12,
20, 27, 49). Electroencephalographic (EEG) findings have decisive importance,
showing continuous or nearly continuous electrographic seizure activity lasting
at least 30 minutes (55). The EEG criteria for diagnosing NCSE include discrete
electrographic seizures, continuous spike-and-wave activity, or rhythmic
recurrent epileptiform activity that usually shows marked improvement with the
injection of benzodiazepines. These EEG findings also differentiate generalized
from focal-onset NCSE (21).
Generalized NCSE (GNCSE), mainly absence status, is more often observed than
partial SE in NCSE occurring in chronic epileptics. In acute symptomatic SE,
however, nonconvulsive complex partial SE predominates, and its poor prognosis
necessitates intensive emergency treatment (55). Accordingly, it is important to
differentiate partial nonconvulsive SE from absence status, which has a better
prognosis. After prolonged generalized convulsive SE (GCSE) or following
incomplete treatment, convulsive symptoms become very subtle, and the
residual persistent disturbance of consciousness and autonomic symptoms may
be confused with transition to NCSE. A careful diagnosis of SE is importantand
should take into account the results of EEG monitoring (55). (SE, mech and
manag, 141)
Absence status Absence status (AS) is characterized by repetitive absences,
either typical or atypical, or prolonged clouding of consciousness, which varies
from light to profound stupor or confusion, together with diffuse spike-and-wave
activity on the EEG. Complex mental testing may be needed to detect absence
status with only mild mental slowing. Blinking, myoclonia of the upper
extremities, or automatisms may occasionally occur but are not distinctive
features of AS. This status, however, has terminologic and nosologic problems,
being variously called petit mal status, epileptic twilight state, ictal stupor, spike-
wave stupor, and minor epileptic status (2, 20, 39, 46). Electrographically,
bilaterally synchronous spike-and-wave patterns are repeated at very short
intervals or continue without interruption. In atypical ASE and some time after
the onset of typical AS, slow spikeand-wave patterns become predominant and
somewhat irregular. Atypical AS associated with Lennox-Gastaut syndrome or
myoclonic-astatic epilepsy is more often observed in childhood epilepsy (12, 20,
46). Petit mal status, typical AS, or spike-wave stupor.Formerly, we (39) proposed
reserving the term petit mal statusfor cases with closely repeated 3-Hz spike-
and-wave bursts corresponding to a series of petit mal absences and applying
the term spikewave stupor exclusively to those cases with uninterrupted
continuous 3-Hz spike-and-wave bursts and prolonged altered consciousness.
.
Typical ASE rarely occurs in childhood absence epilepsy but is somewhat more
frequent in other idiopathic forms of generalized epilepsy and in adults (20).
Minor epileptic status.Reporting findings in 22 children, Brett (7) differentiated
minor epiletic status from AS by the presence of myoclonus and less frequent
spike-and-wave activity intermingled with slow waves on EEG, and by its poor
prognosis. This syndrome is characterized clinically by periodic or fluctuating
lowering of awareness and responsiveness and decreased speech, sometimes so
severe as to simulate dementia (i.e., pseudodementia), and is often associated
with lack of coordination and unsteadiness in walking that resembles ataxia (i.e.,
pseudoataxia) because of the intrusion of repeated small myoclonic jerks or
twiches.
Because both negative and positive myoclonus are observed, Bretts cases (7)
may include or be combined with some type of ESES syndrome or myoclonic
status.
Angelmans syndrome is often accompanied by NCSE, which in most cases may
be this kind of minor epileptic status (9, 33, 50). It is mainly observed from late
infancy to early childhood. NCSE with hypsarrhythmia.The most typical
continuous andlonglasting epileptic EEG abnormality is hypsarrhythmia,
observed in West syndrome. Infants with this condition become stuporous and
apathetic, and their development stagnates or deteriorates. Thus, the twilight
state in hypsarrhythmia may be regarded as a form of NCSE (12, 46).
NCSE in myoclonic-astatic epilepsy and Lennox-Gastaut syndrome.
NCSE is reported in 30%40% of patients with myoclonicastatic epilepsy in
childhood (12). It is characterized by apathy or stupor and slurred or decreased
speech, and 2 3 Hz spike-and-wave bursts on the EEG. There is palpable
irregular twitching of the facial muscles and the extremities, and astatic seizures
and head nodding can appear serially (12). This status of minor seizures may be
a mixed type of atypical AS, myoclonic status, or myoclonic-astatic status.
More than two-thirds of patients with Lennox-Gastaut syndrome experience SE
(4), which usually consists of clouding of consciousness with or without
intermixed serial tonic seizures of short or long duration Myoclonic status
Myoclonic status is usually classified as a form of convulsive status (20) and
subdivided into idiopathic and symptomatic generalized epilepsies. Rhythmic
myoclonic jerks occur in the generalized forms and appear mainly in proximal
muscles. The level of consciousness depends on the underlying disorders, and
sometimes there is no clouding (20). Diffuse polyspike-and-wave discharges
precede the myoclonic jerks. It may be reasonable to classify this status as NCSE
when jerks or twiches are very small and clouding of consciousness is a more
prominent feature (20). Myoclonic status in nonprogressive encephalopathies as
described by Dalla Bernardina et al. (9) belongs to this kind of NCSE and
probably overlaps with atypical ASE, minor epileptic status, or obtunded status.
Nonconvulsive myoclonic status is also observed in eyelid myoclonia with
absences.
Atonic and akinetic SE This rare form of SE is peculiar to very young children
during hyperthermia and is characterized by immobility (without or with tonus),
sometimes with a few minor clonias, and unconsciousness. The EEG shows an
epileptic recruiting rhythm progressively mixed with delta activity, forming some
spike-and-wave patterns (20).
Simple partial SE Nonconvulsive simple partial SE (SPSE) by definition should not
be associated with alterations of consciousness, and is more often neocortical
and extratemporal in origin (46, 60). Rare cases with somatoinhibitory SE (ictal
paralysis), somatosensory SE, visual SE (elementary visual phenomena,
amaurotic, visual hallucination), auditory SE, dysphasic/aphasic SE, SE with pure
transient amnesia (59), autonomic/vegetative SE, and SE with expression of fear,
depression, and anxiety have been reported (46, 60).
Complex partial SE Complex partial SE (CPSE) often originates from temporal or
frontal lobe foci, but on rare occasions it originates from other cortical areas; an
example is visual hallucinatory SE (46). Clinically, CPSE is characterized by
recurrent or prolonged seizures involving some alteration of contact with the
environment, of memory or of consciousness, but it usually stops short of coma.
Automatisms may be observed, but not convulsive movement. Its fundamental
characteristic, which enables differentiation of CPSE from other twilight states
such as AS, is a cyclicity that alternates between total unresponsiveness with
stereotyped automatisms and partial unresponsiveness with reactive
automatisms (46, 56).
ESES syndrome Among the NCSE conditions, ESES (26, 29, 42) is a peculiar
pathologic condition that is observed only in childhood epilepsy. Epilepsy with
continuous spike waves during slow-wave sleep (CSWS) (8) characterized by
ESES has many interesting electroclinical characteristics. However, its
pathophysiology and the points of discriminating epilepsy with CSWS from
related conditions, such as ESES syndrome (37), are still unclear (2, 10, 18, 19,
58). Some authors prefer to use the term CSWS syndrome (2, 19). ESES
syndrome and its developmental aspects are discussed later in the chapter.
Landau-Kleffner syndrome Active epileptic discharges appearing predominantly
from the temporal areas can cause acquired aphasia, mainly due to auditory
agnosia, in children ages 211 years (2, 48, 53). Epileptic seizures such as
generalized tonic-clonic, focal motor seizures, or atypical absences usually
appear before or after the onset of decreased speech, but there are no seizures
in 17%30% of cases (2). Intelligence usually is preserved, but behavior
disorders are often observed. Epilepsy, aphasia, and epileptic EEG abnormalities
disappear by the age of 15. This is understandable in the context of ESES
syndrome (43, 48, 53), as ESES/CSWS is observed, but the spike-wave index
during slow-ware sleep is relatively low, about 30% or more (SE, mech and
manag

Nonconvulsive status epilepticus (NCSE) has been divided into two groups,
largely along electroencephalographic criteria: (1) absence status epilepticus
(ASE), a form of generalized nonconvulsive status epilepticus (GNSE), and (2) a
lateralization-related nonconvulsive state, referred to as complex partial status
epilepticus (CPSE). Each of these groups has been subdivided further. GNSE
includes (1) patients with a history of childhood absences characterized by three-
per-second spike-andwaves (ASE); (2) patients with childhood-onset, secondary
generalized epilepsy, usually with mental retardation, more marked confusion
and myoclonus; (3) elderly patients presenting de novo, usually in association
with a toxic or metabolic dysfunction, psychotropic medications or
benzodiazepine withdrawal, or triggered by a tonic-clonic seizure; and (4)
generalized nonconvulsive status epilepticus from partial onset of temporal or
frontal origin (SE clinical perspect 207)
Typical and atypical ASE have been described as petit mal status, minor epileptic
status, spike-wave stupor, epileptic twilight state, prolonged epileptic
twilightstate,absence continue, epilepsia minoris continua, ictal psychosis, status
pyknolepticus, and tat de mal a lexpression confusionnelle. Typical absence
status epilepticus (TAS) may be recognized initially only in a minority of patients
(19%) and is often misdiagnosed as CPSE, postictal confusion, depression,
posttraumatic amnesia, hysterical behavior, schizophrenia, or toxic
states(43,45). Three-quarters of the cases appear before the age of 20 yr, and in
a third, TAS heralds epilepsy (45). The typical clinical features described in
absence status epilepticus are given in Table 5.
TAS starts abruptly without warning. Typical features include perioral myoclonus,
myoclonic eyelid twitching, mild to marked obtundation, bradyphrenia and
bradykinesia, and confusion (45). The change in responsiveness can be highly
variable, an illustration of which is provided in Fig. 1 (54). Verbal functioning is
usually preserved, but there may be poverty of speech and monosyllabic
answers. Amnesia is not invariably present, and many patients can describe their
experiences as they go into and remain in ASE
As noted, differentiating TAS from CPSE (EEG aside) can be challenging. Some
behavioral distinctions among absence, temporal lobe, CPSE, and frontal lobe
CPSE are given in Table 6. Contrasting with CPSE, TAS typically induces little
amnesia, and there is little postictal confusion after the event (43). In TAS, there
is usually little cycling between periods of unresponsiveness and partial
responsiveness as may be seen in CPSE. Intellectual impairment is usually mild
when compared with the severity of psychic symptoms
Atypical absence status epilepticus (AASE) has been described in patients with
mental retardation and Lennox-Gastaut syndrome. In such patients, there may
be
no precise onset or offset of status, and the interictal state may merge with the
ictal. This may produce relative changes in behavior, responsiveness, and
attention, making such states particularly difficult to identify. Patients are said to
have dysfunctional days with level of consciousness particularly affected. Such
changes in consciousness with AASE are given in Table 7. Unlike in TAS,
convulsions rarely herald or terminate AASE. About 50% of patients may have
perioral, facial, or limb myoclonus
Simple partial nonconvulsive status epilepticus (SPNSE) may be difficult to prove
(seealso Chapter 7). Although subjective symptoms may be striking, the scalp
EEG is frequently unrevealing. In effect, the argument is circular: Patients
diagnosed as having SPNSE musthave an EEG correlate. Depending on the
particular brain region involved, the symptoms differ (7,5659). Autonomic and
vegetative features may appear, including ictal fear, anorexia, weight loss (56),
and poorly described
visceral sensations (7). There may be mild confusion, bad-tempered behavior,
depression, or even suicidal ideation with an anterior temporal focus (57). A
temporal focus may also engender inchoate fright (58). More posterior ictal foci
in the temporo-parieto-occipital junction may induce adversive eye movements
with stepwise contraversive nystagmus (59). Right-hemisphere SE may be barely
perceptible by clinical examination, and only identified by careful
neuropsychologic testing (60). Occipital simple partial seizures can induce
macropsia (distortion of increased size) or micropsia (the inverse), misperception
of spatial orientation, hallucinations of animals, movie scenarios, or simple
patterns of color and light (61).
Transient cortical blindness may occur (62). Although simple partial SE is
frequently presumed in the absence of EEG evidence, all published cases are
supported by an EEG correlate

CPSE can broadly be described as abnormal behavior or level of consciousness

associated with lateralized seizure activity, with impairment of consciousness
ranging from almost nondiscernible clouding of certain higher cortical functions,
to coma. Arguably, the comatose cases associated with electrographic seizure
activity represent a different disease with different etiologies, management
decisions, and prognoses. These cases probably constitute a condition in which
severe brain damageor medical illness is associated with seizure activity as an
epiphenomenon. Contrasting with TAS, CPSE patients may be totally
unresponsive (68). Williamson describes a patient studied with depth electrodes
who clinically manifested verbal unresponsiveness, confusion, and head and eye
deviation, and yet localization lay in the hippocampus (64). Another example
included a patient with head deviation, arm stiffening, and mutism, but with
preserved alertness, with a supplementary motor area origin but subsequent
evolution to CPSE and unresponsiveness
Pada status epileptikus parsial kompleks lobus frontal biasanya kesadaran hanya
sedikit terganggu dan fluktuasi lebih sedikit. pasien bisa terjadi konfabulasi,
gambaran ironi, tawa dan senyum yang tidak cocok.
With frontal lobe CPSE (FLCPSE), there is generally a lesser impairment of
consciousness, and fewer fluctuations (40). Patients may confabulate, have an
ironic appearance, inappropriate laughter and smiling. Patients may appear
indifferent or broodingall characteristics less commonly seen with temporal
lobe CPSE (TLCPSE). Less frequently, fear, anxiety, anger, irritability, negativism,
aggressiveness, agitation, and simple and complex automatisms were seen;
these were more common with TLCPSE, as was psychomotor slowing

Neonatal nonconvulsive status epilepticus differs from its clinical expression at

other ages (seeChapter 17). Premature and term infants may show only mild
facial and limb jerking, eye deviation, eyelid fluttering, apnea, or autonomic
changes with movement suggestive of rowing, pedaling, swimming, or boxing
(7). Seizures lasting for days may be correlated with high-voltage slow EEG
activity, rhythmic activity, or burst suppression. Some electrographic seizures
have no clinical correlate.
Status epileptikus nonkonvulsiv neonatus berbeda dalam gejala klinisnya
dibanding usia yang lain. Pada bayi mungkin hanya menunjukkan kedutan kecil
pada wajah atau ekstremitas, deviasi mata, kedipan mata, apnea atau
perubahan otonom dengan gerakan-gerakan seperti berenang, bertinju,
bersepeda, atau mengayuh.
Epilepsi ensefalopati pada bayi atau sindrom ohtahara khasnya muncul spasme
tonik,fleksor atau ekstensor. Pada sindrom west kemungkinan sulit untuk
menegakkan diagnosa karena kondisi ensefalopati dan perubahan afektif.
TAS or CPSE, properly speaking, do not occur. Infantile epileptic encephalopathy
(EIEE) or Ohtaharas syndrome typically presents with greater flexor, extensor, or
tonic spasms (7). In the setting of West syndrome, diagnosis may be difficult
because of the underlying encephalopathy and fluctuations in clinical state. In
mildly retarded patients, there may be interruption of visual contact and
decrease in affective components, particularly with hypsarrhythmia. Oral
automatisms, eye blinking, hypersalivation, and apathy are described. With
myoclonic-astatic epilepsy, SE presents with apathy, ranging to stupor. There
may be facial muscle twitching, twitching of the limbs, salivation, blank facial
expression, and dysarthric speech (7). Lasting hours to weeks and often varying
with sleep-wake cycles, SE may occur shortly after awakening, with patients lying
obtunded in bed. With Lennox-Gastaut syndrome, or secondary generalized
myoclonicastatic epilepsy, there may be stupor, atonic spells, head nodding,
myoclonic jerks, and
myoclonias of the face (7).
NCSE in children with normal intelligence is rare but occasionally occurs with
benign Rolandic epilepsy, in which there are speech arrest, drooling, problems
with swallowing, weakness of the face, head deviation, and mild confusion (79
81). Nonconvulsive states may also occur over the occipital regions, with nausea,
anorexia, and visual hallucinations.
NCSE pada anak dengan intelegensi normal jarang dijumpai, namun sering
didapatkan dengan epilepsi rolandik dimana terdapat henti bicara mendadak,
keluar air liur, gangguan menelan, kelemahan pada wajah, deviasi kepala dan
kebingungan ringan. Kondisi nonkonvulsiv juga dapat muncul pada regio oksipital
dengan mual, anoreksia dan halusinasi visual.
(SE clinical perspect)

NCSE should obviously be rapidly treated because of the acute neurologic

impairment of the patients, because of the attendant morbidity including
physical injury, and because it may evolve on to generalized convulsions.
There is reasonable concern about possible long-term effects, but
longterm permanent neurologic damage from NCSE occurring in
ambulatory patients has not yet been firmly documented as a mandate for
aggressive treatment (How urgent is the treatment of
nonconvulsive)
NCSE seharusnya mendapatkan pengobatan segera karena pengaruh
neurologis akut, meningkatnya morbiditas termasuk luka fisik dan dapat
menjadi kejang general. (How urgent is the treatment of
nonconvulsive)
(Non-convulsive status epilepticus; the rate of occurrence in a
general hospital)
Sebuah penelitian mencari etiologi yang memicu timbulnya NCSE pada
rumah sakit mereka. dari 42 pasien yang didiagnosa NCSE, sebanyak 38%
disebabkan hipoksia-anoksik, 20% akibat perdarahn intra kranial, 7%
akibat stroke iskemik, sedangkan yang disebabkan tumor,ensefalitis,
sepsis dan multiple sklerosis masing-masing 5%, sebanyak 10% idiopatik,
sedangkan sisanya kurang dari 5% akibat komplikasi transplantasi hati,
DAVF dan kriptogenik. (Non-convulsive status epilepticus; the rate
of occurrence in a general hospital)

The etiologies of NCSE most

frequently identified by neuroimaging are stroke, intracranial tumor, sinus thrombosis
and traumatic contusions

)atlas cross)