10.1097@00004691 200409000 00003
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size of the seizure focus is small, or show nonspecific abnor- serious medical conditions. However, some investigators
malities. If there is an ictal pattern, it can contain any have used NCSE to include all the above-described patients,
frequency. Evolution of the discharge is common; the dis- ranging from those with barely detectable mental status
charges may wax and wane. At other times the EEG may changes to those in coma. I prefer to subdivide NCSE based
show repetitive spikes or spike and wave activity. Noncon- on clinical features, such as degree of impairment of con-
vulsive forms of SPSE will be discussed later. sciousness (Kaplan, 1999). Initially, ambulatory forms of
NCSE will be reviewed.
NONCONVULSIVE STATUS EPILEPTICUS
Nonconvulsive status epilepticus is a more heteroge- Ambulatory Patients
neous and controversial entity than is CSE. Within this group, Nonconvulsive status epilepticus is defined as an epi-
the term absence status or petit mal status is usually used for leptic state lasting more than 30 minutes with some clinically
those patients with generalized EEG findings who are ambu- evident change in mental status or behavior from baseline and
latory and may appear confused, with repetitive automatisms, ictal activity on EEG. There are two major categories of
while those with a partial EEG onset have been described as NCSE based on EEG: generalized and partial. Numerous
complex partial status epilepticus (CPSE). The term NCSE is terms have been used to describe generalized NCSE in
more often applied to patients who are severely obtunded or patients who are often confused but ambulatory (Shorvon,
comatose with minimal or no motor movements, often with 1994); absence status is the most widely used. The EEG is
extremely useful in the identification of NCSE, particularly focal discharge that may characterize complex partial sei-
when the cause of this acute confusional state is not clinically zures can range from the delta to beta frequency (Figs.
apparent. A variety of EEG patterns have been described in 6A– 6C). The EEG findings may show recurrent focal sei-
generalized NCSE. Most commonly, the EEG shows more or zures or a more continuous cycling with alterations of unre-
less continuous, or frequently recurring, generalized, bilater- sponsiveness and partially responsive phases. Rarely, aphasia
ally synchronous, symmetric spike and wave, which may be may be the sole manifestation of partial SE (Kirshner et al.,
at a frequency of 3 Hz, but is often less (Agathonikou et al., 1995). It can be difficult to determine if there is an alteration
1998; Baykan et al., 2002; Gastaut and Tassinari, 1975; of consciousness in such patients. If consciousness is not
Granner and Lee, 1994; Shneker and Fountain, 2003), or impaired, then aphasic SE can be included with SPSE, rather
generalized polyspike and wave discharges. Figures 5A and than with CPSE.
5B demonstrate a twilight state (absence status) in a young At times NCSE can be difficult to classify (generalized
man. versus. partial), even with ictal EEG recordings. In particular,
In contrast to absence status, in CPSE the EEG abnor- frontal seizures may show a generalized pattern on EEG
malities usually are focal, most often affecting the temporal (Thomas et al., 1999). Guberman et al. (1986) hypothesized
area, although they can become generalized. The focal EEG that transitional cases of NCSE exist with lateralizing EEG
abnormalities may not be appreciated, particularly if the features and that some cases are probably due to secondary
recording begins after the onset of the status. The rhythmic generalization from a temporal or frontal focus. Tomson and
colleagues (1992) concluded that most cases of NCSE rep- (Treiman et al., 1984), electrographic SE (Simon and
resent CPSE. However, Granner and Lee (1994), in a large Aminoff, 1986), SE in comatose patients (Lowenstein and
series of patients with NCSE (85 ictal episodes in 78 pa- Aminoff, 1992), generalized electrographic SE (Drislane
tients), found that 69% of the episodes were generalized on and Schomer, 1994), nontonic-clonic SE (Privitera et al.,
EEG, 18% were generalized with focal predominance, and 1994), subclinical SE (So et al., 1995), and NCSE (De-
13% were focal. The difficulty in distinguishing absence Lorenzo et al., 1998; Kaplan, 1999; Litt et al., 1998;
status from CPSE may, in part, explain the differences in Towne et al., 2000). Sometimes the same term has been
interpretation of NCSE after metrizamide myelography, used when describing different disorders, whereas differ-
which has been described as either generalized (Obeid et al., ent terms are often applied to the same clinical entity.
1988) or complex partial status epilepticus (Elian and Fen- These cases are often difficult to diagnose and the diag-
wick, 1985). nosis is usually delayed. Patients may display subtle,
Obtunded/Comatose Patients intermittent, focal, or multifocal rhythmic movements sug-
Patients may be encephalopathic because of ongoing gestive of seizures (Treiman, 1995). Hussain et al. (2003)
epileptic activity with minimal or no motor movements. noted that certain clinical features were more likely to be
This entity, NCSE, is difficult to diagnose in obtunded/ present in patients with NCSE compared with other types
comatose patients (Brenner, 2002; Lawn and Wijdicks, of encephalopathy. These included either remote history of
2002; Walker, 2001). A variety of terms have been used to seizures or ocular movement abnormalities. NCSE can
describe these patients, including subtle generalized SE occur in a variety of disorders, including hypoxia, meta-
bolic disturbances (Drislane and Schomer, 1994; Lowen- periodic epileptiform discharges (PEDs), which can be either
stein and Aminoff, 1992; Simon and Aminoff, 1986), and focal or generalized, and generalized TWs.
after convulsive seizures (Treiman et al., 1990). Periodic lateralized epileptiform discharges (Fig. 7A) oc-
cur in a variety of disorders, most often acute unilateral lesions,
EEG PATTERNS such as infarcts or tumors. They may also be seen in patients
A number of EEG patterns have been described in NCSE, with chronic seizure disorders or old static lesions, especially
and many of these are controversial, particularly as to whether when associated with recent seizures, alcohol withdrawal, or a
they are ictal. These include periodic lateralized epileptiform toxic-metabolic disorder. Chatrian et al. (1964) described
discharges (PLEDs), bilateral independent PLEDs (BIPLEDs), PLEDs as consisting of lateralized complexes usually recurring
every 1 to 2 seconds. The complexes often consist of sharp lesser degree over homologous areas in the contralateral
waves or spikes that may be followed by a slow wave. The hemisphere. In contrast, in patients with BIPLEDs, the com-
clinical picture associated with PLEDs is usually obtundation, plexes are asynchronous, usually differing in morphology,
focal seizures, and focal neurologic signs (Garcia-Morales et al., amplitude, rate of repetition, and site of maximal involvement
2002). The majority of patients with PLEDs will have seizures (Fig. 8). De la Paz and Brenner (1981) reported clinical
during the acute stage of illness and the PLEDs usually will be findings in 18 patients whose EEGs showed this pattern. The
transiently replaced by a new pattern, often consisting of faster most common cause of BIPLEDs was hypoxic encephalop-
rhythmic activity (Fig. 7B). For this reason PLEDs are usually athy (five), central nervous system (CNS) infection (enceph-
considered an interictal pattern, although not all agree (Assal et alitis or meningitis) (five) and chronic seizure disorders
al., 2001; Garzon et al., 2001; Handforth et al., 1994). In several (four). When compared with patients with PLEDs, those with
studies of NCSE, PLEDs alone were not considered an ictal BIPLEDs were more likely to be comatose (72% versus 24%)
pattern (Claassen et al., 2001; Jordan, 1999; Jaitly et al., 1997). and had a higher mortality rate (61% versus 29%), but focal
Pohlmann-Eden et al. (1996) viewed PLEDs as an electro- neurologic deficits and focal seizures were less common.
graphic signature of a dynamic pathophysiological state in Generalized periodic bisynchronous sharp complexes,
which unstable neurobiological processes create an ictal-interic- often with a triphasic configuration, have been described in a
tal continuum. variety of drug-related disorders, metabolic encephalopathies
Periodic lateralized epileptiform discharges are lateral- (Yemisci et al., 2003), after anoxia-ischemia (Brenner and
ized; however, they are often reflected synchronously to a Schaul, 1990), and in convulsive status epilepticus (Treiman
et al., 1990). A similar pattern may be seen with degenerative fronto-occipital lag may be present. The initial negative compo-
diseases, such as Creutzfeldt-Jakob disease. As noted earlier, nent is the sharpest, whereas the following positive portion of the
Treiman and colleagues (1990) described a progressive se- complex is the largest and is subsequently followed by another
quence of EEG changes during generalized CSE. Whether the negative wave (Fig. 10). TWs may increase with stimulation;
final pattern of this proposed sequence, namely PEDs with a this is also true of PLEDs and generalized PEDs (Hirsch et al.,
“flat” background (Fig. 9), should be considered ictal is 2004).
debatable (Brenner and Schaul, 1990; Garzon et al., 2001; When electroencephalographers use the term triphasic
Hirsch et al., 2004; Husain et al., 1999; Nei et al., 1999). waves, they are usually implying a pattern seen with a variety
Furthermore, although some authors have stated that PEDs of encephalopathies, particularly hepatic or renal dysfunction.
may represent NCSE, others view it as an encephalopathy However, the term triphasic can also be used to describe the
where spikes and sharp waves may not impair clinical func- morphology of the waveform in that sharp and slow wave
tion, but rather reflect damage from severe brain injury complexes usually have three phases; hence, they are TWs. It
(Krumholz, 1999; Niedermeyer and Ribeiro, 2000). can be difficult to distinguish TWs due to a toxic-metabolic
Another pattern described by some as consistent with encephalopathy from epileptiform sharp and slow waves
NCSE is TWs. These consist of bursts of moderate- to high- complexes, which may or may not be ictal. This is apparent
amplitude (100 to 300 uV) activity, usually of 1.5 to 2.5 Hz, when reviewing reports of patients who became confused
often occurring in clusters. Although frequently predominant in after treatment with baclofen (Hormes et al., 1988; Zak et al.,
the frontal regions, occasionally they are maximal posteriorly. A 1994) or postmetrizamide myelography (Drake, 1984; Elian
and Fenwick, 1985; Obeid et al., 1988). Some authors con- may straddle the borders between epilepsy and encephalop-
cluded that these patterns represented NCSE, whereas others athy and that the distinction between NCSE and encephalop-
viewed it as an encephalopathy that resolved with discontinu- athy can be difficult, whereas Litt et al. (1998) claimed that
ing the offending agent. Kaplan (1999) reported that TWs monorhythmic TWs could be distinguished from ictal pat-
terns. Clearly there are times when this distinction can be ments. An EEG is the only way to verify the diagnosis, although,
difficult; hence, the terms “triphasic-like waves” and nonepi- as has been emphasized, interpretation can be difficult. An EEG
leptiform “true TWs” (Husain et al., 1999). This topic is is also helpful in monitoring patients who are being treated for
further reviewed in this issue (Kaplan, 2004). SE, particularly because the neurologic examination is affected
Nonconvulsive status epilepticus consists of EEG-ictal by the administration of drugs, such as lorazepam, midazolam,
episodes, which are continuous or recurrent for greater than 30 propofol, pentobarbital, or neuromuscular blocking agents.
minutes without improvement in clinical state or return to Jaitly et al. (1997) emphasized the prognostic value of
preictal EEG pattern between seizures (Young et al., 1996). EEG monitoring after CSE and described patterns with a poor
There are no agreed-on criteria to diagnose NCSE in obtunded/ prognosis after CSE, including PLEDs, burst-suppression, and
comatose patients (Kaplan, 2003). Litt et al. (1998) described after-SE ictal discharges. Patients whose records normalized did
three EEG patterns of electrographic SE: focal (Fig. 11), gener- well. Nei et al. (1999) found that PEDs were associated with a
alized (Figs. 12A and 12B), and bihemispheric (Fig. 13). It is poor outcome independent of etiology. The investigators were
relatively easy to diagnose NCSE when there are frequent uncertain if this was an ictal pattern and concluded that it was
electrographic seizures, particularly when they are focal. The unclear whether PEDs required aggressive treatment.
problem is greater with generalized spike and wave and sharp
and slow wave complexes. The latter usually should not be Simple Partial Status Epilepticus
invariant, nor should changes, if present, be only state related. There are several different types of SPSE, in which, by
For proposed criteria of nonconvulsive seizures, which, in turn, definition, consciousness is preserved. These include convul-
help define NCSE, see Young et al. (1996). sive types (e.g., somatomotor, as discussed previously) and
The effects of medications, such as benzodiazepines, and nonconvulsive types (e.g., somatosensory), as well as psychic
their role in diagnosis are also debated. For example, a test dose and autonomic status and some cases of and aphasic SE.
of lorazepam may complicate matters because most EEG pat- Somatosensory symptoms may include visual hallucinations,
terns may resolve if the patient is given an adequate dose, field cuts, or ictal blindness (Figs. 14A–14C). Both psychic
including TWs due to a metabolic encephalopathy (Fountain and SE, the most common symptom of which is ictal fear (Shro-
Waldman, 2001). This can be attributed to a state change if the von, 1994), and autonomic SE, which may consist of pro-
pattern recurs after painful stimulation. Improvement in the EEG longed abdominal sensations (Gastaut, 1983; Manford and
after treatment does not prove that the discharges were ictal and Shorvon, 1992), are rare.
responsible for the patient’s decreased responsiveness. Clearly
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