The term "aura" is usually referred to that portion of a seizure
experienced before loss of consciousness occurs and for which memory is
retained. In the case of simple partial seizure, the aura is the entire
seizure; but where consciousness is subsequently lost, the aura is, in
fact, the first symptom of a psychomotor or complex partial seizure (Dreifuss
1997).
To the best of my knowledge, Scott and Masland first described somatosensory
hallucinations as a "continuous symptom" of an "aura continua”
(Scott and Masland 1953). The term "aura continua" can be found
in Karbowski as a synonym for continuous psychomotor status (Karbowski
1985; Wieser 2001). Wolf used it as synonym for "status epilepticus
of focal sensory seizures" or for hallucinosis (Wolf 1970; 1980;
1982).
On the other hand, in a recent glossary of epilepsy terminology (Kaplan
et al 1995) under the term “aura,” one can read, "Although commonly
used this term has been ‘officially’ discarded since 1973. . ."
Wieser describes the problems with the terminology of nonconvulsive status
epilepticus, including aura continua (Wieser 2001). Aura continua is a
subtype of nonconvulsive status epilepticus and is synonymous with simple
partial status epilepticus.
CLINICAL MANIFESTATIONS
Long-lasting epileptic phenomena can be motor, nonmotor, or both
(Blum 2002). Although it is reasonable to assume that all types of simple
partial status epilepticus (SPSE) with motor phenomena are associated
with some kind of somatosensory phenomena, we feel that the term aura
continua should be restricted to those subjective feelings without visible
motor phenomena because the involuntary movement is sensed. Thus, we exclude
here SPSE with motor phenomena or any other objective phenomena such as
aphasia.
Dysphasic or aphasic SPSE and the syndrome of acquired epileptic aphasia
(Landau-Kleffner syndrome) and its relationship with electrical status
epilepticus during slow sleep in children is now listed under "epilepsy
with continuous spike-waves during slow wave sleep” (Wieser 2001).
From a clinical point of view, aura continua can be classified into 4
types: (1) somatosensory (ie, dysesthesia phenomena that involve the trunk,
head and extremities); (2) aura continua that involve the special senses
(ie, visual, auditory, vertiginous, gustatory and olfactory); (3) aura
continua with predominantly autonomic symptoms; and (4) aura continua
with psychic symptoms (VanNess et al 1997).
A special subtype in children, the so-called abdominal aura continua
(abdominal epilepsy, recurring abdominal pain) has been described (Schaeffler
and Karbowski 1981; Mitchell et al 1983; Peppercorn and Herzog 1989).
Finally, the French authors have used a category "erratic.” Under
this category, rare other manifestations and boundary conditions can be
summarized (Wieser 1997).
Although well localized (or hemi-) dysesthesia as a cortical phenomenon
of ongoing ictal discharges is likely to exist; convincing evidence (ie,
support by clear-cut EEG findings) is scanty. Pain as an epileptic aura
(Whitty 1953), and painful epileptic seizures are likewise uncommon but
are described (Wilkinson 1973; Young and Blume 1983; Siegel et al 1999).
Direct evidence that long-lasting pain occurs as aura continua (ie, as
a special form of partial status epilepticus) is also scanty, but this
possibility should not be discarded. Certain forms of pain per se might
be closely linked to epileptic basic phenomena (Fromm et al 1987), and
the positive therapeutic effect of antiepileptic drugs in such circumstances
is well-known.
Aura continua with elementary visual phenomena has been encountered by
Gastaut, and prolonged complex visual hallucinations were described by
Gastaut, and Sowa and Pituck (Gastaut 1983; Sowa and Pituck 1989). Status
epilepticus amauroticus has been described by Barry and colleagues, and
ictal visual hallucinations with reversible postictal hemianopia with
anosognosia by Barry and colleagues, and Spatt and Mamoli (Barry et al
1985; Spatt and Mamoli 2000). Sheth and Riggs reported an unusual case
of a clinically silent occipital electrographic status epilepticus persisting
for more than 3 years in a 13-year old girl (Sheth and Riggs 1999). Hadjikoutis
and Sawhney (Hadjikoutis and Sawhney 2003) described a case with occipital
seizures presenting with bilateral visual loss.
Schiffter and Straschill as well as Wieser described aura continua musicalis
(Schiffter and Straschill 1977; Wieser 1980). Our patient was published
under the heading psychomotor status epilepticus because eventually, the
aura continua with musical hallucinations (the patient experienced a song
well-known and familiar to her) in "endless repetition" and
with stereo-EEG documented restricted epileptiform discharges near Heschl's
gyrus spread to the ipsilateral mesiobasal limbic structures, accompanied
by alteration of consciousness. The beginning of this electrical epileptiform
status activity was accompanied by musical hallucinations only. Most cases
with simple auditory hallucinations described in literature do not fulfill
the criteria of an aura continua (Penfield and Perot 1963; Karbowski 1980).
Blanke and colleagues (Blanke et al 2003) described a patient with epilepsy
(secondary to left parieto-temporal brain damage) suffering from the paroxysmal
unilateral experience of hearing a person in her near extrapersonal space
associated with a deficit in spatial auditory perception and other paroxysmal
disorders of somatognosia.
Limbic status with olfactory symptoms has been documented (Wieser 1982).
A "gustatory aura continua" was the leading symptom of case
4 in our 1985 paper (Wieser et al 1985), with left hippocampal status
activity in the depth EEG. It was also associated with subtle higher cognitive
deficits detected with a tachistoscopically presented lexical decision
task.
The main clinical features of autonomic seizures are abdominal sensations,
apnea, arrhythmias and bradyarrhythmias, chest pain, cyanosis, erythema,
flushing, genital sensations and orgasm, hyperventilation, lacrimation,
miosis/mydriasis/hippus, palpitations, perspiration, pilomotor excitation
("gooseflesh"), tachycardia, urinary urgency and incontinence,
and vomiting. Psychomotor partial seizures with autonomic symptoms as
the leading feature are well known (Wieser and Williamson 1993). Under
the category nonconvulsive status epilepticus, Rabending and Fischer (Rabending
and Fischer 1986) describe ictal bradycardia and asystole. Nishiguchi
and colleagues (Nishiguchi et al 2002) described a boy with occipital
lobe epilepsy showing prolonged QTc in the ictal ECG. Zijlmans and colleagues
(Zijlmans et al 2002) determined the prevalence of heart rate changes
and ECG abnormalities during epileptic seizures in 281 seizures in 81
patients. ECG abnormalities were found in 26% of seizures (44% of patients)
and long seizure duration increased the occurrence of ECG abnormalities.
Umbilical sensations in children (VanBuren 1963), long-lasting borborygmi,
widened pupils, pilomotor phenomena, goose-flesh or periodically shivering,
etc. have been described (Brody et al 1960; Wieser 1979; 1981; 1983a;
1983b; 1988; 1991; Wieser et al 1981; Stodieck and Wieser 1986). Certain
peculiarities of personality and behavior are often associated with such
conditions and, therefore, we have described such conditions in the context
of "limbic dyscontrol syndrome" (Girgis and Kiloh 1980; Wieser
and Landis 1983). It is our belief that autonomic phenomena usually are
associated with overt or subtle behavioral changes such as irritability,
fear, panic, and, sometimes, existential emptiness, or some other form
of pathological self-perception. A particularly rare ictal or status symptom
is aggression (Delgado-Escueta et al 1981).
Ictal depression and anxiety in temporal lobe epilepsy is far more frequent
(Weil 1956); Henriksen as well as McLachlan and Blume described a status
with fear as the outstanding clinical expression (Henriksen 1973; McLachlan
and Blume 1980). A large amount of literature exists on this topic (Trimble
and Bolwig 1992; Smith et al 1991). Ictal laughter (Mueller and Mueller
1980) is usually associated with hypothalamic pathology, mainly hamartoma,
in which gelastic seizures are the hallmark.
A rare ictal phenomenon is "hemicrania epileptica" (Isler et
al 1984; Andermann and Lugaresi 1987), which may last longer than 30 minutes
and, therefore, can then be labeled as a form of status epilepticus. Hemicrania
continua is a primary headache disorder that is characterized by a continuous
unilateral headache of moderate severity, exacerbations of severe pain
and complete responsiveness to indomethacin. In hemicrania continua, visual
auras may precede or accompany the pain exacerbations (Peres et al 2002).
Psychic seizures were referred to by Jackson as so-called dreamy state
including deja vu as well as ictal reminiscences (Jackson 1875; 1890;
1898; 1899). He subsequently referred to such events as "psychical.”
Gowers reported 25 patients with "psychical auras" with 10 having
an "emotional aura"; all of these had fear (Gowers 1881). Penfield
introduced the term "experiential" to describe such mental phenomena
and divided the patients’ past experience into illusions and hallucinations,
which could be predominantly visual, auditory, or both, or an "unclassified"
experience such as a dream, a flashback, or a memory without further description.
Lennox referred to 3 categories of "psychic seizures,” which are
(1) dream states, feeling of unreality or illusions; (2) hallucinations;
and (3) mild confusion or disorientation, a feeling of strangeness without
loss of memory and consciousness. Experiential means that the mental phenomena
have a relevance to the patients past; Gloor specified that they typically
combine elements of perception, memory, and affect (Gloor 1990). Mesial
temporal lobe seizures may present as anxiety disorders (Young et al 1995).
The principal ictal "psychic phenomena" may be listed as follows
(Fish 1997):
Theoretically, each part
of the cortex, and probably deep nuclei as well (Wieser et al 1998), can
give rise to long-lasting localized epileptiform discharges. According
to their functional specialization, the epileptic dysfunction of a localized
ganglionic structure of the brain may give rise to "positive"
or "negative" symptoms of a particular quality. The understanding
is that the symptoms and signs are often the result of the interpretation
of the not discharging "healthy" brain, which is confronted
with a pathological "bombardment" or an epileptic dysfunction
of a part of itself. This implies that the localization of the cortex
where symptoms are produced (the "symptomatogenic zone") and
the localization where seizures are generated (the primary "epileptogenic
zone") are not necessarily concordant.
The most common localization of sensory seizures that involve the trunk,
head, and extremities (somatosensory) as well as the special senses (visual,
auditory, vertiginous, gustatory and olfactory) are listed in Table 1
together with some references.
An intriguing question is whether certain brain regions predispose more
than others for such a circumscribed and long-lasting discharge behavior.
By analogy with epilepsia partialis continua (Wieser 2001) and psychomotor
or limbic status epilepticus (Wieser 2001), it is reasonable to assume
that certain brain regions are, in fact, predisposed to this discharge
behavior. From posttraumatic epilepsy, it is well-known that the central
and mesiotemporal lobe cortices are more seizure-prone than other cortices,
but it is less clear whether region-specific differences exist to limit
seizure discharges in time.
PATHOPHYSIOLOGY
An "aura continua" reflects the intrinsic epileptogenic properties
of a discharging epileptogenic focus that remains "well controlled".
Obviously, in such a condition there is no further progression or recruitment
(ie, no relevant increase of the number of epileptically involved neurons).
Metaphorically spoken, the "critical mass" necessary for the
spread of the discharge is not reached. To a certain degree, however,
waxing and waning occurs. The aura content is the product of the "interpretive
cortex" (Penfield 1959) dealing with this discharging focus.
Several of our patients who underwent selective amygdalohippocampectomy
(Wieser and Yasargil 1982) because of drug-resistant mesial temporal lobe
epilepsy, and in whom this operation was successful with no further habitual
seizures, experienced persisting auras in the first months after this
operation. Moreover, a few patients claimed that the feeling of an impending
seizure would, with some fluctuation of its intensity, persist for hours
or even days, and thus mimic an "aura continua.” In such cases, it
is likely to assume that the removal of amygdala and hippocampus led to
a suppression of the full blown psychomotor seizures, but that the temporal
and insular neocortex were still epileptically disturbed and able to produce
prolonged aura phenomena. The so-called "running down" phenomenon
of such postoperatively persisting auras lends further support to the
idea that for the full expression of psychomotor seizures, both the mesial
limbic and the neocortical cortices are necessary.
DIFFERENTIAL DIAGNOSIS
An ongoing continuous or recurrent intermittent epileptic
discharge might be suspected, and consequently proven, with EEG and response
to antiepileptic drug treatment in an epileptic person with known focal
pathology and prolonged aura symptoms consisting of phenomena that fit
well with the localization of the epileptic discharge. However, in the
absence of clear-cut EEG findings, an aura continua in particular (if
expressing itself with strange and unusual phenomena) might be difficult
to diagnose. Not infrequently convincing ictal discharges cannot be detected
without intracranial recordings. Such techniques, of course, are only
justified in the context of surgical epilepsy therapy. Long-lasting autonomous,
emotional, and psychic phenomena and personality, in which the mesial
temporal lobe (in particular the amygdala) and the insular and frontal
cortices are candidate areas for suspected discharges, pose a problem.
Discharges at such a localization are difficult to detect in routine scalp
EEG. Autonomic signs and symptoms occur in simple partial seizures and
are frequent in complex partial seizures. Prolonged autonomic ictal features
can mimic psychiatric, endocrine, cardiac, and gastrointestinal disorders
(Devinsky et al 1986). Cardiovascular and thoracic symptoms are not specific
for temporal lobe seizures, but are also seen in frontal lobe seizures,
and probably more commonly so. Pupillary dilatation is thought by some
authors to be a sign of hypothalamic seizure spread. Table 2 lists the
most important differential diagnosis of autonomic phenomena.
Table 2. Differential Diagnosis of Autonomic
Aura Continua
| • Endocrine disorders |
| |
- Carcinoid |
| - Pheochromocytoma |
| - Hypoglycemia |
| • Organic gastrointestinal disease |
| • Panic attacks |
| • Paroxysmal autonomic dysfunction |
(Liporace and Sperling 1997)
Table 3. Differential Diagnosis of Aura Continua
with Psychic Phenomena and Psychic Seizures
| • Normal experiences |
| • Migraine |
| • Psychoses |
| |
- Postictal |
| - Recurrent intermittent psychoses |
| - Schizophrenia |
| - Schizophrenia-like (Slater and Beard 1963) |
| • Psychological disorders |
| |
- Panic attacks |
| - Hyperventilation attacks |
| - Other psychiatric conditions |
| • Hallucinations/illusions due to a loss of
primary sense |
| • Drug-induced flashbacks |
| • Cardiovascular disorders |
| • Sleep disorders |
| |
- Nightmares |
| - Night terrors |
| - REM sleep behavior disorder |
| • Metabolic disorder |
| |
- Recurrent drug abuse |
| - Hypoglycemia |
| - Porphyria |
| • Acute CNS disorders |
(Liporace and Sperling 1997)
DIAGNOSTIC WORKUP
To diagnose aura continua, two principal requirements have to be fulfilled:
(1) some clinically evident alteration in mental status such as hallucinations
and illusions or behavior from baseline; and (2) seizure activity on the
EEG. Correlating mental, autonomic or emotional changes from baseline
with EEG evidence of ongoing epileptic activity is essential to diagnose
aura continua. Polygraphic recordings, such as electrocardiography, respiration,
and electrodermal skin responses are helpful for detecting and quantifying
autonomic phenomena. If inaccessible for routine scalp electrodes, and
if depth recording is not available, ictal SPECT or ictal PET are helpful
for the diagnosis. Ictal SPECT and ictal PET are easy to obtain in a status
condition. Finally, the prompt response to antiepileptic drugs might be
important for the diagnosis. Since the majority of aura continua phenomena
are associated with some kind of lesion, a thorough examination of the
patient, including neurologic, neuropsychologic examinations, and MRI,
PET, or SPECT in the ictal and interictal state is essential. Polygraphic
video-EEG documentation is recommended.
SYNDROMES AND DISEASES IN WHICH THE SEIZURE TYPE OCCURS
Aura continua might occur de novo, but in most instances it occurs in
patients with known focal epilepsies particular to temporal lobe epilepsy.
My own experience is that aura continua is more often seen in symptomatic
focal epilepsies with evident morphological focal brain pathology. It
might be that certain pathologies, such as certain tumors and focal cortical
malformations, are more often associated with aura continua than other
pathologies, but clear evidence from literature is missing. Olfactory
or gustatory auras often are associated with tumors in the anterior temporal
lobe (Hausser-Hauw and Bancaud 1987).
PROGNOSIS AND COMPLICATIONS
Aura continua appears to cause no lasting clinical negative sequelae.
This contrasts with psychomotor status where the situation is less clear.
Although most reported cases with psychomotor status have returned to
baseline neurologic function (Mayeux and Lueders 1978; Cockerell et al
1994), several well-described patients have had prolonged memory deficits
(Engel et al 1978; Treiman and Delgado-Escueta 1983). Patients with electrographic
status epilepticus in the setting of serious medical illness have a poor
prognosis, but this is due most often to the underlying serious cerebrovascular
or other medical illnesses (Kaplan 1996; Aminoff 1998).
MANAGEMENT
As discussed above, there is no convincing evidence that aura continua
will lead to significant irreversible neurologic deficits. Therefore,
it has been argued that the standard and widespread aggressive use of
intravenous antiepileptic drugs is causing greater morbidity than its
sparing. With concurrent acute brain injury and aura continua, the situation
might be different. There is some experimental evidence that ongoing discharges
are synergistically harming the injured brain (Bogousslavski et al 1992;
Waterhouse et al 1998; Jordan 1999). Treatment, therefore, should find
a balance that considers the potential neurologic morbidity due to ongoing
focal discharges as well as the possible morbidity of IV antiepileptic
drugs (Kaplan 1999; Mikulecka et al 2000).
According to the literature, partial status epilepticus (Shorvon 1994)
is reported to be controlled by diazepam in 88% (of 67 patients). Clobazam
(Corman et al 1998), midazolam, an imidazobenzodiazepine, and lorazepam
(Mitchell 1996) are considered antiepileptic drugs of first choice; midazolam
is short acting and, therefore, can be well titrated on prolonged infusion
if necessary. Paradoxical response to diazepam and midazolam in partial
status epilepticus has been rarely observed (Al Tahan 2000). In children
with Ohtahara-like syndromes and serial partial seizures associated with
cortical dysplasia, pyridoxal phosphate showed good therapeutic efficacy
(Ohtsuka et al 2000).
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ILAE
ILAE Copyright Notice
Abbreviations
NCSE:nonconvulsive status epilepticus
SPSE:simple partial status epilepticus
Synonyms
Simple partial status epilepticus
Subtopics
Epileptic hallucination and illusions
Prolonged autonomic and psychic phenomena
Subclinical status epilepticus
Major keyword descriptors
autonomic changes
EEG seizure pattern
electroencephalographic status epilepticus
epileptic productive symptoms
focal sensory seizures
hallucinoses
limbic system
nonconvulsive status epilepticus
seizure discharges
seizures
simple partial status epilepticus
somatosensory hallucinations
status epilepticus
status hallucinosis
temporal lobe
uncinate fits
waxing and waning
Minor keyword descriptors
alteration in mental status
behavioral change
cognitive abnormalities
depth recording
ictal activity
loss of consciousness
ongoing discharge
Age of presentation
06-12 years
13-18 years
19-44 years
45-64 years
65+ years
Age of typical presentation
06-12 years
13-18 years
19-44 years
45-64 years
Permuted topic, synonyms,
variants
Aura continua
continua, Aura
illusions, Epileptic hallucination
hallucination and illusions, Epileptic
phenomena, Prolonged autonomic and psychic
psychic phenomena, Prolonged autonomic and
autonomic and psychic phenomena, Prolonged
status epilepticus, Subclinical
epilepticus, Subclinical status
partial status epilepticus, Simple
status epilepticus, Simple partial
epilepticus, Simple partial status
Related topics
Absence status epilepticus
Diazepam
Electrical status epilepticus during slow sleep
Epilepsy
Limbic status epilepticus (psychomotor status)
Typical absence seizures
Differential diagnosis
psychiatric disorders
endocrine disorders
cardiac disorders
gastrointestinal disorders
cardiovascular symptoms
thoracic symptoms
papillary dilation
carcinoid
pheochromocytoma
hypoglycemia
organic gastrointestinal disease
panic attacks
paroxysmal autonomic dysfunction
