Review

Localisation in focal epilepsy: a

Pract Neurol: first published as 10.1136/practneurol-2019-002341 on 17 August 2021. Downloaded from http://pn.bmj.com/ on October 4, 2024 by guest. Protected by copyright.
practical guide
Fahmida A Chowdhury ‍ ‍ ,1,2 Rui Silva,1 Benjamin Whatley,1,3
Matthew C Walker1,2

►► Additional online Abstract which is the part of the brain that must
supplemental material is
The semiology of epileptic seizures reflects be removed to render the patient seizure
published online only. To view,
please visit the journal online activation, or dysfunction, of areas of brain free, and is the objective of epilepsy
(http://​dx.​doi.o​ rg/​10.​1136/​ (often termed the symptomatogenic zone) as a surgery.2 Table 1 describes further these
practneurol-​2019-​002341). seizure begins and evolves. Specific semiologies and other relevant concepts in presurgical
1
Department of Epilepsy,
in focal epilepsies provide an insight into the assessment. Electrical stimulation studies
National Hospital for Neurology location of the seizure onset zone, which is have helped to identify regions of brain
and Neurosurgery, London, UK
2
particularly important for presurgical epilepsy involved in generating different types of
Department of Clinical and
Experimental Epilepsy, Institute
assessment. The correct diagnosis of paroxysmal semiology; however, these also show that
of Neurology, London, UK events also depends on the clinician being most of cortex is clinically silent, and clin-
3
Department of Neurology, familiar with the spectrum of semiologies. ical signs and symptoms occur only when
Dalhousie University, Halifax, Here, we summarise the current literature on the seizure spreads to activate or disrupt
Nova Scotia, Canada localisation in focal epilepsies using illustrative cortical networks.3
Correspondence to cases and discussing possible pitfalls in Clinical signs evolve as the seizure
Fahmida A Chowdhury, National localisation. propagates, and reflect the anatomical
Hospital for Neurology and
Neurosurgery, London WC1N
sites involved in its spread. However,
3BG, UK; ​fahmidaamin.​ seizure propagation comprises two
chowdhury@​nhs.​net
Introduction distinct phenomena.4 There is an ictal
The clinical signs (semiology) of seizures wavefront moving at about 1 mm/s as
Accepted 26 May 2021
Published Online First are the manifestation of epilepsy due to neurones are slowly recruited into firing;
17 August 2021
altered electrical activity in the affected this represents, for example, the speed of
part of the brain; these phenomena can the Jacksonian march with slow tangential
be motor, behavioural or subjective, with activation of cortex. There is also a much
or without altered consciousness. Hugh- faster ictal discharge spreading at about
lings Jackson recognised this over 150 300 mm/s, which represents the propa-
​pn.​bmj.​com
years ago and in 1868 described the char- gation of excitation/inhibition into neigh-
acteristic ‘Jacksonian march’—seen in bouring or connected brain areas. It is this
focal motor seizures arising from primary faster propagation that electroencephalo-
motor cortex—writing that ‘there will be gram (EEG) electrodes detect. Thus, an
all varieties of epilepsy, according to the area seen to be recruited into ictal activity
exact position of grey matter altered’.1 on EEG is not necessarily firing, since we
Careful analysis of semiology, using both are seeing the input to that area but not
the patient’s history and data from videote- its behaviour. The seizure semiology can,
lemetry, can help to localise seizure onset. therefore, be generated by both activa-
This is particularly important in presur- tion of brain areas, which occurs slowly,
gical epilepsy assessment, something that and by the disruption of brain networks,
should be considered for everyone with which occurs more rapidly. Propaga-
medically refractory focal epilepsies. tion of seizure activity and semiology is
Clinicians also need to be familiar with further complicated by the recruitment of
© Author(s) (or their the spectrum of epilepsy semiologies in subcortical areas, leading to rapid engage-
employer(s)) 2021. No order to make the correct diagnosis and ment of widespread areas of cortex. It is
commercial re-­use. See rights therefore not surprising that the rate of a
and permissions. Published
to differentiate epileptic seizures from
by BMJ. dissociative events, syncope and other seizure’s progression depends on several
non-­epileptic events. The symptomato- factors, foremost among which is localisa-
To cite: Chowdhury FA,
Silva R, Whatley B, et al. genic zone is where symptoms and clin- tion. For example, temporal lobe seizures,
Pract Neurol 2021;21:481– ical signs originate; it is usually, but not which depend mainly on activation of
491. always, close to the epileptogenic zone, brain areas, evolve more slowly, whereas

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Review

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Table 1 Cortical zones defined in presurgical evaluation
Cortical zone Definition
Symptomatogenic zone The area of cortex which, when activated by an epileptiform discharge, produces the initial ictal symptoms; it is usually
an area of eloquent cortex, and may be outside the epileptogenic zone.
Seizure onset zone The area of cortex from which clinical seizures actually originate.
Epileptogenic zone The area of cortex that is necessary for the generation of seizures, and must be removed (or disconnected) to render
patients seizure-­free after surgery; it may be larger than the seizure onset zone and the epileptogenic lesion.
Irritative zone The area of cortex that generates interictal electrographic spikes
Epileptogenic lesion The macroscopic lesion seen on the MR scan that is causing the epileptic seizures.

frontal lobe seizures, which may depend on disinhi- areas may result more from disinhibition (removal of
bition, propagate quickly. While certain semiologies, higher cortical control) than from direct activation.
in particular those that involve activation of eloquent Together, these can make frontal lobe seizure semi-
brain areas, are pathognomonic (such as focal motor ology complex and difficult to interpret. Neverthe-
seizures that arise from contralateral primary motor less, seizures have characteristic patterns depending
cortex) other signs are of less localising value, for on the area of the frontal lobe involved. In general,
example, automotor seizures that can arise from frontal lobe seizures are typically brief, may cluster,
different brain regions. can have prominent vocalisation and commonly occur
Before there were EEGs, clinical semiology anal- from sleep. Motor features are prominent and include
ysis relied mainly on postmortem studies. Though various different motor phenomena, including simple
EEG enables mapping of the ictal onset and spread, it clonic movements, tonic posturing and eye deviation,
does not tell us about the behaviour of the underlying or more complex movements such as cycling, rocking
cortex when such activity is detected. EEG has further and grimacing. Seizures can appear bizarre, with bilat-
limitations, for example, with seizures arising from eral motor phenomena, but with retained awareness.
deep sources such as orbitofrontal cortex, where there Ictal scalp EEG may show no changes if there is a deep
may be no or widespread EEG changes at the scalp; source, or may be obscured by movement artefact, and
intracranial recordings can overcome this to some so frontal lobe seizures may be mistaken for dissocia-
extent. Advances in neuroimaging have also allowed tive (non-­epileptic) attacks. Figure 1 summarises the
better precision in localising semiology. However, the semiology of seizures arising from the frontal lobe by
epileptic region may involve relatively normal brain region.
adjacent to a lesion, and some lesions may be inci- The ictal onset patterns during intracranial studies
dental. In addition, ictal manifestations may repre- with stereo-­EEG6 7 show that elementary motor signs
sent propagation of epileptic activity from its origin, (clonic, tonic, versive) arise from precentral and
and therefore, the clinician needs to integrate all the premotor regions, while more complex motor signs
available data (history, examination, neuroimaging, arise from more rostral frontal regions. Non-­integrated
neurophysiology and neuropsychology) to determine (less natural) gestural behaviour with proximal stereo-
the epileptogenic zone. However, seizure semiology typies, such as rocking and turning, are from premotor
should certainly not be disregarded. In a recent study and posterior prefrontal regions, and more integrated
looking at a series of patients successfully treated with (natural) gestural behaviour with distal stereotypies,
epilepsy surgery,5 seizure semiology was correctly such as manual automatisms, arise from more anterior
lateralising in 74% and correctly localising in 77% of prefrontal regions (orbitofrontal cortex, frontal pole,
patients, on a par with scalp EEG and MRI. anterior cingulate). In clinical practice, these distinc-
In this paper, we summarise the current literature tions may be confounded by rapid propagation.
on localisation of focal seizures and discuss some of its
complexities and possible pitfalls. We have also made
available case examples from our centre as online Primary motor cortex
supplemental videos. There is extensive literature on Seizures from primary motor cortex comprise contra-
this topic and so we have focused on the most common lateral unilateral clonic or sometimes tonic or myoc-
semiologies. lonic activity that can spread to adjacent areas: the
‘Jacksonian march’, reflecting ictal activity spread
Frontal lobe seizures through the motor cortex (video 1) along the homun-
The frontal lobe is the largest lobe of the brain. Its culus. It is therefore possible to distinguish lateral from
high connectivity with other cortical regions can result medial precentral seizures, depending on whether the
in rapid, widespread ictal propagation. Moreover, seizure starts in face, upper or lower limb.8 The spread
some of the semiological features from more rostral is typically slow, representing the slow recruitment

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Figure 1 Summary of semiological signs by symptomatogenic zone on mesial and lateral structures. DLPFC, dorsolateral prefrontal
cortex; SMA, supplementary motor area.

of neurones during tangential spread of the ictal aura (he is seen reaching for the button but later did
wavefront. not recall the aura), followed by behavioural arrest,
Video case 1: A 50-­year-­old man with seizure onset and clonic movements visible in the left face spreading
at aged 28 reported ongoing focal to bilateral tonic-­ in a Jacksonian fashion over the left arm as the seizure
clonic seizures. MR scan of brain showed a cystic lesion spreads over the right primary motor cortex, followed
in the right middle frontal gyrus (figure 2: T1 MRI). by left arm extension (fencing posture) indicating
Videotelemetry captured a seizure that began with an involvement of supplementary motor area, before
secondary generalisation. The semiology pointed to
right hemispheric onset and ictal EEG showed onset
in the right frontocentral region, in keeping with the
lesion.

Supplementary motor area

Seizures arising in the supplementary motor area
(SMA) are characterised by asymmetric bilateral tonic
posturing, with or without impaired awareness. There
may be extension of the upper limb contralateral to
the hemisphere of onset, and flexion of the ipsilateral
upper limb, known as a ‘fencing posture’ (video 1),
along with head and eye deviation contralateral to
the hemisphere of onset. The motor features can
Video 1 Video case 1. be preceded by sensory features such as tingling, or

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Review

start with impaired awareness and progress to show

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other motor features. Seizures arising from ventro-
medial prefrontal regions, including anterior cingu-
late, have been associated with ‘forced thinking’ and
have hyperkinetic features; patients may appear to be
frightened.6 Patients with seizures arising from the
contralateral dorsolateral frontal cortex (involving
the frontal eye fields) may show early head version
(forced, sustained and extreme turning of the head
with neck extension) and eye version.7 Seizures
arising in the dominant hemisphere, especially if in
or near Broca’s area, can cause dysphasia. Seizures
arising from frontal operculum are characterised by
face clonic movements that may be unilateral, with
laryngeal symptoms, hypersalivation and articulation
difficulties. Spread to the insula means there is signif-
icant overlap of symptoms with seizures arising from
the insula (see later). Focal atonic seizures can occur
in frontal lobe epilepsies and may indicate involve-
ment of negative motor areas,13 or the supplementary
motor area.14 Gelastic seizures and ictal pouting may
also occur in frontal lobe seizures (mesiofrontal and
anterior cingulate) but we discuss these separately
below, along with lateralising signs.
Video case 2: A 24-­year-­old man developed hyper-
kinetic seizures from sleep. MR scan of brain was
Figure 2 Case 1: T1 MR scan of brain showing lesion in right non lesional, and positron emission tomography
middle frontal gyrus (shown with arrow).
(PET) scan showed hypometabolism in the right orbi-
tofrontal region (figure 3). During an intracranial
tightness. Since the supplementary motor area is highly implantation with stereo-­EEG, we captured habitual
connected to other parts of the brain, seizures arising hyperkinetic seizures that progressed to bilateral
from other regions (especially parietal and occipital tonic seizures with loss of awareness. The EEG onset
lobe seizures) can have asymmetric tonic posturing.9 was seen in the right orbitofrontal region, and there
However, seizures arising from extrafrontal regions were tonic features when the EEG seizure spread to
are more likely to be preceded by auras or to involve electrode contacts in the supplementary motor area.
loss of awareness.10 11 Following resection of the right orbitofrontal region
he is now seizure free.
Other frontal regions
Seizures arising from orbitofrontal cortex tend to
have impaired awareness and automatisms,12 and Temporal lobe seizures
may evolve to complex motor seizures (video 2). The temporal lobes are the most frequent site of origin
Olfactory auras occasionally occur and there may of focal seizures; two thirds of people with refractory
be autonomic features such as ictal tachycardia. epilepsy assessed for potential surgery have temporal
Seizures arising from frontopolar cortex can also lobe seizures. They typically comprise behavioural
arrest, manual and oral automatisms in two thirds,
and variable degrees of loss of awareness with postictal
confusion15 (see figure 1 for a summary). Though there
is some overlap with frontal lobe seizures, temporal
lobe seizures tend to be longer (up to 2–3 min) and
less frequent, with longer periods of postictal confu-
sion. Table 2 shows other differentiating factors. Focal
impaired awareness seizures arising from the temporal
lobe can be distinguished clinically from generalised
‘absence’ seizures that occur in the genetic (idiopathic)
generalised epilepsies. Absences are typically very
short periods of motor arrest, lasting seconds, without
preceding aura or postictal confusion and occur many
Video 2 Video case 2. times a day. In comparison, focal impaired awareness

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Figure 3 Case 2: Positron emission tomography (PET) scan of brain showing hypermetabolism in right orbitofrontal region.

seizures are longer, may be preceded by aura and are to 98%.16 Gustatory and olfactory hallucinations are
commonly associated with automatisms, described also relatively specific for temporal lobe epilepsy.
below in more detail. Somatosensory auras can occur and usually manifest
as either unilateral or bilateral tingling.17 The type
Auras of aura can help to differentiate between seizures of
Auras occur commonly in temporal lobe seizures; mesiotemporal origin vs lateral neocortical origin. In
some are relatively specific. For example an abdominal a large study of 187 temporal lobe seizures assessed
aura (such as rising epigastric sensation or abdominal by intracranial stereo-­EEG, abdominal aura and expe-
discomfort) is highly associated with temporal lobe riential phenomena such as fear, déjà vu and jamais vu
epilepsy with a probability of 74%, and the evolu- indicated mesiotemporal seizures, whereas elemental
tion of an abdominal aura into an automotor seizure auditory aura indicated lateral onset (Heschl’s gyrus,
increases the probability of temporal lobe epilepsy primary auditory cortex).18

Mesial temporal lobe including hippocampus

Table 2 Temporal and frontal lobe seizures differentiating Seizures arising in the mesial temporal lobe may have
semiological features. the experiential aura described above, but also early
Features Frontal Temporal autonomic phenomena such as flushing or tachy-
Seizure frequency Often daily Less frequent cardia. Early apnoea and tachycardia indicate involve-
Occurrence during sleep Typical Less common ment of amygdala.19 These symptoms may progress
Seizure onset Abrupt Gradual
to behavioural arrest, automatisms, and impaired
Seizure duration Brief (usually up to Longer (usually 30
30 s)* s to 2 min)*
Evolution Rapid Gradual
Automatisms Less common Common and
longer
Complex motor features Early, frequent, Late, less frequent
prominent
Hypermotor Common Rare
Vocalisation Loud, nonspeech Ictal speech
(scream, grunt) (nondominant)
Secondary generalisation Common Less common
Postictal confusion Shorter, less Longer, more
prominent prominent
*Can be longer. Video 3 Video case 3.

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Video 4 Video case 4.

features (temporoparietal junction). Complex visual

hallucination and illusions can occur with anterome-
dial and temporo-­ occipital lobe seizures, but often
do not clearly distinguish neocortical from mesial
temporal onset. The most common auditory aura is
of elementary sounds (humming, ringing, buzzing)
indicating involvement of primary auditory cortex,
and less frequently complex sounds (voices, songs) or
distortions, indicating involvement of auditory associ-
ation areas. Elementary unilateral auditory aura indi-
cates onset in contralateral hemisphere. Ictal dysphasia
may be an early sign of dominant hemisphere involve-
Figure 4 Case 3: T1 MR scan of brain showing cavernoma in ment (video 4). Lateral temporal seizures are shorter
left mesiotemporal region (shown with arrow). than mesial temporal lobe seizures, with earlier loss of
awareness and propagation to frontal regions resulting
awareness. Automatisms often involve the hands in complex motor features and more common evolu-
(fumbling, picking, fidgeting) or mouth (chewing, lip tion to a bilateral tonic-­clonic seizure.18 However, due
smacking, swallowing) (video 3). Less common autom- to reciprocal connections between mesial and neocor-
atisms associated with temporal lobe seizures include tical temporal cortex, the semiology of these seizures
vocalisations and ictal speech (in non-­ dominant overlaps.
seizures). Unilateral dystonic posturing can occur and Video case 4: A 27-­ year-­
old woman had seizure
is a contralateral sign.20 Seizures tend to be longer that onset at age 18 years, which would begin with a psychic
those arising from lateral temporal lobe, and they less aura of fear and anxiety and sometimes a noise in her
commonly evolve to bilateral tonic-­clonic seizures.21 ears, followed by difficulty speaking. MR scan of brain
Video case 3: A 36-­year-­old right-­handed man had was non-­lesional and PET scan showed hypometabo-
seizure onset at the age of 21, characterised by déjà vu lism in the left temporal region (figure 5), which on
and epigastric rising, evolving to automotor features, statistical analysis pointed to left superior temporal
with rare focal to bilateral tonic-­ clonic seizures. gyrus extending to temporo-­ parieto-­occipital junc-
Imaging showed a cavernoma in the left amygdala and tion. Videotelemetry captured stereotypical seizures
hippocampal head (figure 4). Videotelemetry captured with ictal dysphasia. Assessment by nurses showed
habitual seizures with EEG confirming onset in the she could engage and follow visual commands but not
left temporal region. Seizures had typical features of verbal commands, with impairment of both expressive
mesiotemporal lobe seizures, with psychic and abdom- language and reading. Ictal EEG showed onset in the
inal aura, followed by oral and manual automatisms, left temporal region suggesting a neocortical onset.
then loss of awareness. Before the seizure generalised,
there was head and eye version to the right along Temporal pole seizures
with a ‘figure-­of-­4’ sign (left arm flexed and right arm Temporal pole seizures tend to have earlier loss of
extended), which lateralised the onset to the left hemi- consciousness21 and more commonly present with
sphere. He subsequently underwent a lesionectomy hypermotor features.22
and remains seizure free.
Parietal lobe seizures
Lateral/neocortical temporal lobe Parietal lobe epilepsy is rare, accounting for 5%
Auras are less common in seizures arising from temporal of epilepsies.23 However, seizures arising from the
neocortex, and often have auditory or vertiginous parietal lobe can have diverse semiology and can be

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Figure 5 Case 4: PET scan of brain showing hypometabolism in left temporal lobe (shown with arrow).

difficult to diagnose due to their subjective nature. In macrosomatognosis (body part being enlarged) or
addition, the rich connections to adjacent structures, microsomatagnosis (body part being shrunken),
within and outside the parietal lobe, means that the particularly of the distal extremities.26 Other visual
symptomatogenic zone may be distant to the seizure-­ illusions include epileptic kinetopsia (illusion
onset zone, and therefore, parietal lobe epilepsy may be of movement of a stationary object) in the supe-
both misdiagnosed and also underdiagnosed. Epilepsy rior parietal lobule and intraparietal sulcus,27 and
surgery is least commonly performed on the parietal macropsia (objects appearing larger) or micropsia
lobe, and so there are few data on seizure localisation. (objects appearing smaller) in the precuneus.25
In two of the larger surgical series of patients with pari- Subjective vertiginous sensations may occur in
etal lobe epilepsy,24 25 the most commonly reported seizures arising from various parts of the pari-
aura (in 38% in both series) was contralateral somato- etal lobe. Autoscopic phenomena can occur in
sensory aura. Other subjective reported phenomena of both temporal and parietal lobe epilepsies and
parietal lobe epilepsy include vertiginous aura, distor- often implicate the temporoparietal junction and
tion of body image or visual perception. There may vestibular cortex. In the dominant parietotem-
be auditory, olfactory and epigastric aura, suggesting poral region, there may be language impairment
propagation to temporal and limbic areas. with difficulties reading and writing. Seizures
Primary sensory cortex
arising from the secondary sensory area, in addi-
Seizures arising from the primary sensory cortex in tion to poorly localised sensory aura affecting
the postcentral gyrus tend to start with contralateral distal extremities and face, may involve a feeling of
tingling and or numbness,23 which may then spread inability to move the contralateral limbs.
along adjacent body parts as the seizure propagates Parietal lobe seizures may propagate in different
through the sensory homunculus. Some patients also ways resulting in variable clinical manifestations.
describe pain or altered thermal sensation. The seizure Those that propagate to sensorimotor cortex
may propagate to the frontal lobe resulting in clonic may show focal motor clonic activity contralat-
motor activity often with tonic posturing. eral to the epileptogenic zone; those propagating
to the supplementary motor area may have tonic
Parietal association areas posturing of the limbs; and those propagating to
In seizures arising from the non-­d ominant parietal the temporal region may develop temporal aura or
association cortex, patients may describe distor- automatisms. Seizures from more superior areas of
tions of body image. Others describe sensations of the parietal lobe appear to spread preferentially to
twisting or turning sensations. 23 In seizures arising the frontal lobe while seizures from more inferior
from the superior parietal lobule and precuneus, regions of the parietal lobe appear to propagate to
there may be altered body perception such as temporal areas.

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Occipital lobe seizures patients with seizures arising from the anterior cingu-

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Seizures arising from the occipital lobes are usually (in late: those with typical hypermotor seizures with fear,
40%–75% of patients) characterised by visual aura, autonomic features and infrequent generalisation, and
or less commonly by oculomotor features, such as a subgroup (all with infiltrating astrocytomas) with
eye deviation, blinking or nystagmus. However, their simple motor features and frequent generalisation.
seizure semiology may also reflect propagation to In this series, all those whose seizures arose from the
temporal or frontal lobes.28 29 posterior cingulate had symptoms suggesting temporal
lobe epilepsy with auras (eg, psychic, abdominal and
Primary visual cortex gustatory). However, another study34 reported that
Seizures arising from primary visual (calcarine) cortex seizures arising from the posterior cingulate showed
cause an elementary visual aura, and tend to be brief, semiology that varied depending on their spread: those
usually less than 2 min, and thus are shorter than a spreading to frontal areas showed motor manifesta-
typical migrainous aura. Patients may describe flashing tions—including bilateral asymmetric tonic seizures
coloured or bright white lights, often spherical, and hypermotor seizures—while those spreading
but they may take different forms and shapes. The to temporal areas showed dialeptic seizures (that is
discharges originating in the lateral occipital cortex seizures where the main feature is loss of awareness
probably produce the pulsating quality of the visual and behavioural arrest) or automotor seizures. Gelastic
features. In most patients, the visual phenomena are seizures may also arise from the anterior cingulate
lateralised and contralateral to the hemisphere of as well as ‘chapeau de gendarme’ (down turning of
seizure onset; however, up to 30% have more diffuse the mouth); these are discussed separately below. In
visual changes.30 True ictal visual field loss or amau- summary, seizures arising from the anterior cingulate
rosis is rare, and may represent a postictal phenom- tend to present with typical features, whereas those
enon, particularly if long lasting. from the posterior cingulate show variable features
depending on propagation patterns.
Visual association areas
Complex visual hallucinations may occur in occip-
ital lobe epilepsy but are less common than elemen- Insular seizures
tary phenomena. They occur when seizures involve The insular lobe has two parts, a larger anterior part
the prestriate cortex or may indicate propagation to and a smaller posterior part, and is highly connected
adjacent temporo-­parietal areas. Patients may report with several other brain regions, integrating informa-
complex formed visual hallucinations connected with tion required for cognitive, behavioural, and sensory
past events, or kinetopsia, macropsia or micropsia. processing.35 Seizures arising from the insular lobe are
Rarely, patients report autoscopy, perceiving mirror rare, but have heterogenous semiology that include
images of themselves. viscerosensory, somatosensory, autonomic symptoms
(nausea, hypersalivation, piloerection, heart rate
Parieto-occipital junction changes), language disturbance, complex auditory
Oculomotor manifestations may occur, including eye aura and (due to propagation to frontal and temporal
version, blinking and nystagmus. In a large series,30 areas) automotor and hypermotor behaviours, tonic
20% of patients had eye blinking at seizure onset. and/or clonic motor manifestations.36 This variability
Although eye version may represent propagation, in along with often non-­localising EEG due to deep loca-
two reported cases this occurred while ictal discharges tion, can mean that clinicians may not recognise such
were localised to occipital lobe. Epileptic nystagmus seizures as arising from the insula.
may also develop, typically with the fast phase to the Some studies have suggested that viscerosensory
contralateral hemisphere and the slow component to symptoms—such as abdominal aura and laryngeal
the ipsilateral side.31 constriction, and autonomic signs, including nausea,
hypersalivation, and sweating—arise from the anterior
Cingulate gyrus insula whereas painful sensations—including burning,
The cingulate gyrus forms part of the limbic network; electric shock and tingling—more likely arise from
it consists of anterior, middle and posterior parts, and the posterior insula.37 Non-­ painful somatosensory
has extensive functional connectivity with other brain aura can also occur. Stimulation studies have identi-
regions. Most data on semiology of seizures are from fied several sensory features that help to differentiate
case series of lesional cases. Seizures arising from the insular seizures from seizures arising from primary and
anterior cingulate are characterised by intense fright, secondary somatosensory cortex: insular responses
screaming and vocalisations, with autonomic signs tend to affect a larger skin territory including perioral
and complex gestural automatisms or hypermotor regions, can be bilateral or ipsilateral, and more likely
activity.32 A later case series33 that included cases evolve to complex motor phenomena.
with seizures arising from both posterior and anterior Seizures arising from hippocampus almost always
cingulate, identified that there were two groups of propagate to the insula; thus an abdominal aura is

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Video 5 Video case 5.

also common in mesiotemporal seizures. Due to rapid

propagation, insular seizures can mimic seizures arising
from the temporal or frontal lobe. Nevertheless, the
combination of complex visceral and somatosensory
auras points towards an insular onset.
Figure 6 Case 5: Fluid attenuated inversion recovery (FLAIR)
MR scan of brain showing hypothalamic hamartoma (shown
Special seizures with arrow).
Gelastic seizures
Gelastic seizures typically arise from hypothalamic Video case 6: A 20-­ year-­
old man had onset of
hamartomas. However, the act of laughing is part of seizures at aged 12 years. He had seizures with motor
a complex network involving cingulate, frontal and features and loss of awareness indicating propaga-
temporal regions and so laughing also occurs in seizures tion to frontal areas. MR scan of brain was non-­
arising from other regions. In a case series from our lesional. Videotelemetry captured seizures involving
centre,14 a third of cases had hypothalamic hamar- behavioural arrest and then facial grimacing with
tomas, whereas a third of seizures were temporal, and downturned mouth (chapeau de gendarme) along with
a third frontal. other motor features including rocking, chewing and
►► Gelastic seizures arising from hypothalamic hamartomas head movement. Ictal EEG was non-­localising.
usually present in early childhood, are brief, stereotyped
and very frequent (video 5). The laughter is usually Reflex seizures
mirthless and without impaired consciousness, but auto- Reflex seizures are triggered by specific stimuli.40
nomic signs, such as facial flushing and pupillary dila- Photosensitive epilepsy is the most common reflex
tion, are common. There may also be a dacrystic (crying) epilepsy; although more commonly associated with
component. idiopathic (genetic) genetic generalised epilepsy, this
►► Gelastic seizures arise from brain structures other than also occurs in some focal epilepsies, where it suggests
the hypothalamus usually present in adulthood. Those in an occipital onset. Other reflex focal epilepsies are
temporal lobe epilepsy are usually part of more complex rare but may also have localising value. For example:
seizure semiology with automotor features and loss of ►► Musicogenic seizures localise to the temporal lobe and
consciousness. can occur in both mesial and neocortical temporal
Video case 5: An 18-­year-­old right-­handed man had epilepsies.
onset of gelastic seizures from aged 2 years. Imaging
confirmed a hypothalamic hamartoma (figure 6).
Videotelemetry captured typical gelastic seizure,
without mirth, though he lost awareness in some of
his seizures.

Chapeau de gendarme
The ‘chapeau de gendarme’ or ictal pout sign is char-
acterised by a downturned mouth from bilateral lip
and chin contraction.38 If seen early in the seizure it
is highly localising to frontal lobe onset, in particular
anterior cingulate, orbitofrontal region or mesio-­
prefrontal or premotor cortex (video 6).39 Video 6 Video case 6.

Chowdhury FA, et al. Pract Neurol 2021;21:481–491. doi:10.1136/practneurol-2019-002341 9 of 12

Review

clinical signs contralateral to the side of onset. Other

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Table 3 Localising value of auras
lateralising motor phenomena are a ‘figure-­of-­4’ sign
Aura Localisation before secondary generalisation (video 7) where the
Somatosensory extended arm is contralateral to seizure onset, there is
Marching sensory aura Primary sensory cortex asymmetric clonic ending of generalised phase with the
Tingling, numbness Primary or secondary somatosensory last clonic jerk ipsilateral to seizure onset, and postictal
cortex, supplementary motor area, insula Todd’s paresis contralateral to seizure onset.20 These
Pain/ warmth Secondary somatosensory cortex, insula features do not differentiate between frontal or extra-
Visual aura frontal onset. Unilateral sensory aura is a contralateral
Elementary (lights, Primary visual cortex sign.23
shapes) Video case 7: A 33-­year-­old man had seizure onset
Illusions/ distortions Temporo-­parieto-­occipital at age 21 years, starting with psychic/gustatory aura
Complex visual Temporal and evolving to an automotor seizures with some focal
hallucinations to bilateral tonic-­ clonic seizures. MR scan of brain
Auditory was non-­lesional and PET scan showed left temporal
Elementary Heschl’s gyrus, superior temporal hypometabolism. Videotelemetry captured two
Illusions/distortion Lateral temporal, insula habitual seizures with automotor features evolving to
Other bilateral tonic-­ clonic seizures. Before generalisation,
Gustatory Insula, mesiotemporal there was head version (forced, sustained and extreme
Olfactory Insula, mesiotemporal, orbitofrontal turning of the head) to the right, followed by a ‘figure-­
Vestibular Posterior temporal, parietal of-­4’ with right arm extension, indicating left hemi-
Autonomic Insula, amygdala, cingulate spheric onset. In one of the seizures, the last clonic jerk
was on the left, which also pointed to left hemispheric
onset. Ictal EEG was non-­localisable in one but later-
alised to the left in another.
►► Startle-­
induced tonic or atonic seizures triggered by
Ictal or postictal dysphasia lateralises to dominant
auditory stimuli localise to onset in or near supplemen-
hemisphere but is of poor localising value and has
tary motor area.
to be differentiated from speech arrest which is non-­
►► Eating-­induced seizures usually arise from temporal or
lateralising. Ictal speech—speech with formed nonsen-
extratemporal perisylvian area.
sical phrases—is a non-­ dominant sign. Preserved
In these epilepsies, spontaneous seizures can also awareness during ictal automatism points to the non-­
occur. dominant hemisphere.15 Rhythmic ictal non-­ clonic
hand motions may occur as a contralateral sign in
Aura temporal lobe epilepsy.41 A simple auditory aura, if
This is often the first ictal symptom, and therefore unilateral (which is rare) is a contralateral sign. Other
can provide useful localising information. Table 3 lateralising signs in temporal lobe epilepsy include
summarises various aura types . peri-­ictal drinking, ictal spitting, ictal vomiting and an
urge to urinate, which point to a non-­dominant focus.
Lateralising signs Hemifield visual aura has good lateralising value
As well as helping with localisation, some signs have to the contralateral occipital lobe.30 Unilateral eye
lateralising value. Robust lateralising motor signs blinking lateralises to the hemisphere ipsilateral to
(positive predictive value of >80%) already discussed the eye-­blinking.42 In epileptic nystagmus, the fast
include unilateral clonic movements, unilateral tonic component is to the side contralateral to hemisphere
or dystonic posturing and early head version: these are of seizure onset.21

Conclusion
Careful analysis of seizure semiology can help to
localise seizures, which is particularly important
when evaluating people for epilepsy surgery. Identi-
fying seizure semiology requires a meticulous history
to ascertain details of auras, and careful analysis of
seizures captured during video-­EEG recordings.
Many seizure symptoms and signs have robust local-
ising and lateralising value. However, seizure local-
isation can still be challenging for several reasons,
including the fact that seizure semiology may become
apparent only once a seizure has spread to an eloquent
Video 7 Video case 7. area or has sufficiently engaged brain networks.

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 Review
4 Liou J-­Y, Smith EH, Bateman LM, et al. A model for focal

Pract Neurol: first published as 10.1136/practneurol-2019-002341 on 17 August 2021. Downloaded from http://pn.bmj.com/ on October 4, 2024 by guest. Protected by copyright.
Key points seizure onset, propagation, evolution, and progression. Elife
2020;9:e50927.
►► Seizure semiology reflects altered electrical activity in 5 Elwan S, Alexopoulos A, Silveira DC, et al. Lateralizing and
the affected part of the brain. localizing value of seizure semiology: comparison with scalp
►► Seizures can manifest as motor, subjective and EEG, MRI and PET in patients successfully treated with
behavioural phenomena, or loss of awareness, resective epilepsy surgery. Seizure 2018;61:203–8.
and careful analysis of their semiology can allow 6 Bonini F, McGonigal A, Trébuchon A, et al. Frontal lobe
localisation of seizure onset. seizures: from clinical semiology to localization. Epilepsia
►► Ictal manifestations may represent propagation of the 2014;55:264–77.
epileptic discharge from its origin. 7 McGonigal A, Chauvel P. Prefrontal seizures manifesting as
motor stereotypies. Mov Disord 2014;29:1181–5.
8 McGonigal A, Chauvel P. Frontal lobe epilepsy: seizure
semiology and presurgical evaluation. Pract Neurol
Further reading 2004;4:260–73.
9 Ikeda A, Sato T, Ohara S, et al. "Supplementary motor area
1. Noachtar S, Peters AS. Semiology of epileptic seizures: (SMA) seizure" rather than "SMA epilepsy" in optimal surgical
candidates: a document of subdural mapping. J Neurol Sci
A critical review. Epi and Behav 2009; 15: 2-­9
2002;202:43–52.
2. Foldvary-­Schaefer N, Ungewanse K. Localizing and
10 Werhahn KJ, Noachtar S, Arnold S, et al. Tonic seizures: their
Lateralizing features of aura and seizures. Epi and
significance for lateralization and frequency in different focal
Behav 2011; 20: 160-­166 epileptic syndromes. Epilepsia 2000;41:1153–61.
11 Sitthinamsuwan B, Usui N, Tottori T, et al. Seizures with tonic
posturing: Semiologic difference between supplementary
Moreover, its semiology may relate not only to acti- sensorimotor area (SSMA) origin and extra-­SSMA origin.
vation of brain areas but also to disruption of brain Epilepsia 2016;57:e39–44.
function and disinhibition of networks that mediate 12 Chibane IS, Boucher O, Dubeau F, et al. Orbitofrontal
complex behaviours. Nevertheless, careful interpreta- epilepsy: case series and review of literature. Epilepsy Behav
tion of seizure semiology enhances seizure localisation, 2017;76:32–8.
13 Ikeda A, Hirasawa K-­ichi, Kinoshita M, et al. Negative motor
particularly when collated with other information
seizure arising from the negative motor area: is it ictal apraxia?
including ictal EEG activity, structural and functional
Epilepsia 2009;50:2072–84.
imaging, and neuropsychometry. 14 Kovac S, Diehl B, Wehner T, et al. Gelastic seizures: incidence,
clinical and EEG features in adult patients undergoing video-­
Acknowledgements With thanks to Dr Kelly Pegoretti EEG telemetry. Epilepsia 2015;56:e1–5.
Baruteau, Consultant Neuroradiologist, for her help
15 Blair RDG. Temporal lobe epilepsy semiology. Epilepsy Res
with selecting MR images for this manuscript, and to the
Treat 2012;2012:1–10.
Videotelemetry Team at the National Hospital for Neurology
16 Henkel A, Noachtar S, Pfänder M, et al. The localizing value
and Neurosurgery for their contribution to acquiring the
of the abdominal aura and its evolution: a study in focal
Videotelemetry data.
epilepsies. Neurology 2002;58:271–6.
Contributors FAC drafted and edited the manuscript. RS
17 Erickson JC, Clapp LE, Ford G, et al. Somatosensory auras in
helped with the literature review and collection of video
cases. MCW provided critical review of the manuscript. BW refractory temporal lobe epilepsy. Epilepsia 2006;47:202–6.
contributed to figures and editing the article. 18 Maillard L, Vignal J-­P, Gavaret M, et al. Semiologic and
Funding The authors have not declared a specific grant for this electrophysiologic correlations in temporal lobe seizure
research from any funding agency in the public, commercial or subtypes. Epilepsia 2004;45:1590–9.
not-­for-­profit sectors. 19 Du X, Usui N, Terada K, et al. Semiological and
Competing interests None declared. electroencephalographic features of epilepsy with amygdalar
Patient consent for publication Not required. lesion. Epilepsy Res 2015;111:45–53.
20 Marashly A, Ewida A, Agarwal R, et al. Ictal motor
Provenance and peer review Commissioned; externally peer
reviewed by Khalid Hamandi, Cardiff, UK. sequences: lateralization and localization values. Epilepsia
2016;57:369–75.
ORCID iD 21 Chabardès S, Kahane P, Minotti L, et al. The temporopolar
Fahmida A Chowdhury http://​orcid.​org/​0000-​0001-​9546-​0197 cortex plays a pivotal role in temporal lobe seizures. Brain
2005;128:1818–31.
References 22 Wang L, Mathews GC, Whetsell WO, et al. Hypermotor
1 Eadie MJ, Bladin PF. Disease once sacred: a history of the seizures in patients with temporal pole lesions. Epilepsy Res
medical understanding of epilepsy, 2001. 2008;82:93–8.
2 Rosenow F, Lüders H. Presurgical evaluation of epilepsy. Brain 23 Salanova V. Parietal lobe epilepsy. J Clin Neurophysiol
2001;124:1683–700. 2012;29:392–6.
3 Foldvary-­Schaefer N, Unnwongse K. Localizing and 24 Asadollahi M, Sperling MR, Rabiei AH, et al. Drug-­Resistant
lateralizing features of auras and seizures. Epilepsy Behav parietal lobe epilepsy: clinical manifestations and surgery
2011;20:160–6. outcome. Epileptic Disord 2017;19:35–9.

Chowdhury FA, et al. Pract Neurol 2021;21:481–491. doi:10.1136/practneurol-2019-002341 11 of 12

 Review
25 Francione S, Liava A, Mai R, et al. Drug-­Resistant 33 Alkawadri R, So NK, Van Ness PC, et al. Cingulate epilepsy:

Pract Neurol: first published as 10.1136/practneurol-2019-002341 on 17 August 2021. Downloaded from http://pn.bmj.com/ on October 4, 2024 by guest. Protected by copyright.
parietal epilepsy: polymorphic ictal semiology does not report of 3 electroclinical subtypes with surgical outcomes.
preclude good post-­surgical outcome. Epileptic Disord JAMA Neurol 2013;70:995–1002.
2015;17:32–46. 34 Enatsu R, Bulacio J, Nair DR, et al. Posterior cingulate
26 Yang Y, Wang H, Zhou W, et al. Electroclinical
epilepsy: clinical and neurophysiological analysis. J Neurol
characteristics of seizures arising from the precuneus based
Neurosurg Psychiatry 2014;85:44–50.
on stereoelectroencephalography (SEEG). BMC Neurol
35 Isnard J, Guénot M, Sindou M, et al. Clinical manifestations of
2018;18:110.
insular lobe seizures: a stereo-­electroencephalographic study.
27 Perumal MB, Chinnasami S, Shah A, et al. Epileptic kinetopsia
Epilepsia 2004;45:1079–90.
localizes to superior parietal lobule and intraparietal sulcus.
36 Peltola ME, Trebuchon A, Lagarde S. Anatomoelectroclinical
Neurology 2014;83:768–70.
features of SEEG-­confirmed pure insular-­onset epilepsy.
28 Blume WT, Wiebe S, Tapsell LM. Occipital epilepsy: lateral
Epilepsy Behav 2020;21:105.
versus mesial. Brain 2005;128:1209–25.
37 Jobst BC, Gonzalez-­Martinez J, Isnard J, et al. The insula and
29 Kuzniecky R. Symptomatic occipital lobe epilepsy. Epilepsia
its epilepsies. Epilepsy Curr 2019;19:11–21.
1998;39(Suppl 4):S24–31.
38 Hayakawa I, Kubota M. Ictal pouting: Kabuki visage or
30 Salanova V, Andermann F, Olivier A, et al. Occipital lobe chapeau de gendarme? Pract Neurol 2018;18:410–2.
epilepsy: electroclinical manifestations, electrocorticography, 39 Tan Y-­L, Muhlhofer W, Knowlton R. Pearls and Oy-­sters: the
cortical stimulation and outcome in 42 patients treated chapeau de gendarme sign and other localizing gems in frontal
between 1930 and 1991. surgery of occipital lobe epilepsy. lobe epilepsy. Neurology 2016;87:e103–5.
Brain 1992;115(Pt 6):1655–80. 40 Italiano D, Ferlazzo E, Gasparini S, et al. Generalized versus
31 Lee S-­U, Suh H-­I, Choi JY, et al. Epileptic nystagmus: a partial reflex seizures: a review. Seizure 2014;23:512–20.
41 Lee GR, Arain A, Lim N, et al. Rhythmic ictal nonclonic hand
case report and systematic review. Epilepsy Behav Case Rep
(RINCH) motions: a distinct contralateral sign in temporal
2014;2:156–60.
lobe epilepsy. Epilepsia 2006;47:2189–92.
32 Bancaud J, Talairach J. Clinical semiology of frontal lobe 42 Benbadis SR, Kotagal P, Klem GH. Unilateral blinking: a
seizures. Adv Neurol 1992;57:3–58. lateralizing sign in partial seizures. Neurology 1996;46:45–8.

12 of 12 Chowdhury FA, et al. Pract Neurol 2021;21:481–491. doi:10.1136/practneurol-2019-002341