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Frontal Lobe Epilepsy in A Pediatric Population: Characterization of Clinical Manifestations and Semiology

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Original article

pISSN 2635-909X • eISSN 2635-9103


Ann Child Neurol 2022;30(3):102-110
https://doi.org/10.26815/acn.2022.00185

Frontal Lobe Epilepsy in a Pediatric Population:


Characterization of Clinical Manifestations and Semiology
Dajeong Lee, MD, Jiwon Lee, MD, Jeehun Lee, MD
Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea

Received: May 8, 2022


Revised: May 17, 2022 Purpose: Frontal lobe epilepsy (FLE) has various clinical presentations depending on the anatomy
Accepted: May 18, 2022 involved. Seizures are brief and can mimic psychiatric conditions, and patients often cannot de-
scribe the aura. Therefore, it is difficult to characterize the semiology, especially in pediatric pa-
Corresponding author: tients. This study investigated the characteristics of pediatric FLE.
Jeehun Lee, MD
Methods: We retrospectively reviewed the data of pediatric patients with FLE who underwent
Department of Pediatrics, Samsung
long-term video-electroencephalography (EEG) monitoring between January 2010 and June
Medical Center, Sungkyunkwan
2020. Patients’ demographic data, seizure-related clinical presentations, semiology, brain mag-
University School of Medicine, 81
Irwon-ro, Gangnam-gu, Seoul netic resonance imaging (MRI), and EEG data were analyzed.
06351, Korea Results: Fifty-six patients were included (31 males, 25 females). The age of seizure onset varied
Tel: +82-2-3410-3538 from 1 month to 14 years (mean±standard deviation, 6.1±4.4 years). Seizures were classified into
Fax: +82-2-3410-0043 nine categories, including focal tonic (30/56), aura (22/56), hypermotor (17/56), focal clonic
E-mail: [email protected] (15/56), versive (13/56), and bilateral asymmetric tonic (4/56). Seventeen patients (30.4%) had
abnormal MRI results, including focal cortical dysplasia, heterotopic gray matter, and neuroepi-
thelial tumors. Ictal EEG changes were commonly observed in the dorsolateral premotor and cen-
tral cortices. In focal tonic seizures, EEG changes often originated in the premotor cortex. The lo-
cation of the lesions on MRI and EEG coincided in six cases.
Conclusion: In pediatric FLE, various seizure types occur depending on the ictal anatomic origin,
and individual patients had multiple semiologies. Brain MRI was normal in two-thirds of patients,
and interictal EEG did not reveal epileptiform discharges in approximately 25%. Semiology re-
ported on the basis of home videos and interictal EEG will help localize the ictal onset zone.

Keywords: Epilepsy, frontal lobe; Seizures; Electroencephalography; Magnetic resonance imaging

Introduction tion of ictal onset in clinical practice [4-8].


Several previous studies involving adult patients have described
Frontal lobe epilepsy (FLE) has a difficult-to-understand and com- the semiological seizure characteristics of FLE as follows: very
plex seizure semiology, which depends on the location of the epi- short duration, sudden onset and offset, stereotypic form, a ten-
leptogenic area [1-6]. The frontal lobe is large and has several com- dency for clustering, nocturnal disposition, and rapid secondary
bined functions [1,3]. The epileptic propagation of FLE is com- generalization [2,9-15]. Because the frontal lobe is involved in not
plex and rapid; therefore, it is often difficult to predict the localiza- only motor function, but also in language and emotional control,

Copyright © 2022 Korean Child Neurology Society


This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/)
which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

102 www.annchildneurol.org
Ann Child Neurol 2022;30(3):102-110

the ictal appearance of FLE can present as psychiatric symptoms 3. Ethics


such as fearful feelings or paranoid delusions [1,7,16]. FLE is often This study was approved by the Institutional Review Board of
misdiagnosed as pseudoseizures related to psychiatric conditions Samsung Medical Center (IRB file no. 2022-04-183). The require-
[7,9,16-20]. ment for informed consent from patients was waived because of
The few studies focusing on FLE in the pediatric population the retrospective nature of this study.
have revealed that frontal lobe seizures in children differ from
those of adults [1,2,9,21-23]. Therefore, the semiological char- 4. Localization of EEG
acteristics of pediatric FLE require definition using ictal data, We classified the frontal lobes into the dorsolateral, mesial, and
which could provide much-needed information for clinicians in basal frontal lobes based on the anatomical locations, and the dor-
terms of diagnosis and treatment. The aim of this study was to solateral cortex was subdivided into the prefrontal, premotor, and
identify the semiological characteristics of FLE in pediatric pa- central cortices. When electrodes were attached according to the
tients using long-term video electroencephalography (EEG) international 10 to 20 system, the corresponding position of each
data. electrode was checked. Based on this criterion, the Fp1 and Fp2
electrodes were classified as corresponding to the prefrontal cortex,
Materials and Methods the F3 and F4 electrodes as being placed on the premotor cortex,
the C3 and C4 electrodes as corresponding to the central cortex,
1. Patients and the Fz and Cz electrodes as being placed on the mesial frontal
We retrospectively reviewed the medical records of pediatric pa- cortex.
tients admitted to the Samsung Medical Center, Seoul between
January 2010 and June 2020. During that period, 71 patients were Results
diagnosed with FLE. Among them, patients with ictal events were
included as subjects of this study. The inclusion criteria were as fol- 1. Clinical manifestations
lows: (1) patients who underwent long-term video EEG monitor- In total, 56 patients (25 females, 31 males) met the inclusion crite-
ing; (2) patients under 18 years of age at the time of the long-term ria. The age of onset ranged from 1 month to 14 years (mean,
video EEG recordings; (3) patients who had clinical seizures 6.1 ± 4.4 years) (Table 1). Seventeen patients (30.4%) had a medi-
during monitoring; and (4) patients with a final diagnosis of FLE. cal history of neurological conditions such as febrile seizures
Autosomal dominant nocturnal FLE and genetic FLE were not (5/56, 8.9%), developmental delay (4/56, 7.1%), intellectual dis-
clinically suspected in any patients. No patients underwent genetic ability (4/56, 7.1%), encephalitis (2/56, 3.6%), and cerebral hem-
studies, such as a next-generation sequencing-based gene panel orrhage (2/56, 3.6%). The number of patients who underwent
study. monotherapy and polytherapy was 12 (21.4%) and 34 (60.7%),
respectively.
2. Data collection
Data on clinical variables, such as demographics, age of onset, sei- 2. Seizure semiology features
zure semiology, EEG, brain magnetic resonance imaging (MRI) Based on patients’ medical records and ictal events during the long-
findings, and anti-seizure medications used were collected. The term video EEG monitoring, we categorized seizures into nine cat-
seizure types were described using the semiological seizure classifi- egories according to the semiological seizure classification: focal
cation proposed by Luders et al. [24] and Dugan et al. [25]. When clonic, focal tonic, asymmetric tonic, hypermotor, versive, dialep-
describing patient seizure types, we included both the medical his- tic, automotor, generalized tonic clonic, and aura (Table 2). Auras
tory and the seizure semiology confirmed during long-term video were further classified as somatosensory, psychic, autonomic, visu-
EEG monitoring at the time of admission. al, abdominal, or vertiginous. If there was more than one semiolo-
In this study, we summarized the characteristics, including de- gy in a single patient, each seizure type was counted. The 56 pa-
mographics, medical history, EEG, and neuroimaging findings, of tients showed 102 seizure types in nine categories. Simple motor
pediatric FLE patients who underwent video EEG monitoring, seizure (specifically, focal tonic seizure) was the most common
with a focus on describing the seizure semiology. Furthermore, we type (45/56, 80.4%), followed by aura (21/56, 37.5%). Nine pa-
analyzed the clinical manifestations of 10 patients with psychic au- tients had somatosensory auras (9/56, 16.1%), and nine had psy-
ras to identify any common aspects. chic auras (9/56, 16.1%).

https://doi.org/10.26815/acn.2022.00185 103
Lee D et al. • Pediatric Frontal Lobe Epilepsy

Table 1. Demographic characteristics (n=56) Table 2. Semiology, EEG, and brain MRI features of the 56 patients
Clinical characteristic Value
diagnosed with FLE
Sex Variable Value
Male 31 (55.4) Seizure semiology 102 (44.1)
Female 25 (44.6) Simple motor seizure 45 (44.1)
Age of onset (yr) 6.1 ± 4.4 (0.08–14)   Focal tonic 28 (27.5)
Past medical history   Focal clonic 13 (12.7)
No 39 (69.6)    Bilateral asymmetric tonic 4 (3.9)
Yes 17 (30.4) Aura 21 (20.6)
  Febrile seizure 5 (8.9)   Somatosensory 9 (8.8)
  Developmental delay 4 (7.1)   Psychic 9 (8.8)
  Intellectual disability 4 (7.1)   Autonomic 3 (2.9)
  Encephalitisa 2 (3.6)   Visual 3 (2.9)
  Cerebral hemorrhage 2 (3.6)   Abdominal 1 (1.0)
Family history   Vertiginous 1 (1.0)
No 51 (91.1)   Hypermotor seizure 17 (16.7)
Yesb 5 (8.9) Versive seizure 12 (11.8)
  Epilepsy 3 (5.4) Focal seizure with impaired awareness 4 (3.9)
  Febrile seizure 2 (3.6) Generalized tonic clonic seizure 2 (2.0)
No. of anti-seizure medications Automotor seizure 1 (1.0)
1 12 (21.4) Inteictal EEG 56 (100)
2 13 (23.2) Normal 10 (17.9)
3 12 (21.4) Non-epileptiform dischargesa 30 (53.6)
4 6 (10.7) Epileptiform discharges 43 (76.8)
5 1 (1.8) Brain MRI findings 56 (100)
6 2 (3.6) Normal 39 (69.6)
None 10 (17.9) Abnormal 17 (30.4)
Values are presented as number (%) or mean±standard deviation (range).    Focal cortical dysplasia 5 (29.4)
a
The patient was diagnosed with encephalitis based on clinical features    Heterotopic gray matter 3 (17.6)
such as altered mentality and seizure, and no pathogen was detected;   Neuroepithelial tumor 2 (11.8)
b
Of the three patients with a family history of epilepsy, two had a sibling
  Tuberous sclerosis 1 (5.9)
and one had a father with epilepsy. The fathers of two patients had febrile
seizures.    Hypoxic ischemic encephalopathy 1 (5.9)
   Tissue defect due to previous epilepsy surgery 1 (5.9)
  Porencephalic cyst 1 (5.9)
3. EEG   Hypothalamic hamartoma 1 (5.9)
Interictal EEG was normal in 10 patients (17.9%) (Table 2).   Cortical gliosis 1 (5.9)
Twenty patients had interictal epileptiform discharges with normal    Neonatal intracranial hemorrhage 1 (5.9)
background activity (35.7%); three patients had only non-epilepti- Values are presented as number (%).
form discharges, such as regional slow waves or generalized slow EEG, electroencephalography; MRI, magnetic resonance imaging; FLE,
frontal lobe epilepsy.
waves (5.4%) without any epileptiform discharges. Both epilepti- a
Background slow or diffuse or regional slow waves.
form and non-epileptiform discharges were observed in 23 pa-
tients (41.0%). The ictal rhythm showed various forms such as re-
petitive spikes or sharp waves, fast activity, or rhythmic alpha or tients (5/17, 29.4%) (Fig. 1). Other abnormal brain MRI findings
theta activity. The ictal onset zones were the left, right, both, or were heterotopic gray matter (n = 3, 17.6%), neuroepithelial tumor
midline frontal or central areas. (n = 2, 11.8%), tuberous sclerosis complex (n = 1), hypoxic isch-
emic encephalopathy (n= 1), tissue defect due to previous epilepsy
4. Neuroimaging surgery (n = 1), porencephalic cyst (n = 1), hypothalamic hamar-
Thirty-nine patients (69.6%) had normal structural brain MRI toma (n = 1), cortical gliosis (n = 1), and neonatal intracranial
findings. Abnormal structural lesions were observed in 17 patients hemorrhage (n = 1).
(30.4%) (Table 2). Focal cortical dysplasia was detected in five pa-

104 https://doi.org/10.26815/acn.2022.00185
Ann Child Neurol 2022;30(3):102-110

A B C

D E F

Fig. 1. Axial T2-weighted (A) and fluid attenuated inversion recovery (FLAIR) (B) images and a coronal FLAIR (C) image demonstrate high
signal intensity (white arrow) in the right frontal lobe, suggesting focal cortical dysplasia. Axial FLAIR (D) and coronal T2-weighted (E)
images show high signal intensity (white arrowheads) in the right precentral gyrus, suggesting a neuroepithelial tumor. An axial FLAIR
image (F) shows high signal intensity (red arrow) in the right frontal lobe, suggesting cortical gliosis.

5. Analysis of seizure semiology (Table 3). The dorsolateral cortex was the most common (28/56,
50.0%) onset zone (premotor cortex: 19/56, 33.9%; central cor-
1) Seizure frequency and types tex: 4/56, 7.1%; and prefrontal cortex: 2/56, 3.6%). Eleven pa-
Based on the medical records, 39 patients had daily seizures, nine tients (19.6%) had seizures originating in the mesial frontal lobe.
had weekly seizures, and eighth had monthly seizures. The average Six patients (10.7%) had diffuse frontal lobe seizure origins (in the
period of hospitalization for the 56 patients was approximately 2.6 dorsolateral and mesial frontal lobes).
days. The patients experienced 102 types of seizures in nine cate- The ictal onset zones of the 102 types of seizures were also ana-
gories during hospitalization, and a total of 641 seizures were ob- lyzed based on the four types of semiology commonly observed
served. About 1.8 types of seizures were observed per patient, and based on ictal EEG (Table 4). Focal tonic seizures commonly orig-
an average of 4.3 seizures were observed per day. Ten patients had inated in the premotor cortex (6/28 patients). In addition, mesial
nocturnal seizures, and none had clustering seizures. frontal cortex (5/28) and diffuse/non-localized onset (4/28) were
frequently observed. When focal clonic seizures occurred (n= 13),
2) Ictal onset zone and seizure semiology the EEG changes were diffuse/non-localized in six patients and lo-
Considering the division of the frontal lobe into the dorsolateral, calized to the premotor area in three patients. Hypermotor seizures
mesial, and basal frontal lobes and further the subdivision of the (n = 17) were accompanied by EEG changes in the prefrontal cor-
dorsolateral cortex into the prefrontal, premotor, and central corti- tex (4/17) and premotor cortex (3/17). Although auras revealed
ces, we classified the ictal onset zone observed for each patient no EEG changes in most cases (8/21), psychic auras often showed

https://doi.org/10.26815/acn.2022.00185 105
Lee D et al. • Pediatric Frontal Lobe Epilepsy

EEG changes. The ictal onset zone for psychic auras was the pre- lobe (n = 2), diffuse in the frontal lobe (n = 1), and diffuse/
frontal cortex in one case, the premotor cortex in two cases, the non-localized (n = 1). Three cases showed no EEG changes, and
mesial frontal lobe in three cases, and an undetermined location in in two cases, the ictal onset zone could not be determined (diffuse
one case; there was no change in two cases. background attenuation). The location of the lesion on brain MRI
coincided with the ictal onset zone on EEG in six cases. The le-
3) Correlation between anatomic abnormalities and the ictal sions were focal cortical dysplasia in two cases, neuroepithelial tu-
onset zone mor in two cases, heterotopic gray matter in one case, and tissue
In the analysis of the ictal onset zones of the 17 patients with ana- defect due to previous epilepsy surgery in one case. In the six pa-
tomical abnormalities on brain MRI, the most common localiza- tients with concordance between the location of the anatomic le-
tion of onset was observed to be in the dorsolateral frontal lobe sion on MRI and the EEG changes, the ictal onset zone was the
(8/17) (Table 5). The ictal onset zone was in the mesial frontal dorsolateral frontal lobe in five cases and the mesial frontal lobe in
one case.

Table 3. Anatomic location of the representative ictal onset zone 4) Patients with psychic auras
for each patient based on EEG changes
There were 10 patients with psychic auras (Table 6). Nine of
Location Value these patients showed psychic auras during long-term video EEG
Dorsolateral 28 (50.0)
monitoring. One patient did not have this type of seizure during
Prefrontal 2 (3.6)
hospitalization, but had them habitually otherwise (listed as pa-
Premotor 19 (33.9)
tient number 10 in Table 3). Patients complained of fears such as
Central 4 (7.1)
Prefrontal and premotor 1 (1.8) a feeling of being squeezed in the throat, being somewhere else,
Premotor and central 2 (3.6) being sucked into something, or everyone disappearing. EEG
Mesial frontal 11 (19.6) changes were observed in eight of the nine patients, mainly in the
Diffuse frontal 6 (10.7) frontal or central areas, when they had a psychic aura. Only one
Generalizeda 3 (5.4) patient showed no EEG changes. Patients with psychic auras had
Multifocal 1 (1.8) several types of motor seizures and other types of auras as well,
Undeterminedb 3 (5.4) such as autonomic or visual auras. One patient (number 6)
Unknownc 4 (7.1) showed a structural abnormality on brain MRI (heterotopic gray
Values are presented as number (%). matter in the right periventricular white matter) (Fig. 2). In this
EEG, electroencephalography.
a
There were ictal EEG patterns such as generalized repetitive spikes or sharp patient, ictal changes on EEG were observed in the midline fron-
waves or generalized spike and slow waves; bBackground attenuation/ tocentral areas.
suppression or no EEG changes; cArtifact-obscured EEG.

Table 4. Anatomic location of the ictal onset zone based on electroencephalography in four commonly observed types of semiology
Variable Focal tonic (n = 28) Focal clonic (n= 13) Hypermotor (n= 17) Aura (n= 21)
Dorsolateral
Prefrontal 2 4 1
Premotor 6 3 3 2
Central 3 1 1 2
Mesial frontal 5 1 2 4
Diffuse frontal 1 1 1
Hemisphere 1
Generalizeda 4 6 3
Multifocal
Undeterminedb 3 2 8
Unknownc 2 1 3
Others except frontal lobe 2 1
a
There were ictal electroencephalography (EEG) patterns such as generalized repetitive spikes or sharp waves or generalized spike and slow waves;
b
Background attenuation/suppression or no EEG changes; cArtifact-obscured EEG.

106 https://doi.org/10.26815/acn.2022.00185
Ann Child Neurol 2022;30(3):102-110

Table 5. Concordance between the location of the ictal onset zone based on EEG and MRI lesions in 17 patients with structural
abnormalities
MRI findings
Patient Sex Age (yr) Ictal onset zone in EEG Concordancea
Etiology Location of the lesion
1 F 3 Dorsolateral HGM Lt. postcentral gyrus Yes
2 F 9 Diffuse frontal Cortical gliosis Lt. basal ganglia No
3 M 15 Dorsolateral FCD Rt. superior frontal gyrus Yes
4 M 5 Dorsolateral HIE Basal ganglia, thalamus, multiple white matter No
5 M 15 General Neonatal ICH Unknown (no image) No
6 M 16 Dorsolateral FCD Rt. postcentral gyrus Yes
7 F 12 Dorsolateral TSC Sporadic No
8 M 16 Undetermined Porencephalic cyst Rt. parietal lobe No
9 F 14 Dorsolateral Tissue defectb Lt. superior frontal gyrus Yes
10 F 10 Mesial HGM Rt. postcentral gyrus No
11 M 19 Undetermined FCD Lt. superior frontal gyrus No
12 M 8 Dorsolateral FCD Rt. basal frontal lobe No
13 M 17 Unknown FCD Rt. middle frontal gyrus No
14 M 1 Dorsolateral Neuroepithelial tumor Rt. precentral gyrus Yes
15 F 10 Mesial Neuroepithelial tumor Lt. paracentral gyrus Yes
16 F 11 Unknown HGM Rt. middle frontal gyrus No
17 F 2 Unknown HH Hypothalamus No
EEG, electroencephalography; MRI, magnetic resonance imaging; HGM, heterotopic gray matter; Lt., left; FCD, focal cortical dysplasia; Rt., right; HIE, hypoxic
ischemic encephalopathy; ICH, intracranial hemorrhage; TSC, tuberous sclerosis; HH, hypothalamic hamartoma.
a
Concordance between the ictal onset zone and the location of the MRI lesion; bTissue defect due to previous epilepsy surgery.

Discussion zures were frequent and stereotypic and had rapid onset and offset.
In their study, semiology was classified into six categories, among
This retrospective study describes the characteristics of pediatric which hypermotor seizures (20/22) and psychic auras (15/22)
patients with FLE based on an analysis of their actual seizures us- were the most common. However, in our study, where semiology
ing long-term video EEG monitoring. The seizure semiology ob- was classified into nine categories, simple motor seizures were the
served was diverse, and included simple motor seizures, hypermo- most common type (45/56, 80.4%), and hypermotor seizures
tor seizures, and auras. Only 17.5% (10/57) of patients had noc- were the third most common type (17/56, 30.4%). Auras were the
turnal seizures and no patient had clustering seizures in long-term second most common type (21/56, 37.5%), and psychic auras
video EEG monitoring, although many of them had nocturnal and were found in only nine patients (16.1%). Compared with previ-
clustering seizures in their history. The dorsolateral cortex was the ous studies, this study was conducted with a larger number of pa-
most common ictal onset zone (28/56, 50.0%; premotor cortex: tients. It is significant in that it analyzed all the ictal onset zones of
19/56, 33.9%; central cortex: 4/56, 7.1%; prefrontal cortex: 2/56, 641 ictal events and correlated them with the semiology.
3.6%). Focal tonic seizures often began with EEG changes in the In our study, MRI revealed that 30.4% of patients (17/56) had
premotor or mesial frontal cortices. The lesion location on brain various abnormalities, including focal cortical dysplasia (n = 5),
MRI matched the ictal onset zone on EEG in six patients. heterotopic gray matter (n = 3), and neuroepithelial tumor (n= 2).
In this study, we classified semiology into nine categories. Focal The abnormal findings of brain MRI in our study were different
tonic seizures (50.0%), psychic auras (16.1%), and hypermotor from those found in adult FLE patients. In a previous study includ-
seizures (30.4%) were the most frequent categories. Interictal EEG ing 36 adult patients, encephalomalacia (8/17, 47.1%) was the
was mostly normal, and was well-localized in 80.4% (45/56) and most common etiology, followed by traumatic brain injury, post-
poorly localized in 19.6% (11/56) of patients (Table 3). In a previ- operative changes, malformation of cortical development (6/17,
ous study that included 22 pediatric patients with FLE, the semiol- 35.3%), and vascular malformations (2/17, 11.8%) [9]. In a pedi-
ogy was as follows: focal tonic, focal clonic, hypermotor, versive, atric study, 14.3% to 100.0% of patients had focal cortical dysplasia
dialeptic, and psychic aura [1]. They reported that frontal lobe sei- [1,2], whereas in our study, only 8.9% (5/56) had focal cortical

https://doi.org/10.26815/acn.2022.00185 107
Table 6. Clinical features, EEG findings, neuroimaging, and ASM history of 10 patients presenting with psychic auras (patient 10 did not show psychic auras during hospitalization)

108
Age at Other sz semiology Scalp EEG
Age at
video Past medical Family Ictal onset zone
Patient Sex onset of MRI ASM
recording history history Motor sz Aura Interictal
seizure (yr) Psychic aura Other sz
(yr)
1 F 16 5 N N Hypermotor sz Autonomic Normal Bi-Fr Bi-Fr, gen Normal CBZ
2 F 14 5 N N Rt. facial tonic, hemi Autonomic Lt. Fr or hemi Lt. Fr No ictal Normal OXC
clonic, frontal ab- events
sence, and versive sz
3 M 8 8 N N Rt. arm tonic sz N Rt. or both Fr Lt. Fr No ictal Normal CLN, LEV
events
4 M 17 13 Subependymal N None N Normal Midline or bi No ictal Normal LMT, OXC
hemorrhage and Fr, Lt. FrT, or events
Lee D et al. • Pediatric Frontal Lobe Epilepsy

neonatal seizure gen


5 M 1 1.3 Febrile seizure N GTC sz N Lt., Rt., or Lt. C Lt. or midline Normal DPH, LEV, PB
midline C C
6 F 10 4 N N Lt. arm tonic, and ver- N Midline FrC Midline FrC Midline FrC Heterotopic gray matter CBZ, LEV, TPM,
sive sz in the Rt. periventricu- VPA
lar white matter
7 M 8 7 N N GTC sz Visual Lt. C, Rt. CT, or No EEG Rt. C, gen Normal OXC
midline P change
8 M 5 3 N N Hypermotor, and versive Visual Lt. and/or Rt. Midline Fr or Midline Fr or Normal OXC
sz or midline Fr FrC FrC
9 M 18 1 N N None N Normal Gen No ictal Normal CBZ, CLB
events
10 M 17 13 N N Rt. facial, arm, leg, or N Normal No ictal Lt. Fr Normal CLB, LMT,
hemi tonic or clonic, events TPM, VPA
Lt. hemi tonic, and
versive sz
EEG, electroencephalography; ASM, anti-seizure medication; sz, seizure; MRI, magnetic resonance imaging; F, female; N, none; bi, bilateral; Fr, frontal; gen, generalized; CBZ, carbamazepine; Rt., right;
Lt., left; OXC, oxcarbazepine; M, male; CLN, clonazepam; LEV, levetiracetam; T, temporal; LMT, lamotrigine; GTC, generalized tonic-clonic; C, central; DPH, diphenylhydantoin; PB, phenobarbital; TPM,
topiramate; VPA, valproate; CT, computed tomography; P, parietal; CLB, clobazam.

https://doi.org/10.26815/acn.2022.00185
Ann Child Neurol 2022;30(3):102-110

A B C D

Fig. 2. Axial and coronal T2-weighted (A, C) and fluid attenuated inversion recovery (B, D) images show high signal intensity (arrows)
with an irregular margin in the right periventricular white matter, suggesting heterotopic gray matter.

dysplasia. The abnormal brain MRI findings observed in the previ- nosed with FLE. Brain MRI findings were normal in two-thirds of
ous pediatric studies were only focal cortical dysplasia. patients, and interictal EEG did not reveal epileptiform discharges
Long-term video EEG monitoring was mainly conducted to dif- in 23.2% of the patients. Because interictal EEG and brain MRI are
ferentiate semiology in this study, but it has been used in previous often normal in patients with FLE, it is important to understand
studies for the purpose of presurgical evaluations of patients with the characteristics of frontal lobe seizures in order to diagnose FLE
anatomic abnormalities on MRI. Therefore, the proportion of fo- and differentiate these seizures clinically. To determine the ictal on-
cal cortical dysplasia among the patients was different. set zone in the patients already diagnosed with FLE, it is necessary
Lesions located deep in the brain (9/17, 52.9%) had poor EEG to understand the functional anatomy of the frontal lobe and sei-
correlations, whereas lesions near the cortex (8/17, 47.1%) tended zure semiology and to confirm the ictal EEG through long-term
to be well correlated (6/8). The reason for the poor correlation is video EEG. Reports of semiology on the basis of home videos ob-
thought to be that the epileptiform discharges become rapid and tained by parents and interictal EEG will be the first steps in local-
widely-propagating because of the rich connectivity between the izing the ictal onset zone. Thereafter, long-term video EEG may be
frontal lobe and other lobes [9]. This proposal is supported by re- helpful for further localization. For FLE with a nonlocalized struc-
ports that the introduction of stereoelectroencephalography tural etiology, sEEG will be helpful for defining the ictal onset
(sEEG) is helpful for confirming ictal spreading [4,8]. A study con- zone.
ducted by Bonini et al. [4] on 54 patients with adult FLE showed
the process of clear localization using sEEG; seizures were catego- Conflicts of interest
rized based on 31 ictal signs, and the electrode at which each sei-
zure started was confirmed, leading to the finding of meaningful Jeehun Lee is an editorial board member of the journal, but he was
correlations between semiology and anatomy. Because our study not involved in the peer reviewer selection, evaluation, or decision
was based on scalp EEG, there was a limitation in determining the process of this article. No other potential conflicts of interest rele-
precise ictal onset zone. In routine clinical practice, if the ictal onset vant to this article were reported.
zone cannot be precisely localized using scalp EEG, sEEG could be
considered for surgical planning. ORCID
This study has several limitations. Because this study was fo-
cused on pediatric FLE patients who had ictal events during hospi- Dajeong Lee, https://orcid.org/0000-0002-6158-3663
talization, it did not represent all pediatric FLE patients. In addi- Jeehun Lee, https://orcid.org/0000-0002-8499-3307
tion, because the patients in this study showed various clinical
characteristics, seizure semiology, EEG, and brain MRI findings, a Author contribution
limitation is that it was not possible to perform a statistical analysis
because the number of patients in the groups to be compared was Conceptualization: DL, JL, and JL. Data curation: DL. Formal
small. analysis: DL and JL. Project administration: DL. Visualization:
In conclusion, this study summarizes the characteristics of pedi- DL, JL, and JL. Writing-original draft: DL. Writing-review & edit-
atric patients admitted to our epilepsy monitoring unit and diag- ing: JL and JL.

https://doi.org/10.26815/acn.2022.00185 109
Lee D et al. • Pediatric Frontal Lobe Epilepsy

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