2nd English Case Report To whom respectfully

dr. Melisha L. Gaya Dr …………………………………..

ATYPICAL ABSENCE SEIZURE

Introduction
Absence seizure, formerly termed petit mal seizure, is an idiopathic, generalized
non-convulsive epilepsy with a multifactorial genetic etiology, which
characterized by an abrupt cessation of activity, change in facial expression, and
impairment of consciousness. Absence seizure is not common, accounting for less
than 10% of all seizure types.1-2
Absence seizures are classified as typical or atypical in type. Main features
of typical absence seizure are a sudden, brief impairment of consciousness,
usually associated with a blank facial appearance without other motor or
behavioral phenomena, that is accompanied by a generalized, synchronous,
bilateral, 2.5–4 Hz spike and slow-wave discharge (SWD) in the
electroencephalogram (EEG).1-3-4 Atypical absence seizure is more likely to be
initiated by diminished postural tone, or tonic or myoclonic activity.1 It has similar
but more severe motoric behavior as typical absence (such as atonia which causes
the sufferer to fall), but the impairment of consciousness happens slower and
more progressive. Restoration of consciousness takes longer, with < 2,5 Hz spike-
wave in EEG.4
Seizures in childhood absence epilepsy start between 3-8 years of age
(peaking at 6-7 years), generally occur many times each day (up to 200 times per
day),5 they may be subtle and often go unnoticed for prolonged periods, even with
multiple seizures per day. It remits by early puberty in most patients without
sequelae, although persistent cognitive or psychiatric comorbidities may be
present in some children.6

1
Case Report
A male patient aged 7 years and 9 months came to outpatient clinic of Dr. M.
Djamil Hospital in March 2020.
Chief complaint: Lack of attention and blank when studying.
Present Illness History
The patient has been known as absence epilepsy since March 2019. The patient
started having seizures since August 2018, the patient walks back and forth
holding objects around the patient, scribbling books while writing homework,
flipping pages of books while reading or reciting, didn't answer when summoned,
with an unfocused gaze. The seizures lasted for ± 3-5 seconds, the frequency was
± 5-10 times / day, the seizures stopped on their own, the patient was conscious
after the seizure. Patients routinely consume valproic acid, folic acid, and vitamin
B6 from pediatricians from August 2019 to April 2020, but since April 2020
treatment has been stopped by the family with the reason of Covid pandemic and
time constraints. The last dose of valproic acid that the patient took was 16 mg /
kg / day. During taking the medication, there were still complaints of seizures but
the frequency and duration were reduced. Fever was not there. Nausea and
vomiting were absent.
Cough, runny nose and shortness of breath, and blueness are absent. History of
watery ears is absent. There is no history of contact with people with long coughs.
Currently the patient is in grade 2 elementary school, the achievement in school is
quite good, the patient always gets the top 3 rank in his class.
 
Past Illness History
Never had a seizure with fever preceded before. A history of falling from height
± 1 meter with the back of the head hitting the ground in February 2018, not
accompanied by nausea, vomiting, or loss of consciousness and not taken any
treatment.

2
Family Illness History
The patient's biological grandmother was a sufferer of senile heart disease and
cataracts. No family member complained of seizures with or without fever. There
are no family members who suffer from prolonged coughs
 
History of Delivery, Immunization and Development
The patient is the first child of 3 siblings, born spontaneously, helped by a
midwife, birthweight 2900 gr, body length 50 cm, immediately burst into tears.
There was no history of shortness of breath or blueness. Complete basic
immunization history. Has received Td immunization at the age of 7 years.
 
Family and Social Economy History
Biological mother, 36 years old, graduated as bachelor, works as a teacher. The
biological father is 44 years old, graduated from D3, is a private employee. The
patient lives with his aunt's family (mother's younger sibling), 42 years old aunt,
bachelor, works as a teacher with an income of ± IDR 5,000,000 / month, 42-year-
old uncle, graduated from vocational school, works as a security officer for ± IDR
3,000 000 / month. The patient and his family live in a permanent house with
good environmental hygiene and sanitation.
 
Physical Examination
The patient looks moderately ill, conscious, pulse 110 beats per minute, breaths
22 times per minute, temperature 36.9°C , body weight 25 kg, height 128 cm.
Weight for age was 100%, length for age was 100.7%, weight for length was
96.1% , with an impression of good nutritional status. There was no anemic,
edema, jaundice nor cyanosis. Skin was warm, turgor return rapidly. There was no
regional lymph enlargement. Head was round and symmetric, head circumference
53 cm (normal based on Nellhauss standard), hair was black and not easily be
pulled, conjunctiva were not anemic, sclera were not icteric, pupil isochors with
diameter 2mm / 2mm, light reflex was positive normal. No abnormality on ears.
No nasal flare. Tonsil was T1-T1, not hyperemic and pharynx was not hyperemic.
Mouth's mucous was wet, no oral trush, no cyanosis. The chest was symmetric

3
and no retraction. On lungs examination, phremitus was difficult to be assessed,
and there was no rales nor wheezing on both lungs. On heart examination, apex
couldn't be seen in inspection but palpable at 2 finger medial of left
midclavicularis line, intercostal space V, heart sound was regular rhythm without
murmur. There were no abdominal distension, liver was palpable ¼-¼, sharp
edge, rubbery consistency, flat surface, spleen was not palpable, peristaltic sound
was normal. No abnormality found in genitalia, puberty state A1M1P1.
Extremities were warm with good perfusion.
Laboratory finding:
Hematology
Complete blood count (CBC): Haemoglobin 12 gr/dl, white blood cell 6.810/mm3,
differential count 0/11/4/33/49/3, hematocrite 37%, platelets 259.000/mm3, red
blood cell 4,49x106/mm3, reticulocyte 1,1%, sodium 140 mmol/l, potassium 4,1
mmol/l, calcium 8,2 mg/dl, SGOT 19, SGPT 10 Interpretation: laboratory result in
normal range.
Urinalisis: colour: yellow, turbidity: negative, leucocyte 0-1/HPF, erythrocyte 1-
2/HPF, cilinder hyalin negative, protein (-), reduction (-), bilirubin (-),
urobilinogen (-).
Routine stool: Normal

List of Problem
1. Epileptic Seizure

Working Diagnosis
1. Suspected absance type epilepsy

Management
1. Absance type epileptic seizure
a. Diagnostic: anamnesis, physical examination, laboratory finding
b. Treatment
Folic Acid 1 x 1 mg
Vitamin B6 1 x 10 mg

4
 Depakene 2x4cc
c. Planning: EEG test
d. Education:
The parents were given information about the disease, diagnosis,
management, complication, and prognosis.
 Management : epileptic drugs
 Monitoring : before, during and after seizure
 Complication : ADHD
 Prognosis : dubia et bonam
EEG pada tanggal 22 Februari 2019 :
   The recording was done in a conscious state without premedication,
cooperatively. In the awake recording, the background rhythm of alpha waves,
with a frequency of 8-13 Hz and an amplitude of up to 80, is symmetrical and
reactive. There is an abnormality in the form of waves 2-3 Hz Spike and Wave
Complex, comprehensive, at the time of hyperventilation. When recording in a
sleep state, slow eye movement is obtained. There are no abnormalities.
Impression: Abnormal EEG with 2-3 Hz Spike Wave Complex Suggestive to the
diagnosis of Absence Seizure Epilepsy.

5
6
7
8
Laboratory examination (February 18th, 2019):
Examination Result Interpretation
Hemoglobin 13,5 g/dl Normal
leukocyte 8,420 /mm3 Normal

9
 Trombocyte 353,000/mm3 Normal
Hematocrit 40 % Normal
SGOT 26 u/l Normal
SGPT 12 u/l Normal

Control March 1 5 th 2019

Patient control as suspect epilepsy. The child does not turn around when the child
is too active. The child is moderately ill and conscious. From the EEG
examination, abnormal results were obtained with waves of 2 - 3 Hz complex
spike waves suggestive of absence seizure epilepsy. Laboratory result SGOT 26
u / l, SGPT 12 u / l, hemoglobin 13.5 mg / dl, leukocytes 8,420 / mm 3 ,
hematocrit 40%. The patient was diagnosed with epilepsy. Treatment: Depakene
syrup from 10mg / kg / day.
 
Control April 12 th 2019
Patient control with epilepsy. The last seizure was 1 day ago. There are still absent
spasms. The child is moderately ill and conscious. Children are advised to do a
complete blood count, SGOT and SGPT. Treatment: Depakene syrup 2x250mg,
Folic Acid 1x1 tablet and Vitamin B6 1x1 tablet.

Control May 3 rd 2019

Control children diagnosed with epilepsy. The child is still having absent spasms.
The child had the last seizure 1 day before admission to the hospital. The child
does not have a fever. Children do not look sick, heart rate 100 times / minute,
respiratory rate 20 times / minute, temperature 36.8 ° C . Conjuctiva was not
anemic, sclera was not icteric . There is no retraction on chest, no distended
abdomen, there is no palpable liver and spleen. Extremities were warm with good
perfusion. The patient diagnosed with epilepsy. Treatment: Depakene syrup
2x400
mg, Folic Acid 1x1mg and Vitamin B6 1x10 mg.

LITERATURE REVIEW
Definition

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 Childhood absence epilepsy (CAE) is a common generalized epilepsy
syndrome with a presumed genetic cause, characterized by typical absence
seizures (TAS) appearing in otherwise healthy school-aged children. Its main
features are a sudden, brief impairment of consciousness that is accompanied by a
generalized, synchronous, bilateral, 2.5–4-Hz spike and slow-wave discharge
(SWD) in the electroencephalogram (EEG)1.

Epidemiology
Atypical absences usually begin before the age of 5 years and often are associated
with other seizure types and mental retardation.1
Children absence epilepsy (CAE) is one of the most common forms of pediatric
epilepsy, accounting for 12 percent of epilepsy diagnoses in one prospective
community-based study that included 613 children with epilepsy aged 0 to 16
years4. Of note, estimates of the annual incidence of CAE are derived from studies
performed in primarily Caucasian populations. In a retrospective analysis of EEG
findings of Swedish children aged 0 to 15 years, the annual incidence of "absence
epilepsy" was 6.3 per 100,000. In another report based on a questionnaire sent to
patients with seizures in southwest France, the annual incidence of CAE was
estimated at 8 per 100,000 [4]. CAE is one of the rare epilepsy syndromes in
which prevalence is higher in girls than boys 5,6.
The prevalence of absence seizures is highest during the first 10 years of life and
then drops dramatically to a very low level. Absence seizures are more common
in girls than in boys. Absence seizures can begin as early as the first year of life.1
Etiology
CAE is classified as an epilepsy syndrome with presumed genetic cause
according to the current International League Against Epilepsy (ILAE)
classification system. In previous versions of the classification system, CAE was
referred to as an idiopathic syndrome7.
A genetic cause has been suggested by family and twin studies, although
clear single-gene defects that reliably result in a typical CAE phenotype have not
yet been identified. Family studies indicate a 17 percent risk of having typical

11
absence seizures (TAS) in first-degree relatives of patients with CAE, and there is
a higher concordance rate for CAE in monozygotic than dizygotic pairs8.
Several candidate regions and genes have been identified as possible
susceptibility loci in CAE. Copy number variations and microdeletions in regions
such as 8q and 15q have been reported in a minority of patients with generalized
epilepsy syndromes, including some with CAE. One group performed sequencing
of the 15q candidate region in 380 patients with CAE and identified heterozygous
point mutations in NIPA2, a gene that encodes for a selective magnesium
transporter, in 3 of 380 patients with CAE and in none of the 400 controls9.

Pathogenesis and Pathophysiology

Studies in humans and rodent models have implicated a variety of cortical
and thalamocortical circuits in the genesis of typical absence seizures (TAS).
Most data point to prominent involvement of deep layers of the frontal and
somatosensory cortex in the generation of slow wave discharges, with rapid
spread to other regions of the cortex and secondary invasion of the thalamus 10.
The orbital-medial frontal and medial-lateral parietal cortices have been
implicated in studies utilizing simultaneous EEG and functional MRI during
typical slow wave discharges11. In another study, the posterior cortical regions
were active at the onset of the slow wave discharge, followed by spread to other
areas later in the seizure, including the thalamic medial dorsal nuclei and the
thalamostriate network12.
Animal data indicate that particular cortical and thalamic excitability and
coupling conditions must occur to produce the slow wave discharges of TAS.
Enhanced tonic gamma-aminobutyric acid A (GABA-A) receptor inhibition in
thalamocortical neurons has been demonstrated to be necessary and sufficient for
the expression of TAS in various pharmacologic and genetic mouse models. Such
inhibition is due to increased extracellular GABA concentrations related to
dysfunction of GAT-1, an astrocytic transporter involved in GABA synaptic
uptake13.

Diagnosis

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 Simple behaviors, such as rubbing the face or hands, licking the lips,
chewing, grimacing, or fumbling with clothes, tend to be de no novo automatisms.
Speech, if it occurs during the seizure, usually is perseverative and may be slow
and slurred, but also may be totally normal. 1 It is accompanied by semi purposeful
behaviors in which the patient is unaware and subsequently cannot recall.1

Typical absence seizures (TAS) present as short and frequent episodes of

profound impairment of consciousness without loss of body tone, lasting about 10
seconds. They are abrupt in onset and termination and easily provoked by
hyperventilation. These typically come to the attention of parents or teachers after
a prolonged period of observation, despite their high frequency. TAS may occur
tens of times daily, but their subtle clinical symptoms allow them to go unnoticed
or misdiagnosed as inattention. The electroclinical spectrum of TAS in CAE is
illustrated by two studies that reviewed a combined total of over 2000 absence
seizures in more than 450 drug-naïve children with a new diagnosis of CAE 14:
> Earliest clinical sign – Approximately half of all recorded seizures begin with
an arrest in activity, which is typically complete. Less common first signs include
eyelid movements, eye opening, and oral automatisms.
> Ictal signs and symptoms – The majority of electrographic seizures
(approximately 80 percent) are associated with at least one clinical sign. The three
most common seizure features are pause/stare (80 to 95 percent), motor
automatisms (40 to 60 percent), and eye involvement (55 percent). About one-
third of seizures involve three hertz (Hz) regular eyelid movements. Motor
automatisms are predominantly oral.
> Seizure duration – The average seizure duration is 9 to 10 seconds.
Approximately 25 percent of seizures last less than four seconds and
approximately 10 percent are longer than 20 seconds.

Hyperventilation is an effective trigger of typically absance seizure,

provoking seizures in 90 percent of children old enough to perform the maneuver.
TAS are not provoked by sensory or visual stimuli. The presence of violent limb
myoclonias, head nods, hypotonia, or focal signs are not consistent with TAS and

13
should raise suspicion for other types of seizures, such as atypical absences or
focal seizures. In addition, atypical absences should be suspected if the duration of
the events exceeds the classic 10 to 20 seconds of TAS, especially if onset and
termination are progressive rather than abrupt14.
Children with CAE typically have intact cognition and intellect at the time
of diagnosis, although formal neurocognitive testing may detect mild deficits in a
variety of domains, particularly executive function. Similar findings have also
been reported in children with other generalized epilepsy syndromes that are
typically associated with a favorable prognosis. In these studies, cognitive, social,
emotional, or psychiatric comorbidities frequently antedate the epilepsy onset and
require special educational interventions [54-59].

Electroencephalography
One of important examination is electroencephalography (EEG). EEG in
patients with suspected CAE should be a sleep-deprived video-EEG study that
includes both intermittent photic stimulation (IPS) and hyperventilation (HV) in
order to maximize the chance of recording an absence seizure. Among 47
consecutive patients with newly diagnosed CAE, diagnostic 30-minute sleep-
deprived video EEG recordings captured an average of 6 seizures per child; of
these, 47 percent occurred with HV, 25 percent during drowsiness, 13 percent
during the awake state, 9 percent during IPS, and 7 percent during sleep. At least
one seizure was provoked during HV in 83 percent of children14.
1. Interictal EEG
The interictal EEG in CAE shows normal background activity, although it
is common to see occasional generalized spike wave discharges or focal
abnormalities. In a review 445 pre-treatment EEGs, occipital intermittent
rhythmic delta activity (OIRDA) was noted in 21 percent, focal sharp waves in
2.5 percent, and focal slowing in 0.7 percent [64]. In another study, focal
intermittent paroxysmal activity was noted in 38 percent of 29 children with CAE,
consisting in most cases of frontal spike wave or polyspike and wave discharges.
2. Ictal EEG

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 The EEG appearance of a typical absence seizure (TAS) consists of
generalized 2.5 to 5 Herz (Hz) spike wave discharges (classically, 3 Hz) with
abrupt onset and termination. Spike wave discharges are frequently disorganized,
and pre- and post-ictal slowing is often present.

The morphologic characteristics of the spike wave discharges vary across

recordings. In one study, 87 percent of spike wave discharges contained only one
or two spikes per wave during the seizure; four or more spikes per wave were
present in 8 percent of the 47 children when analyzed individually. In another
study, single spike wave discharges were noted in 604 of 721 discharges (84
percent), whereas polyspike wave discharges were present in only 3 percent14.
In up to 50 percent of cases, the earliest ictal abnormalities are focal. This
is often predominant in the frontal leads bilaterally, a phenomenon referred to as
"frontal lead-in" or "frontal absences" by some authors. Similar features have also
been reported arising from the occipital lobes. The ictal EEG of atypical absences
usually shows discharges of irregular generalized spike waves of lower frequency
(< 2.5 Hz) than those observed in TAS14.

Neuroimaging
Brain magnetic resonance imaging (MRI) is by definition normal in
patients with CAE, and structural imaging is not necessary for the diagnosis if
patients have typical clinical and EEG findings. Imaging is indicated only if there
are focal findings on clinical or EEG evaluation. High-resolution structural
imaging has been used to demonstrate subtle anatomical variation in various brain
regions in patients with CAE, although the functional and clinical significance of
these findings is uncertain. Reported abnormalities include decreased thalamic
grey matter volume and subcallosal gyrus atrophy, grey matter loss in the left
orbital-frontal gyrus and bilateral temporal lobes, and amygdala volume loss in
CAE patients with symptoms of attention deficit hyperactivity disorder15.
CAE is diagnosed based on history, physical examination, and video-EEG
findings. The most widely accepted diagnostic criteria were proposed by
Panayiotopoulos in 1997 and accepted by the International League Against

15
Epilepsy (ILAE) in 2005. According to these criteria, a diagnosis of CAE requires
all of the following16:
-Age at onset of 4 to 10 years
- Normal neurological and developmental state
- Brief (4 to 20 seconds) and frequent (tens per day) absence seizures, with abrupt
and severe loss of consciousness
- Generalized rhythmic spikes or double spike wave discharges at around 3 Hz
In addition, the following criteria are exclusionary for the diagnosis of CAE:
- Other seizure types preceding or observed during the active stage of typical
absence seizures
- Massive and sustained eyelid, perioral, head or limb myoclonias
- Mild or no impairment of consciousness during the spike wave discharge
- Spike wave discharges of less than 4 seconds
- More than 3 spikes or spike wave fragmentation
- Visual or other sensory seizure precipitants
Children with TAS beginning before the age of three who otherwise fulfill
the above criteria may show a similarly favorable clinical course as those with
CAE 17.

Management
Typical absence seizures are characteristically extremely frequent,
occurring multiple times each day. Once they are recognized, they should be
treated to reach the therapy goals to improve patients quality of life, school
performance, and social acceptance, and possibly to reduce the risk of (rarely)
associated convulsive seizures. Complete seizure freedom can often be attained by
pharmacologic treatment.
Ethosuximide is recommended as first-line therapy in most children with
CAE. This recommendation is supported by results of a randomized, double-
blind trial that compared the efficacy and tolerability of ethosuximide, valproate,
and lamotrigine in 453 children with CAE. After 16 weeks of treatment,
ethosuximide and valproate were significantly more effective than lamotrigine
(16-week freedom from seizure rates of 53, 58, and 29 percent, respectively), and

16
the rate of drug discontinuation for adverse effects was similar among the three
groups. However, valproate was associated with more frequent attentional
dysfunction than ethosuximide (49 versus 33 percent). A follow-up study that
assessed the sameparameters after 12 months of treatment also confirmed that
ethosuximide had better efficacy than lamotrigine and fewer side effects than
valproate18.
The typical starting dose of ethosuximide is 5 to 10 mg/kg/day in two
divided doses for children younger than six years, and 250 mg twice daily for
children age six years and older. The usual maintenance dose is 15 to 40
mg/kg/day in divided doses. Blood levels should be checked initially after one to
three weeks, with a goal therapeutic concentration of 40 to 100 mcg/mL.
The most common side effects include nausea, vomiting, sleep
disturbance, drowsiness, and hyperactivity. Initial monotherapy should not be
abandoned before ensuring that the maximum tolerated dose has been achieved. In
most cases, response can be assessed clinically, as TAS are usually easy to follow
once they are recognized. In some cases, EEG can be used to aid in determining
the response to therapy if the clinical improvement is unclear after two to four
weeks of therapy3.
Patients who fail or do not tolerate first-line therapy — In patients who
have inadequate control of TAS or intolerable side effects on ethosuximide, we
suggest switching to valproate monotherapy, although lamotrigine may be a better
alternative in females of childbearing age based on the increasingly well
characterized fetal risks of valproate3.
In the randomized trial described above, 208 patients who experienced
treatment failure during the double-blind phase were enrolled in an open-label
extension study and randomly assigned to second monotherapy with one of the
remaining two study drugs. After 16 to 20 weeks of therapy, freedom from seizure
rates were higher for ethosuximide and valproate (53 and 58 percent, respectively)
than for lamotrigine (29 percent). For ethosuximide and valproate, these response
rates are comparable with those observed in the first-line setting and support the
practice of using second monotherapy rather than add-on therapy in children who
do not respond adequately to first-line therapy3.

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 For patients who develop generalized tonic clonic seizures on
ethosuximide, valproate is also an effective second-line therapy, either as an add-
on agent or as monotherapy, since it may be effective for both seizure types.
Additional options for refractory CAE include topiramate, benzodiazepines,
acetazolamide, ketogenic dietary therapy, and vagus nerve stimulation. For all of
theseoptions, trials that formally evaluate their effectiveness and side effect profile
are lacking [84]; however, observational data suggest benefit3.
Vagus nerve stimulation has been shown to reduce seizure frequency in
six patients with CAE and refractory absences. There are mixed reports from
small studies with regard to levetiracetam in CAE, with one study suggesting
efficacy, another reporting aggravation of absences in certain patients, and a third
study of 72 children showing apparent benefit in 25 percent but a high rate of
discontinuation (74 percent) due to poor seizure control and/or side effects19.
Several antiseizure drugs have the potential to aggravate absence seizures
in patients with CAE and should be avoided. These include carbamazepine,
vigabatrin, gabapentin, and tiagabine. Phenytoin and phenobarbital are known for
their ineffectiveness in treating absences and should also be avoided3.
In most cases, seizures respond well to first-line drug therapy and remit
before puberty. Antiseizure drug therapy should be continued for a minimum of
two years of seizure freedom. After this period, progressive tapering of antiseizure
drugs can be considered3.

Outcome
Over 90 percent of children with CAE diagnosed according to the 1989
ILAE classification were seizure free after a mean follow-up of 15 years. Those
who fulfilled the 2005 criteria had fewer generalized tonic clonic seizures, but
their final overall outcome was not significantly different than those who did not.
The total duration of epilepsy and the mean age at final remission were 3.9 and
9.5 years, respectively. Epilepsy duration was longer in those children who had
become seizure-free more than six months after treatment initiation20.
A relatively small proportion of children with typical absence seizures are
refractory to typical therapeutic approaches for a prolonged period. In one study

18
of 92 such children with refractory typical absence seizures, approximately 50
percent eventually achieved prolonged seizure freedom with or without
antiseizure drugs20.

CASE ANALYSIS
[JR 3] A typical absence is a non-convulsive epileptic seizure. Its main features
are a sudden, brief impairment of consciousness that is accompanied by a
generalized, synchronous, bilateral, 2.5–4-Hz spike and slow-wave discharge
(SWD) in the electroencephalogram (EEG)1. The diagnosis of absence epilepsy in
children is diagnosed based on history, physical examination, and video-EEG
findings. We present a case of a 7,9 years old boy with absence epilepsy. The
incidence of absence epilepsy accounting for 12 percent of epilepsy diagnoses in
one prospective community-based study that included 613 children with epilepsy
aged 0 to 16 years. In a retrospective analysis of EEG findings of Swedish
children aged 0 to 15 years, the annual incidence of "absence epilepsy" was 6.3
per 100,0003.
  In this case, a 7.9 years old boy came to the outpatient clinic of RSUP M
Djamil Padang Hospital for control with the main complaint of lack of attention
and blankness while studying. The patient has been known to suffer from absence
epilepsy since March 2019. The patient started seizures since August 2018, the
patient walks back and forth holding objects around the patient, scribbling books
while writing homework, flipping pages of books while reading or reciting , didn't
answer when summoned, with an unfocused gaze. Seizures last for ± 3-5 seconds,
frequency ± 5-10 times / day, seizures stop on their own, the patient is conscious
after the seizure This is consistent with the literature where absence type epilepsy
is present as short and frequent episodes of profound impairment of consciousness
without loss of body tone, lasting about 10 seconds. They are abrupt in onset and
termination and easily provoked by hyperventilation. These typically come to the
attention of parents or teachers after a prolonged period of observation, despite
their high frequency. TAS may occur tens of times daily, but their subtle clinical
symptoms allow them to go unnoticed or misdiagnosed as inattention.
Approximately half of all recorded seizures begin with an arrest in activity, which

19
is typically complete. Less common first signs include eyelid movements, eye
opening, and oral automatisms. The majority of electrographic seizures
(approximately 80 percent) are associated with at least one clinical sign. The three
most common seizure features are pause / stare (80 to 95 percent), motor
automatisms (40 to 60 percent), and eye involvement (55 percent). About one-
third of seizures involve three hertz (Hz) regular eyelid movements. Motor
automatisms are predominantly oral 3 . 
The patient was tested for laboratory and EEG. For laboratory testing,
Complete blood count (CBC): Haemoglobin 12 gr/dl, white blood cell
6.810/mm3, differential count 0/11/4/33/49/3, hematocrite 37%, platelets
259.000/mm3, red blood cell 4,49x106/mm3, reticulocyte 1,1%, sodium 140
mmol/l, potassium 4,1 mmol/l, calcium 8,2 mg/dl, SGOT 19, SGPT 10
Interpretation: laboratory result in normal range. Then from the EEG examination,
abnormal results were obtained in the form of waves 2-3 Hz Spike and Wave
Complex, overall, at the time of hyperventilation. When recording in a sleep state,
slow eye movement is obtained. There are no abnormalities. Impression:
Abnormal EEG with 2-3 Hz Spike Wave Complex Suggestive to the diagnosis of
Absence Seizure Epilepsy.
The recommendation treatment for absence epileptic seizure is
Ethosuximide. Ethosuximide is recommended as first-line therapy in most
children with CAE. Another treatment recommendation are valproic acid and
lamotrigine. After 16 weeks of treatment, ethosuximide and valproate were
significantly more effective than lamotrigine (16-week freedom from seizure rates
of 53, 58, and 29 percent, respectively), and the rate of drug discontinuation for
adverse effects was similar among the three groups. However, valproate was
associated with more frequent attentional dysfunction than ethosuximide (49
versus 33 percent). In this case report, the patient received therapy Folic Acid 1 x
1 mg, Vitamin B6 1 x 10 mg and Depakene 2x4cc. Inadequate treatment with
inadequate doses is the cause of recurrent seizures in patients. Eethosuximide or
lamotrigine can be added to prevent recurrence of seizures. The typical starting
dose of ethosuximide is 5 to 10 mg / kg / day in two divided doses for children
younger than six years, and 250 mg twice daily for children ages six years and

20
older. The usual maintenance dose is 15 to 40 mg / kg / day in divided doses.
Additional options for refractory CAE include topiramate, benzodiazepines,
acetazolamide, ketogenic dietary therapy, and vagus nerve stimulation. For all of
these options, trials that formally evaluate their effectiveness and side effect
profile are lacking; however, observational data suggest benefit5.
Vagus nerve stimulation has been shown to reduce seizure frequency in
six patients with CAE and refractory absences. There are mixed reports from
small studies with regard to levetiracetam in CAE, with one study suggesting
efficacy, another reporting aggravation of absences in certain patients, and a third
study of 72 children showing apparent benefit in 25 percent but a high rate of
discontinuation (74 percent) due to poor seizure control and/or side effects19.
Several antiseizure drugs have the potential to aggravate absence seizures
in patients with CAE and should be avoided. These include carbamazepine,
vigabatrin, gabapentin, and tiagabine. Phenytoin and phenobarbital are known for
their ineffectiveness in treating absences and should also be avoided 5. At the time
of diagnosis of CAE, it is not possible to predict eventual remission for most
children. Remission is very unlikely for a small group with a history of absence
status or slowing of the EEG background in association with a positive family
history of generalized seizures in first degree relatives. If a child develops GTC or
myoclonic seizures during treatment with AEDs, remission from CAE is
infrequent and development of Juvenille Myoclonic Epileptic (JME) is very
likely. While 65% of children with CAE eventually have remission of their
epilepsy, nearly half of those without remission will develop JME3.

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 EVIDENCE BASED MEDICINE

A. Clinical Question
What are the long term outcome of absence epileptic seizure?

B. Component of foreground question (PICO)

Population/problem : Absence epileptic seizure
Intervention : None
Comparator : None
Outcome : Approximately two-thirds of patients were seizure
free at a minimum follow up of nine years after seizure onset in one study.
Seventeen percent were still having seizures but did not take medications.
Forty-four percent of patients not in remission had progressed to JME.
Significant factors predicting no remission included cognitive difficulties at
onset, absence status epilepticus before or during drug treatment, generalized
tonic clonic or myoclonic seizures after treatment onset, abnormal
background on initial EEG, and a history of generalized seizures in first-
degree relatives.
In another study, over 90 percent of children with
CAE diagnosed according to the 1989 ILAE classification were seizure free
after a mean follow-up of 15 years. Those who fulfilled the 2005 criteria had
fewer generalized tonic clonic seizures, but their final overall outcome was
not significantly different than those who did not. The total duration of
epilepsy and the mean age at final remission were 3.9 and 9.5 years,
respectively. Epilepsy duration was longer in those children who had become
seizure-free more than six months after treatment initiation.
A relatively small proportion of children with typical
absence seizures are refractory to typical therapeutic approaches for a
prolonged period. In one study of 92 such children with refractory typical
absence seizures, approximately 50 percent eventually achieved prolonged
seizure freedom with or without antiseizure drugs.

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C. Searching Methode
Investigation was done based on keywords “epilepsy, absence seizure”. By
using limitation of the study in human and establish within the last 15 years.
Founded an article answered the clinical question with title: Childhood
absence epilepsy on Up to Date Journal (2021). Authors: Korff CM, Nordli
DR and Dashe JF.

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 REFFERENCE

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2021 [cited 2021 Feb 2]. p. 1–23. Available from:
https://www.uptodate.com/contents/childhood-absence-epilepsy

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