Case Report

Diajukan tanggal :
Januari 2021
CASE REPORT
DRUG RESISTANCE EPILEPSY (ATONIC EPILEPSY/DROP ATTACK)

WITH
CORPUS CALLOSOTOMY
Oleh:
Ika Rara Rosita
Pembimbing :
Dr. dr. Alifiani Hikmah P, Sp.A(K)
DEPARTMENT OF CHILD HEALTH

FACULTY OF MEDICINE DIPONEGORO UNIVERSITY/
DR. KARIADI HOSPITAL SEMARANG
2020
TABLE OF CONTENTS
TABLE OF CONTENTS................................................................................................1
FIGURE LIST.................................................................................................................3
TABLE LIST..................................................................................................................3
ABBREVIATIONS........................................................................................................4
CHAPTER I. INTRODUCTION....................................................................................6
CHAPTER II. CASE.......................................................................................................7
IDENTITY..................................................................................................................7
HISTORY...................................................................................................................7
PHYSICAL EXAMINATION..................................................................................12
INVESTIGATION RESULTS.................................................................................17
PROBLEMS LIST....................................................................................................20
WORKING DIAGNOSIS.........................................................................................21
MANAGEMENT......................................................................................................21
PROGNOSIS............................................................................................................23
FOLLOW-UP............................................................................................................23
SCHEME OF ANALYSIS........................................................................................26
CHAPTER III. DISCUSSION......................................................................................27
HIV INFECTIONS...................................................................................................27
TUBERCULOSIS INFECTION IN HIV CHILDREN............................................37
OSTEOARTICULAR TUBERCULOSIS................................................................41
LYMPHADENOPATY............................................................................................44
ANEMIA...................................................................................................................47
HEPATITIS C VIRUS INFECTION........................................................................49
SEVERE MALNUTRITION....................................................................................53
GLOBAL DEVELOPMENTAL DELAY................................................................57
HYPERCALCEMIA.................................................................................................58
CHAPTER IV. SUMMARY.........................................................................................62
1
REFERENCES..............................................................................................................63
2
FIGURE LIST
Figure 1. Pedigree........................................................................................................10
Figure 2. Clinical appearance.......................................................................................15
Figure 3. Chest x-ray....................................................................................................19
Figure 4. AP-Lateral Femur X-ray and Left Cruris (March 29th , 2017).....................19
Figure 5. Relative levels of HIV RNA (red) and CD4 (blue) in adults (broken lines)
and in children (unbroken lines) after being infected with the HIV-1 virus.................30
Figure 6. HIV Cycle and the Mechanism of Action for Different Types of ARVs.....36
Figure 7. Conceptual framework of maternal and child undernutrition.......................54
Figure 8. Hypercalcemia diagnostic algorithm............................................................60
TABLE LIST
Table 1. WHO clinical staging of HIV/AIDS for children with HIV infection...........31
Table 2. Initiation of co-trimoxazole prophylaxis in infants and children...................34
Table 3. Recommendations for initiating ART in infants and children.......................35
Table 4. Recommendations for initiating ART in HIV-infected infants and children
according to clinical stage and immunological markers...............................................35
Table 5. First-line guidelines if children get TB therapy with rifampicin....................36
Table 6. Tuli Classification; The Natural History of Tuberculous Arthritis Progresses
Through 5 Stages..........................................................................................................42
Table 7. Differential diagnosis of generalized lymphadenopathy................................44
3
ABBREVIATIONS
3TC Lamivudine
AFB acid fast bacilli
AIDS acquired immunodeficiency syndrome
ANC antenatal care
APTT activated partial thromboplastin time
ART anti-retroviral therapy
ARV Antiretroviral
AZT Zidovudine
CD cluster of differentiation
CLHIV children living with HIV AIDS
CMV Cytomegalovirus
d4T Stavudine
EBV Epstein-Barr virus
ED emergency department
EFV Efavirenz
FDC fixed dose combination
FTC Emtricitabine
GGT gamma glutamyl transpeptidase
Hb Hemoglobin
HCV hepatitis C virus
HIV human immunodeficiency virus
INH Isoniazid
IRIS immune reconstitution inflammatory syndrome
JRA juvenile rheumatoid arthritis
LDL low density lipoprotein
LPA line probe assay
MCH mean corpuscular hemoglobin
MCHC mean corpuscular hemoglobin concentration
MCV mean corpuscular volume
MTCT mother to child transmission
4
NNRTI non-nucleoside reverse transcriptase inhibitor
NRTI nucleoside reverse transcriptase inhibitor
NS non-structural
NVP Nevirapine
PEG-IFN pegylated interferon
PPT plasma prothrombin time
PRC packed red cell
PTHRP parathyroid hormone related protein
PUR paradoxical upgrading reaction
PZA Pyrazinamide
RDW red cell distribution width
SGOT serum glutamic oxaloacetic transaminase
SGPT serum glutamic pyruvic transaminase
SLE systemic lupus erythematosus
TB Tuberculosis
TDF Tenofovir
TLR toll-like receptor
VCT voluntary counseling and testing
VLDL very low-density lipoprotein
WHO World Health Organization
5
CHAPTER I
INTRODUCTION
Epilepsy is the most prevalent serious neurologic condition, affecting approximately 70

million people worldwide. Epilepsy is associated with increased risk for morbidity and
mortality and can severely affect the quality of life of patients. Although antiepileptic drug
(AED) therapy is effective in stopping seizures in most cases of epilepsy, seizure control is not
achieved for some epilepsy patients. Studies have estimated that 60%‐70% of patients with
epilepsy respond, whereas those of 30%‐40% are resistant to the treatment using AEDs. (NO 3
KALILANI2018)
Drug‐Resistant Epilepsy (DRE) is defined as failure of adequate trials of 2 tolerated,
appropriately chosen, and used AED schedules (whether as monotherapies or in combination)
to achieve sustained seizure freedom (all types of seizures) for 12 months, or 3 times of the
inter-seizure interval before treatment starts. Patients with drug‐resistant epilepsy (DRE) have
considerable socioeconomic and psychological constraints that decrease their quality of life and
increase their risk of mortality. (NO 3 KALILANI2018)
In Egypt in 2013, based on door‐to‐door screening among the residents (≥6 months of
age), the point prevalence of DRE was 0.08 per 100. This was slightly higher than the
estimated prevalence in Singapore (0.07 per 100) and Finland (0.07 per 100). In France, the
prevalence was 0.14 per 100. The prevalence of DRE was based on epilepsy patients. In a
Canadian study of 196 children (age range 2‐17 years), the pooled incidence proportion
was 15% in children (<17 years) and 34% in adults. (NO 3 KALILANI2018)
Corpus callosotomy is a palliative treatment for patients with either generalized or
multifocal refractory epilepsy characterized by injurious drop attacks. Typically, a worthwhile
improvement will be seen in 65– 85% of patients following a total corpus callosotomy with the
greatest effect being on drop attacks. However, there is very few literatures on long-term
outcomes. From the literatures, it was found that 77% of patients had at least 50% reduction in
seizures following surgery, whereas 54% of patients had at least 75% reduction in seizures.
Generally, complete corpus callosotomy resulted in a greater reduction than partial corpus
callosotomy but was associated with an increased risk of permanent disconnection syndrome
compared with partial corpus callosotomy. (CORPUS CALLOSOTOMI)
This case reports a 12-year-old girl diagnosed with drug-resistant epilepsy who
underwent a surgical procedure of epilepsy. The aim of this case development was to analyze
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the treatment in cases of drug resistant epilepsy and to increase the knowledge on the surgical
outcome of epilepsy in drug resistant epilepsy cases. It is hoped that deepening the case of
drug-resistant epilepsy can be the basis for making a proper diagnosis and can provide the
earliest possible management to achieve optimal output in children with drug-resistant
epilepsy.
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CHAPTER II
CASE PRESENTATION
IDENTITY
The patient in this case is a girl with the following identities:
Name : NK
Age : 12 years old
MR/Reg number : C660204
Date of birth : May 22th, 2011
Sex : Female
Date of admission : October 3th,2019
PARENT IDENTITY
Father’s name : Mr. K Mother’s name : Mrs. NC
Age : 41 y.o Age : 34 y.o
Occupation : Farmer Occupation : Housewife
Education : Junior High School Education : Junior High School
HISTORY
Allonamnesis was performed on the 2nd day of admission (October 4th 2019, 10.00 IWST) at
pediatric ward of Dr Kariadi General Hospital with the mother. The data was obtained from parents
and medical record.
Chief complaint: Seizure

Present History
Seven days before admission, the patient had seizures like she would faint and weakness. She
was about to fall with the frequency at least 7-20 times per day and the duration of seizure of less than
5 seconds. She was unconscious during the seizure. Before and after the seizure she was conscious.
The patient tended to sleep more often than usual. The patient did not have a fever, had pain in the
gums, did not cough, did not have shortness of breath, no vomitus, and defecated and urinated within
normal limits. She was diagnosed with atonic epilepsy. She was treated with Valproic acid 5,6 ml/8
8
hours, Topamax 75 mg – 0 – 75 mg, Lamictal 30 mg/8 hours, Keppra 250 mg 1-0-1, and Vit B6 10
mg/8 hours.
In the physical examination in the C1L1 ward at October 4th, 2019, the general state of the
patient was conscious, looked weak and less active, the body weight was 17 kg and the height was 128
cm, both z-score HAZ and BMI was below the -3 percentile. The vital signs were within normal limit.
The eyes did not appear anemic with positive light reflexes in both eyes and isochoric pupils Ø
3mm/3mm. Mouth examination revealed dry mucous lips and gingival hypertrophy. Cardiac, lungs,
and abdomen were within normal limits. Neurological examination revealed normal positive
physiological reflexes, left limbs tone was positive decreased and negative for both pathological
reflexes and clones.
On the laboratory findings, initial complete blood count showed platelets concentration of
71.000/uL with thrombocytopenia. Blood glucose was 71 mg/dl (80-160), electrolytes : Sodium 134
mmol/L (136-145), Potassium 2,5 mmol/L (3,5 – 5,1), Chlorida 101 mmol/L (98-107), Calcium 2,17
mmol/L (2,12 – 2,52) with impression of hypoglycemia, hyponatremia, and hypocalcemia. Liver
function tests revealed AST 28 U/L (15-34) and ALT 10 U/L (15-60) were within normal limit.
Past medical history

The first seizures were at the age of 7 months old, and the seizure form was like stiffness and
she was about to fall, eyes glancing upward, seizures were 10-20 times per day, duration of seizure
was 3-5 minutes. Before and after the seizures, she was conscious, and she was unconscious during the
seizure. The patient was never examined before but taken to alternative medicine. She was taken to
Purwodadi Hospital at 8 years old, diagnosed with epilepsy, and administered Valproic Acid. She
routinely had the controls at the Kariadi Hospital since the past 1.5 years. The patient had performed
EEG and Head MRI. She had been treated at the ICU of RST Demak in January 2019 for 9 days.
Then. because there was no improvement, she was referred to Kariadi Hospital with
thrombocytopenia (10,000) dd / ITP
9
Family medical history
Family history with seizures or epilepsy were denied.
Gambar 1. Pedigree
Socio-Economic History
Father and mother work as farmers. Monthly household income was IDR 2,000,000, supporting
one child who was not independent yet. The medical and hospital expenses were covered by BPJS
PBI.
Social-economic impression : lacking.
Perinatal History
The patient was born from a G2P1A0 mother of 25 years old, aterm pregnancy, antenatal care
more than 4 times in the public health center. There was no antenatal bleeding, neither diseases during
pregnancy such as pre-eclampsia, diabetes mellitus nor hypertension. There was no history of
consumption of drugs and herbs out of a doctor's prescription. The patient was born spontaneously by
midwife, she cried immediately after birth, birth weight was 3500 grams, neither a history of jaundice
nor cyanotic reported.
Nutritional history
Natural history revealed that the patient was exclusively breastfed until 6 months. She was
breastfed until 2 years old. Complementary feeding was carried out since the patient was 6 months old,
started with milk porridge, fine porridge, and coarse porridge. Family food was given from the age of 1
year in the form of rice with chicken, tempeh, tofu, vegetables, or eggs. Since the age of 2 years to
present, the patient was given formula milk with SGM 90 - 110 mL and 3 x ½ servings of rice with
chicken, tempeh, tofu, and vegetables. But after the child had seizure, her appetite decreased.
Impression: insufficient quantity and quality, exclusive breastfeeding (+)
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Growth history
 Birth weight : 3500 g
 Current bodyweight : 17 kg
 Current body length : 128 cm
 Ideal bodyweight : 25 kg
 Head circumference : 54 cm
 WAZ : -3,69
 BMI : -4,72
 HAZ : -1,09
 Growth impression : Severe acute malnutrition
Developmental history
The patient was able to walk but unable to run at the age of 10 years old. She was able to babble, but
she could not say the words yet. She was not able to drink and eat herself with a spoon, could not wear
and undress herself.
Impression: patients experienced delays in the fine motor, gross motor sectors, and verbal.
Immunization history
 Polio : 0, 2, 3, 4, 18 months
 BCG : 1 month
 Hep B : 0, 2, 3, 4, 18 months
 DPT : 2, 3, 4, 18 months
 HiB : 2, 3, 4, 18 months
 Measles : 9, 18 months
Impression: complete basic immunization, booster (+)
PHYSICAL EXAMINATION
The patient was examined on Friday, March 4th, 2019 in pediatric ward.
General appearance: alert, weak, very thin, attached nasogastric tube.
Vital signs
 Blood pressure : 100/60 mmHg (P50)
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 Pulse : 114 times / minutes, regular, adequate
 Breath : 22 times / minutes
 Temperature : 37,1 degrees Celsius (armpit)
General examination
Head : fontanel was closed, head circumference 54 cm (mesocephaly)
Eyes : the conjunctiva was not anemic, sclerae was not icteric, isochoric pupil  3 / 3
mm
Nose : No nasal flaring, no nasal discharge
Ears : no discharge
Mouth : no oral thrush, no ulcer
Tongue : no discoloration
Gingival : no bleeding
Teeth : no caries
Throat : tonsils T1-1, no crypt enlargement, no hyperemic
Chest : symmetric, no retraction, protruding ribs
Lung
Inspection : symmetric
Palpation : right and left fremitus symmetric
Percussion : resonant at all lung fields
Auscultation : base sound: vesicular at all lung fields
Additional sound: not heard any additional sound
Heart
Inspection : apex beat not seen
Palpation : apex beat palpable at fourth intercostal space at left medial clavicular line
Percussion : left border: as apex beat location; upper border: second intercostal space at left
parasternal line; right border: second intercostal space at right parasternal line
Auscultation : normal heart sound, no thrill or gallop
Abdomen
Inspection : convex, no visible distended vein
Auscultation : normal bowel peristaltic movement
Percussion : tympanic, no shifting dullness
Palpation : supple, no tenderness
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Liver : hepatomegaly, liver tip palpated at 3 cm below costal arch, tapper, flat surface
Spleen : not palpable
Extremities : superior inferior
Cyanosis -/- -/-
Pale -/- -/-
Cold -/- -/-
Capillary refill time <2” <2”
Tone normal normal
Clonus -/-
Physiologic reflex +N/+N +N/ +N
Pathologic reflex -/- -/-
Tone decrease/decrease decrease/decrease
Muscle strength >3/>3 >3/>3
Cranial nerve examinations

NI : sensory  not examined
N II : sensory  pupillary reflex (+) at both eyes
N III-IV-VI : no strabismus
NV : corneal reflex (+)
N VII : symmetric nasolabial fold
N VIII : there was response toward sound
N IX-X : no swallowing difficulties
N XI : can turn to right and left
N XII : no tongue deviation
INVESTIGATION RESULTS
Hematology results
Examination Units Reference 1/10/2019 9/10/20 11/10/20
value
Hemoglobin g/dL 10.5 – 15 12,3 7,1 9,9
Hematocrit % 36 – 44 37,3 21,8 29,8
Erythrocyte 10^6/μL 3 – 5.4 3.7 2,8 3,4
MCH Pg 23.00 – 31.00 25.5 24,6 24,9
13
MCV fL 77 – 101 83.2 78 80
MCHC g/dL 29 – 36 30.6 30 30,2
Leukocyte 10^3/μL 5 – 14.5 12,8 23,8 17,4
Platelets 10^3/μL 150 – 400 224 137 146
RDW % 11.6 – 14.8 13.2 11,8 12,8
MPV fL 4 – 11 9.2 7,8 8,6
Clinical chemistry results

Examination Units Reference value 1/10/20 5/10/20 7/10/20 9/10/20
Blood glucose mg/dL 80 - 160 105
Sodium mmol/L 135 - 145 136 142 135
Potassium mmol/L 3.5 – 5.1 2,9 3,4 4,1
Chloride mmol/L 98 - 107 104 103 108
Calcium mmol/L 2.12 – 2.52 2.4 2,29 2,7
Ureum mg/dL 15-39 32
Creatinin mg/dL 0,6-1,3 0,4
From the electroencephalography (EEG) examination on February 6th, 2019, the impression
of epileptic waves in the left dominant bifrontal region and left front of temporal region and diffuse
electrophysiological disorder were obtained.
Figure 2. Electroencephalography
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On radiology findings, in the head MRI with contrast examination on April 16th, 2018, there
was an impression of minimal cortical and sulcy widening on the left and right temporal and parietal
regions suspect of mild atrophy. In addition, the right and left hippocampus does not appear
atrophy, there is no brain malformation, and there is no visible bleeding or intracranial SOL.
Figure 3. Head MRI with contrast
From all these findings, the diagnosis of Drug epilepsy (atonic epilepsy), CP flaccid, drug
induced thrombocytopenia, electrolytes imbalances (hyponatremia and hypokalemia), gingival
hypertrophy, and Severe acute malnutrition could be made. The patient was given infusion therapy
D10% 480/20 ml / hour + NaCl 3% (2meq) 69 ml + KCl otsu (3meq) 53 cc in a D10% 500cc
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solution, per oral drugs Valproic acid 5.6 ml / 8 hours (40mg/kg/day), Topamax 75 mg-0-75 mg
(7,5mg/kg/day), Lamictal 30 mg / 8 hours (5 mg/kg/day), Keppra 500 mg 1-0-1 (58 mg/kg/day),
clobazam 10mg/12 hours, and Vitamin B6 10 mg / 8 hours.
Non-medical therapy
1. Thermoregulation
2. Pediatric nutrition care
24-hours 50 1 1
requirement
Fluids Calories Proteins Fats
(mL) (kcal) (gram) (gram)
1540 1250 17 17
D5 ½ NS 480 81,6 - -
Pediasure 1200 1200 34,8 52,8
8x150cc
Total 1680 1281 34,8 52,8
%AKG 109% 102,5% 204% 310%
Ideal bodyweight = 25 kilograms. Route of administration: NGT
Care plan
1. Wound care
2. Hand hygiene
Monitoring plan
1. Monitoring of patient’s symptoms
2. Monitoring of general status and vital signs
3. Monitoring of seizure
4. Monitoring of nutrition intake
5. Monitoring Therapy response
6. Monitoring side effect of therapy
Consultation plan
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Consultation and integrated care with neurology, nutrition and metabolic disease, and neurosurgeon
division or departments
Communications, information, and educations plan

1. Wound care
2. Nutritional care
3. Care of plan
4. Prognosis
PROGNOSIS
1. Ad vitam : dubia ad bonam
2. Ad functionam : dubia ad malam
3. Ad sanam : dubia ad malam
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FOLLOW-UP
Date Clinical Condition Assesment Therapy/ Intervention Program
1-2 Neurology Follow up Day :1-2 BW : 20 A: P:
/10/2019 kg - Intractable eplilepsy - IVFD D5 ½ NS 480/20/7 dpm
00.35 S: seizure (-) last seizure was on ER, fever (atonic) - PO:
(-), defecating and urinating within normal - CP flaccid o Valproic acid 4 ml/8 hours (30 mg/kgBB/
limit - Hypokalemia (2,9) day)
O: General condition: awake o Topamax 75 mg – 0 – 75 mg (7,5 mg/kg/
Vital signs: day)
HR: 100 x/ minute o Lamictal 30 mg/8 hours (4,5 mg/kg/hari)
RR: 24 x/ minute o Keppra 250 mg 2-0-1 (37,5 mg/kg/day)
t: 36,6 C
o
o Vit B6 10 mg/8 hours
SpO2 : 99%
N: regular, adequate pulse
Program :
- Consulting to dr. Aris, Sp.S (K)
Physical examination :
- Pro EEG long term
Eye : anemic (-/-), icteric (-/-), light reflex
(+/+), isochoric pupil 3mm/3mm
Nose : nasal flaring (-)
Mouth : cyanosis (-), dry mucous (+),
gingiva hypertrophy (+)
Neck : lymph nodes enlargement (-), neck
stiffness (-)
18
Thorax : symmetric, retraction (-)
Cor : normal Heart sound I-II, murmur (-),
gallop (-)
Pulmo : Vesicular (+/+) (+/+)
Rales (-/-) (-/-)
Ronchi (-/-) (-/-)
Wheezing (-/-) (-/-)
Abdomen : flat, abdominal sound (+)N,
liver/spleen not palpated
Extremities : Cold acral (-/-) (-/-)
Cyanosis (-/-)
Laboratory (01/10/2019):
Hb : 12,3
Ht : 37,3
L : 12.800
Tr : 224.000
Blood glucose : 105
Na/K/Cl/Ca : 136/2,9/104/2,4
Head MRI with contrast (16/4/2018) :

- Suspect of mild atrophy
- The right and left hippocampus
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does not appear atrophy
- There is no brain malformation
- There is no bleeding or intracranial
SOL
EEG 6/12/2019 :
- Epileptic waves in the left dominant
bifrontal region and left
frontotemporal region
- Diffuse electrophysiology disorder
3-4 Neurology Follow up Day:3-4 BW : 20 A: P:
/10/2019 kg - Intractable eplilepsy - IVFD KaEN 3B 480/20/7 dpm
07.00 S: seizures (+) 7x/24 hours,atonic, duration (atonic) - PO:
± 1 seconds - CP flaccid o KSR 1 tab/24 hours
O: General conditio n: awake - Hypokalemia (2,9) o Other therapies are continued
Vital signs: Program :
HR: 100 x/ minute - On EEG long term
RR: 24 x/ minute - Consulting to Prof Zaenal, Sp.BS
t: 36,5 Co
- Checking electrolytes and Ca evaluation
SpO2 : 96% 5/10/2019
N: regular, adequate pulse - Consulting to Psychologist
- Performing Quality of life test of pre and
post surgery
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5/10/2019 Neurology Follow up Day:5 BW : 20 kg A: P:
06.00 S: seizure (-), fever (-), vomit (-) - Intractable eplilepsy - IVFD KaEN 3B 480/20/7 dpm
O: General condition: awake (atonic) - Other therapies are continued
Vital signs: - CP flaccid
HR: 104 x/ minute - Hypokalemia (2,9) Program :
RR: 24 x/ minute - Waiting for the result of EEG long term
t: 36,7oC - Waiting for the results of electrolytes
SpO2 : 96% and Ca
N: regular, adequate pulse - Waiting for the results from Psychology
Answer from Neurosurgery division : - Performing Quality of life test pre and
A : Drop attack post surgery
P : Wait for the result of EEG, Patient will
be followed up
6-8 Neurology Follow up Day :6-8 A: P:
/10/2019 S: seizures (+) 6x/24 hours, duration <10 - Intractable eplilepsy - IVFD KaEN 3B 480/20/7 dpm
06.00 seconds, stop spontaneously, fever (-), (atonic) - PO:
vomit (-), want to eat and drink - CP flaccid o KSR stop
O: General condition: awake - Hypokalemia (3,4) o Other therapies are continued
Vital signs: improvement
HR: 102 x/ minute - Drop attack Program :
RR: 24 x/ minute - Waiting for the result of EEG long term,
t: 36,8oC results from Psychology
SpO2 : 98% - Performing Quality of life test of pre and
21
N: regular, adequate pulse post surgery
- Pro craniotomy corpus callostomy
Laboratory (05/10/2019): (Tuesday 8/10/2019)
Na/K/Cl/Ca : 142/3,4/103/2,29
9/10/2019 Neurology Follow up Day :9 A: P:
06.00 S: fever (+), seizure (-) - Intractable eplilepsy - IVFD D5 ½ NS 480/20 ml/hour
Post craniotomy corpus callostomy surgery (atonic) - Inj. Paracetamol 300 mg/8 hours (t≥ 38ºC/
O: General condition: awake - CP flaccid analgetic)
Vital signs: - Hypokalemia (3,4) - PO:
HR: 118 x/ minute improvement o Other therapies are continued
RR: 24 x/ minute - Drop attack
t: 37oC - Post corpus callosotomy
SpO2 : 98% surgery (D1) Program :
N: regular, adequate pulse - Waiting for the result of EEG long term
Physical examination : - PedsQL post surgery
Eye : anemic (-/-), icteric (-/-), light reflex - Checking routine blood, electrolytes, Ca
(+/+), isochoric pupil 2mm/2mm
Nose : nasal flaring (-)
Mouth : cyanosis (-)
Neck : lymph nodes enlargement (-)
Thorax : symmetric, retraction (-)
Cor : normal Heart sound I-II, murmur (-),
gallop (-)
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Pulmo : Vesicular (+/+) (+/+)
Rales (-/-) (-/-)
Ronchi (-/-) (-/-)
Wheezing (-/-) (-/-)
Abdomen : flat, abdominal sound (+)N,
liver/spleen not palpated
Extremities : Cold acral (-/-) (-/-)
Cyanosis (-/-)
Physiological reflex (+/+)
(+/+)
Pathological reflex (-/-) (-/-)
Tone N/N N/N
Clonus (-/-)
/10/2019 S: intermittent fever (+), seizures (+) 7x/24 - Intractable eplilepsy - IVFD D5 ½ NS 480/20 ml/hour
06.00 hours, vomit (-), the patient refuses to eat, (atonic) - Inj. Paracetamol 300 mg/8 hours (t≥ 38ºC/
still want to drink 15 ml of milk - CP flaccid analgetic)
Post craniotomy corpus callostomy surgery - Drop attackPost corpus - PO: Other therapies are continued
(8/10/2019) callosotomy surgery (D2) Program :
O: General condition: awake - Waiting for the result of EEG long term
Vital signs: - PedsQL post surgery
HR: 120 x/ minute - NGT insertion
RR: 24 x/ minute - 150cc, 150cc PRC Transfusion
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t: 37,1oC - Consulting to Nutrition Division
SpO2 : 98% - Inj. Ceftriaxone 100 mg/kgBB/day ~ 2
N: regular, adequate pulse g/24 hours
Laboratory (9/10/2019): - Performing blood culture
Hb : 7,1
Ht : 21,8
L : 23.800
Tr : 137.000
Na/K/Cl/Ca : 135/4,1/108/2,7
/10/2019 S: fever (-), seizures (+) 4x/24 hours, - Intractable eplilepsy - IVFD D5 ½ NS 480/20 ml/hour
06.00 atonic, watery stool (+) 14x/24 hours, pulp (atonic) - Inj. Ceftriaxone 2 g/24 hours (D2)
(+), yellow coloured - CP flaccid - Inj. Paracetamol 300 mg/8 hours (t≥ 38ºC/
O: General condition: awake - Drop attack analgetic)
Vital signs: - Post corpus callostomy - PO:
HR: 110 x/ minute surgery (D4) Zinc 20 mg/24 hours
RR: 22 x/ minute - Acute diarrhea without Oralit 100 cc/diarrhea
t: 36,7oC signs of dehydration
SpO2 : 98% Program :
N: regular, adequate pulse - Waiting for the result of EEG long term
BC : -70 ml - PedsQL post surgery
D : hard to evaluate - Waiting for the result of blood culture
Laboratory (11/10/2019): (10/10/2019)
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Hb : 9,9 - Diet : LLM 8x175 ml
Ht : 29,8
L : 17.400
Tr : 146.000
Routine feces (11/10/2019) :
Yellow coloured, soft consistency, worms
(-), entamoeba (-), fats (+), carbohydrates
(+), proteins (+), erithrocytes (-),
leukocytes 0-1/field of view, epithels
0-1/field of view, bacteria (+) 2, yeast (+)
15/10/201 Follow up Neurology Day :15 A: P:
9 S: fever (-), seizure (+) 1x/24 hours, atonic, - Intractable eplilepsy - IVFD D5 ½ NS 720/30 ml/hour
05.00 watery stool (-) (atonic) - Inj. Paracetamol 300 mg/8 hours (t≥ 38ºC/
O: General condition: awake - CP flaccid analgetic)
Vital signs: - Drop attack - Inj. Ceftriaxone 2 g/24 hours (D2)
HR: 110 x/ minute - Postcorpus callosotomy - Other therapies are continued
RR: 22 x/ minute surgery D7 Program :
t: 36,9oC - Acute diarrhea without - Waiting for the result of EEG long term
SpO2 : 97% signs of dehydration - PedsQL post surgery
N: regular, adequate pulse (improvement) - Waiting for the result of blood culture
(10/10/2019)
- Diet : LLM 8x175 ml
25
SCHEME OF ANALYSIS
A 12 y.o girl
The first seizures at the age of
7 months old. The patient was Prediktor Keluaran bedah
never examined before, but epilepsy :
Drug Resistant Epilepsy Medical Therapy:
taken to alternative (atonic) AED (LoE 1)
medicine.The patient had Prognosis baik:
seizures like she would faint
and weakness. She was about
Non-Medical Therapy: Prognosis buruk:
to fall, frequency at least 7-20 Corpus callosotomy
times per day, the duration of
seizure was less than 5
seconds.
Tone : decrease CP Flaccid

Optimal
EEG : epileptic waves in the Growth
left dominant bifrontal region Prognosis &
and left frontotemporal region Ad vitam: dubia ad malam Development
and diffuse electrophysiological Ad fuctionam: dubia ad
disorder malam
Ad sanationam: ad malam
Head MRI with contrast:
minimal cortical and sulcy
widening in the left and right Monitoring:
temporal and parietal regions AED Therapy (side effect
suspect of mild atrophy and medication adherence)
Seizure frequency
The patient received peroral drugs drug induced Medical Therapy : Quality of life and
Valproic acid 5,6ml/8jam (maximal thrombocytopenia Adjusting the dose of AED nutritional status
dosetherapy) and Platelete
137.000
Paediatric nutrition care
WAZ -2,97, HAZ -0,12, BMI - 4,55 Severe Acute malnutrition Vitamin supplementations
Risk factors & findings Diagnosis & complication Management Prognosis
Stimulating – Loving – Caring – Integrated Psychosocial Support
26
CHAPTER III
LITERATURE REVIEW
3.1 Epilepsy
3.1.1 Definition
An epileptic seizure is a transient occurrence of signs and/or
symptoms due to abnormal excessive or synchronous neuronal activity in
the brain. Epilepsy is a brain disorder characterized by an enduring
predisposition to generate epileptic seizures, and by the neurobiologic,
cognitive, psychological, and social consequences of this condition. The
definition of epilepsy requires the occurrence of at least one epileptic
seizure. Epilepsy is a brain disease defined by any of the following
conditions:
1. At least two unprovoked (or reflex) seizures occurring >24 h apart
2. One unprovoked (or reflex) seizure and a probability of further
seizures similar to the general recurrence risk (at least 60%) after two
unprovoked seizures, occurring over the next 10 years
3. Diagnosis of an epilepsy syndrome
Epilepsy is considered to be resolved for the individuals with an
age-dependent epilepsy syndrome but are now past the applicable age or
those who have remained seizure-free for the last 10 years, with no seizure
medicines for the last 5 years. (NO 6)
3.1.2 Incidence of Epilepsy

3.1.3 Epilepsy Classification (NO 7.1)
The new Classification of the Epilepsies is a multi-level classification,
designed to cater for classifying epilepsy in different clinical environments.
This is in acknowledgement of the wide variation in resources around the
world meaning that different levels of classification will be possible
depending on the resources available to the clinician making the diagnosis.
Where possible, a diagnosis at all three levels should be sought as well as the
etiology of the individual’s epilepsy.
27
Figure. Classification of the Epilepsies
From the moment that the patient presents with a first epileptic
seizure, the clinician should be aiming to determine the etiology of the
patient’s epilepsy. A range of etiologic groups has been recognized, with
emphasis on those that have implications for treatment. the first investigation
carried out often involves neuroimaging, ideally MRI where available. This
enables the clinician to decide if there is a structural etiology for the patient’s
epilepsy. The five additional etiologic groups are genetic, infectious,
metabolic, and immune, as well as an unknown group. A patient’s epilepsy
may be classified into more than one etiologic category; the etiologies are not
hierarchical, and the importance given to the patient’s etiological group may
depend on the circumstance.
. (DROP ATTACKS)
3.2 Drug Resistance Epilepsy (DRE)

There are many definitions of drug-resistant epilepsy. The
definition of drug-resistant epilepsy according to ILAE 2010 includes two
stages, the first stage is to determine the category of outcome from the
therapy given (seizure-free, failure of therapy or cannot be determined)
28
and the second stage is to determine the state of drug-resistant epilepsy
based on the information on anti-epileptic treatment provided so that it has
an impact to be a failure of therapy. The categorization of the treatment
outcome is determined generally from the control of the seizures and
whether or not there are unexpected effects on each of these assessments.
The assessment of control of seizures is determined based on the first
category, namely seizure-free. If not present, then the second category is
failure of therapy. If it does not meet the first or second category with the
provision of appropriate and adequate antiepileptic drugs, it will fall into
the third category which cannot be determined. Each of these categories
will be further divided into three options based on the incidence of
unexpected effects (Table 2) because there are clinical differences in the
occurrence of drug-resistant epilepsy with or without unexpected effects.
Table 2 Scheme for categorizing outcome of an intervention for epilepsy

Outcome dimension
Seizure control Occurrence of adverse Outcome category
effects
1. Seizure-free A. No 1A
B. Yes 1B
C. Undetermined 1C
2. Treatment failure A. No 2A
B. Yes 2B
C. Undetermined 2C
3. Undetermined A. No 3A
B. Yes 3B
C. Undetermined 3C
Administration of adequate therapy is a therapy with the appropriate

dose within a certain period of time. This does not include the condition that the
dose of the drug is decreased because of unexpected effects. If the patient is not
routinely treated, the category of seizure control and the incidence of adverse
29
effects are included in the criteria that cannot be determined (3C). There is a list
of questions to obtain information in determining the treatment outcome category
(Table 3).
Table 3. Minimum dataset required to determine whether the trial of a therapeutic

intervention is informative
No A list of questions
1 Nature of the intervention (e.g., type of drug, in the case of antiepileptic
drug treatment)
2 Mode of application (e.g., formulation, dose, dosing interval, and patient’s
compliance in case of an antiepileptic drug)
3. Duration of exposure
4. Occurrence of seizures and adverse effects during the trial period
5. Whether there was any effort to optimize dose
6. Reason(s) for discontinuation (if applicable)
- Unsatisfactory seizure control
- Adverse effects
- Long-term seizure freedom
- Psychosocial reasons, for example, planning for pregnancy
- Administrative reasons, for example, lost to follow up
- Financial issues, for example, cannot afford treatment
- Patient/caretaker preference
- Other reasons
The term seizure-free is a condition free from all types of seizures

including aura. Hanley and Lippman-Hand introduced the term "rule of three" to
estimate seizure-free incidence, which is said to be seizure-free if there is the
longest seizure-free period and it recurs at least three times after receiving new
therapy. For example, if there is a patient who has been seizure free for 6 months
during control after receiving new therapy, he can be said to be seizure-free if the
child is seizure free in 18 months. This is then developed as an indicator of a
positive response to epilepsy treatment or in other words, category 1 indicates a
30
response to epilepsy treatment. Based on the agreement from ILAE, the definition
of seizure-free is the absence of seizures at least 3 times of the distance between
the longest seizures before receiving therapy (if the seizures occurs less than 12
months) or seizure-free 12 months, or longer. Then, therapy failure is defined as
recurrence of seizures after adequate seizure therapy.
The second stage in determining drug-resistant epilepsy is determining
whether it fits the definition of drug-resistant epilepsy. The state of drug-resistant
epilepsy is defined as the failure of two times of adequate therapy, the right choice
and according to the schedule of anti-epileptic drugs (either monotherapy or
combination therapy) to achieve a seizure-free state. The ILAE consensus defines
it as a two-fold failure to avoid late evaluations and is a revision based on
researched data. After applying the classifications in table 1, drug-resistant
epilepsy is assigned to the results of treatment that fall into category 2 with at
least two anti-epileptic drug treatments (either monotherapy or combination)
without occurring in category 1. Determination of drug resistance epilepsy must
also be determined. Based on at least informative therapy according to the list of
questions in table 2, it ensures that it is not a result of treatment failure not due to
an undetermined cause. In other words, failure after two treatments based on
already informative and appropriate data.
There are various definitions of drug-resistant epilepsy. The ILAE
working group established and validated the definition so that it is repeatability,
consistency and practical (practicalability). The ILAE 2010 definition of drug-
resistant epilepsy has been validated using the Kappa test and a result of more
than 0.8 shows that the closeness and agreement is very strong. There are many
terms for drug-resistant epilepsy, including pharmacoresistence, intractable
epilepsy or refractory epilepsy. According to ILAE, these terms are easily
understood by all medical personnel, not only specialist doctors. Then, the term
drug-resistant epilepsy is agreed. The determination of drug-resistant epilepsy is
important to determine when invasive therapy such as surgery is indicated. The
prevalence of drug-resistant epilepsy was 28.4% -37% in the adult population in
Canada. A smaller number was reported in France at 15.6%. Prolonged and
31
repeated seizures had been reported to cause brain parenchymal damage and lower
the seizure threshold.
Etiology of Drug Resistant Epilepsy

A point mutation in the multidrug resistant-1 (MDR1) gene C3435T,
which functions as brain protection against drugs and harmful substances
suspected to be one of the causes. This gene mutation causes an increase in gene
expression so that OAE with the molecular structure which is not a harmful
substance cannot enter brain cells and return to blood vessels. Even though the
patient has been given high doses of therapy and high drug levels in the blood, the
patient still experiences epileptic seizures
3.3 Surgical Epilepsy

Epilepsy surgery is performed by complete resection or disconnection of
the epileptic zone (an important area in the cortex that causes seizures clinically)
by trying to maintain good areas (especially those with important functions) in the
cerebral cortex.
Epilepsy surgery was first performed by Victor Horsley in 1829 in
America. Three out of the five patients operated were seizure-free and two
patients experienced seizure improvement. The following are the types of epilepsy
surgery performed based on the location of the focus:
a. Limbus epileptic resection surgery
Patients with mesial temporal lesions get the best results after
epilepsy surgery. An indication for temporal surgery is a patient with a history
of typical seizures with onset in the medial temporal lobe and an aura before
the onset of the seizure. Surgical treatment for seizures is recommended for
temporal lobe epilepsy which is the most common type of epilepsy, and this
type is the most common "Stubborn" and most resistant to drugs. It has been
known from many studies that surgery provides seizure-free results in 65%,
and improvement in 21%, while those that do not improve are still found at
14%. The results of a study compared surgery with the optimal use of OAE in
temporal lobe epilepsy, and proved that surgery is much better (seizure-free
32
rate 64%) than optimal OAE therapy (only 8% seizure-free). The greater
benefit for temporal lobe epilepsy disorders and other partial epilepsy is caused
by localized neocortical lesions rather than OAE treatment. It is therefore
highly recommended to refer them to surgical centers for epilepsy
b. Neocortical resection
The results of the procedure are somewhat less satisfactory when
compared to temporal lobe surgery but are sufficiently considered in patients
with local extratemporal lesions defined by imaging techniques and in the
patients whose neurophysiological investigations revealed a consistent focal
onset for external temporal lobe seizures. Complex partial seizures arising from
the frontal-orbital region may be greatly assisted by surgery.
c. Hemispherectomy
This procedure may not be suitable for the patients with drug-resistant
epilepsy and in children with hemiplegic or in children with chronic
progressive focal encephalitis (Rasmussen's syndrome). Overall, 70% -80% of
patients become seizure free after this operation, and behavioral abnormalities
may also increase.
d. Corpus callosotomy
The corpus callosum and commissure sections of the hippocampus are
accepted palliative procedures for the occurrence of uncontrolled secondary
generalized seizures. The selection criteria for corpus callosotomy are poorly
defined than for any other surgical procedures. This operation is most often
applied to the children and adolescents with very severe epilepsy, with a
multifocal origin for seizures, or with a sudden onset of seizures resulting in
falls.
From 1999 to December 2018, there were a total of 671 epilepsy
surgeries for both children and adult patients carried out at Dr. Kariadi
Hospital, where the majority of patients came from Central Java. Surgery was
33
superior (could reduce seizures to 64%) than treatment (reduced seizures at
8%) in the cases of epilepsy with temporal lobe lesions.
A randomized controlled trial study in India first conducted in 2017
found that the benefits of epilepsy surgery in the first year could make 77% of
children seizure-free compared to the group receiving standard treatment therapy
(7% of children seizure-free).
Evaluation measures before epilepsy surgery have been shown to
improve control over seizure incidence, quality of life and social activity,
especially in patients with drug-resistant epilepsy. The pre-surgical evaluation is
briefly divided into three phases, in which the first phase is a non-invasive
examination, the second phase is an intracranial examination which is usually
invasive and the third phase is surgery. Most of the 80% of phase one will
progress to the third phase, only 20% go through the second phase. The ILAE
working group in 2014 issued guidelines for epilepsy surgery and the diagnosis of
epilepsy before surgery. The guideline has 7 provisions, consisting of:
1. Determining service centers with sufficient numbers of trained personnel
These personnel include epileptologists (neurologists and pediatric
neurologists), neurosurgeons, psychologists, radiologists or consultants in the
MRI department.
2. Using minimal techniques and equipment that have been standardized
Standardized equipment needed includes VEM (video-EEG monitoring),
MRI 1.5 Tesla and at least SPECT (single photon emission computed
tomography), PET (positron emission tomography), fMRI (functional MRI),
MEG (magnetoencephalography) and ESI. (EEG with source imaging).
3. Having trained personnel
Desired trained personnel are a neurologist or pediatric neurologist and
surgeon certified by the ILAE working group.
4. Monitoring during the procedure with video electroencephalography (EEG)
monitoring (VEM)
Intensive monitoring is needed because stopping anti-epileptic therapy
for 24 hours before surgery can increase the risk of an emergency during
surgery but on the other hand it also aims to see the semiology clearly.
34
5. There are clinical data and outcomes systematically arranged
The data before and after surgery and disease course one year after
should be in the same health service place so that it can be a comparison.
6. There are reports of findings and actions
Epilepsy surgery with appropriate standard is expected to be
implemented in the respective health centers. A minimum of 25 epilepsy
surgical procedures per year is expected to further improve and have sufficient
ability to perform epilepsy surgery.
7. Cooperation with epilepsy centers
The secretary of the ILAE working group opens the opportunity to hold
a case conference especially in dealing with complex cases.
Epilepsy surgery is considered successful in treating children with drug-

resistant epilepsy. The recommendations published since 2006 also differentiate
epilepsy surgery in children and adults. In children, there is still brain
development and plasticity. There are several tests to make a diagnosis in the
surgical evaluation of epilepsy, namely the examinations to find local
epileptogenic regions and critical areas in the cortex. The examinations to look for
epileptogenic regions include interictal EEG, MRI, 3D EEG, PET and SPECT,
electrocortocography. A psychiatric role is also required before surgery to obtain
baseline cognitive data or when there are behavioral disorders that will be used to
compare outcomes after surgery.
35
CHAPTER IV
DISCUSSION
4.1 Diagnosis
In this case the patient had more than two seizures without
provocation and occurred in the time intervals of more than 24 hours. The
seizures recurred without provocation and occurred in the last 11 years,
but there was no epilepsy syndrome in this patient. This condition fulfills
the criteria for defining epilepsy based on ILAE 2014.
The incidence of epilepsy varies between industrialized countries
and developing ones. In Western countries, new cases per year are
estimated to be 33.3–82/100,000, in contrast to the maximum incidence of
187/100,000 estimated in developing countries. In particular, recent
studies showed that the maximum incidence occurs in the first year of age
with a rate of 102/100,000 cases per year, just like the age range from 1 to
12. In children from 11 to 17 years old, the incidence is 21–24/100,000
cases. Previous studies suggest that the total incidence of epilepsy is
constant from 25 years, showing a slight increase in males. In Italy,
epilepsy incidence is 48.35/100,000 new cases per year, and it is
comparable with the data recorded in other industrialized countries. The
peak of incidence occurs in children younger than 15 years old
(50.14/100,000 new cases per year) and especially in the first year of life
with an incidence of 92.8/100,000 new cases per year. In this case, the
child was diagnosed with epilepsy for the first time when he was 8 years
old, according to the findings of the age group that the most epilepsy is
found in children.
Based on ILAE 2017, the child has a type of atonic seizure, and the
EEG results are suspicious of originating from the dominant left bifrontal
and left frontto-temporal regions, while the long term EEG results in
October 2019 have not been obtained. Based on the type of epilepsy, the
EEG images and the MRI results were found in these patients, and there
was no specificity that led to a particular epilepsy syndrome. The estimates
36
of the etiology of epilepsy in these children may still be a structural result
based on EEG examinations suspected of being in the left bifrontal and
fronto-temporal areas although the MRI results suggest that there is a
suspicion of mild atrophy in the right and left temporal and parietal areas.
Genetic disorders such as gene polymorphisms in these patients cannot be
excluded. Meanwhile, the other causes such as infection, metabolic or
immune disorders can be ruled out based on the history and physical
examination.
4.2 Epilepsy management

Management of epilepsy with medical therapy of anti-epileptic
drugs (AED) is the first choice. Before starting AED, the diagnosis of
epilepsy or epilepsy syndrome must be made first. The individual response
to AED depends on the type of seizure, the classification of epilepsy and
the epilepsy syndrome. Giving AED to epilepsy sufferers must also
consider the risks and benefits. The principle of epilepsy treatment is to
start with first-line monotherapy using AED according to the type of
seizure (Table 5). Drug administration starts from a low dose and is
increased gradually until an effective dose is reached or side effects occur.
If the seizure cannot be stopped with the maximum dose of first-line AED,
second-line monotherapy will start. If second-line monotherapy is
successful in stopping the seizure, the first-line AED is tapered off. If there
is a rise when the first line AED decreases, the second OAE is still given.
The child had received anti-epileptic drugs since the age of 8 years
old when he first received first-line AED, namely valproic acid. The
choice of AED is suitable as first-line AED in the type of atonic seizure.
The rate of tapering off for three months of using epilepsy drugs is
recommended when there is a requirement for seizure-free idiopathic
epilepsy with one type of AED and normal EEG image before tapering
off. While tapering off for six months was considered in the cases of
symptomatic epilepsy, epilepsy syndrome, reduced EEG image before the
dose still showed epileptiform waves and there was developmental
37
disturbance. Studies in children found no significant difference in
recurrence rates between gradual discontinuation of drugs over a period of
9 months or 6 weeks.
After getting first-line AED, the child still had complaints of
seizures so that AED was not tapered off. Then, the frequency of seizures
in the child increased. The child's AED dose was then increased from the
initial dose, but there was still no change in seizure frequency. Therefore,
the patient was given a combination of therapy.
Table 5. Antiepileptic Drugs for the treatment of seizure
If the first OAE fails as monotherapy, there are considerations for

starting a combination OAE. Before starting combination OAE, there are
several things to consider, including (1) whether the diagnosis is correct;
(2) whether the monitoring of taking medication is good; and (3) whether
the choice and dose of OAE are appropriate. In this case, the patient's
diagnosis was right, taking medication was also good, and the choice of
first-line OAE was also right, but there was still an increase in the
frequency of seizures so that combination OAE therapy could be
considered for the patient.
38
If first-line treatment with sodium valproate is unsuitable,
ineffective or not tolerated, lamotrigine is offered as adjunctive treatment
to children, young people and adults with tonic or atonic seizures.
Discussion with a tertiary epilepsy specialist is necessary when adjunctive
treatment is ineffective or not tolerated. Other AEDs that may be
considered by the tertiary epilepsy specialist are rufinamide and
topiramate. carbamazepine, gabapentin , oxcarbazepine, pregabalin,
tiagabine or vigabatrin must not be offered.( source: NICE)
During in outpatient at Dr. Kariadi Hospital Semarang, the child
had EEG examination and the result was epileptogenic waves, and then the
child was given first-line combination AED therapy with valproic acid and
second-line AED with topiramate. The frequency of seizures in children
was not changed so that the topiramate dose increased to 75 mg / 12 hours.
The frequency of seizures was felt to be the same compared to before it,
and the child continued to take routine medication with the dose increased
according to the evaluation in the outpatient.
Based on the guidelines for AED combination, when the initial
drug has been given in optimal doses, drugs are added with different
mechanisms of action, avoiding combining drugs with the same
mechanism of action, titrating the new drug slowly and carefully, reducing
the dose of the first drug, and replacing the drug that is used. It is less
effective when the response is still poor. Then, various combinations of
two different kinds of AED should be tried and add a third drug when the
control is still not optimal. The effect of the interaction between valrpoic
acid and topiramic acid is clinically irrelevant, and the mechanism is
unknown.
Approximately two years after the consumption of the AED
combination, the frequency of seizures in the child increased. The child
received additional second-line AED therapy, namely levetiracetam at a
dose of 500 mg / 12 hours. These AED indications are suitable for
generalized epilepsy. Valproic acid and topiramic acid are metabolized in
the liver in common, affect the enzyme cytochrome P450, can affect the
39
metabolism of other types of AED while levetiracetam does not. AED side
effects may occur and are the biggest cause of discontinuation of therapy.
Some dose-related side effects are predictable but also unpredictable.
All anti-epileptic drugs have a relatively high incidence of adverse
reactions, and they are a major cause of discontinuing treatment.
Monotherapy is recommended wherever possible – monotherapy is
effective in controlling the majority of seizures and adverse effects and
interactions are less likely than with the multi-drug regimens. All anti-
epileptic drugs are associated with and often cause CNS adverse effects
which include behavioural effects and cognitive dysfunction. This effect is
more likely with polytherapy. Children may be more susceptible to these
effects which can have consequences in terms of difficulty with behaviour,
learning and development. Many adverse effects of anti-epileptic drugs are
dose related and may occur at therapeutic doses. To minimise these, the
drug should be introduced at a low dose and be slowly increased to the
minimum dose that achieves seizure control.
The common adverse effects attributable to sodium valproate are :
• Hepatobiliary disorders – increased liver enzymes are common,
particularly in early treatment and may be transient (there are cases of
severe liver dysfunction – see below)
• GI disorders – nausea and diarrhoea occur frequently at the start of
treatment but disappear after a few days without discontinuing treatment.
It is taken with or after food. Very rare cases of pancreatitis have been
reported
• Nervous system disorders – occasional sedation is reported, usually
when sodium valproate is used in combination with other anti-epileptic
drugs. Hyperactivity and behavioural deterioration have also been
reported. Tremor is reported at high doses.
• Metabolic disorders – the cases of isolated and moderate
hyperammonaemia without change in liver function tests may occur
frequently, are usually transient, and should not cause treatment
discontinuation. However, they may present clinically as vomiting, ataxia
40
and increasing clouding of consciousness. When these symptoms occur,
treatment should be stopped.
• Weight gain – this is significant, and being overweight at the start of
treatment may be a significant predictor of further weight gain with
sodium valproate
• Hair loss – it is usually transient and sometimes dose related. Regrowth
normally begins within 6 months
• Blood dyscrasias – it is relatively frequent and usually return to normal
when sodium valproate is discontinued
• Reproductive system and breast disorders – amenorrhoea and
irregular periods have been reported
Severe liver damage has been very rarely reported with sodium
valproate. Those most at risk, especially in cases of multiple
anticonvulsant therapy, are infants and young children less than 3 years of
age and those with severe seizure disorders, organic brain disease and/or
congenital metabolic or degenerative disease associated with mental
retardation. In most cases, reported liver damage occurs during the first 6
months of therapy with the period of maximum risk being 2-12 weeks. It is
suggested that liver function be monitored before and then periodically
during the first 6 months of therapy, especially in those at most risk and
those with a prior history of liver disease.
In these patients, there were no reports of idiosyncratic AED
reactions such as severe hypersensitivity reactions. Behavioral, cognitive
and developmental disorders were also reported in these children. Children
are said to be more likely to be quiet and difficult to grasp about
something than their peers. There were no reports of gum hypertrophy,
signs of impaired liver function, duct disorders, weight gain, and reactions
related to the side effects of AED used. Several months after the child met
the criteria for the three types of AED, there were complaints of loss and
the frequency of child seizures increased compared to before. Of the three
types of AED given valproic acid, they have the potential for unexpected
effects (side effects) to become alopecia. The choice of a few months and
41
the child got additional clobazam therapy. For now, the patient does not
handle hair loss complaints.
Clobazam is a derivative of the benzodiazepine class of drugs and
is included in second-line AED. This drug, which has anticonvulsant and
anti-anxiety effects, has been shown to be effective and safer in treating
refractory epilepsy in both children and adults. Clobazam works by
binding to the GABA, a receptor where clobazam will bind to local
receptors consisting of (alpha2 and gamma 2), in contrast to other
benzodiazepines that bind with (alpha1 and gamma2). This is what makes
clobazam a lighter sedative effect than other benzodiazepine drugs. Drug
interactions between clobazam and the drugs that stimulate the production
of CYP3A4 and CYP2C19 such as carbamazepine, phenobarbital and
phenytoin will cause the clobazam concentration to fall to about 44.4% in
children. Clobazam has been shown to be effective as an adjunct to
refractory epilepsy, including with focal, generalized tonic-clonic,
myoclonic or absent seizure types. The research by Kalra et al. 88 children
with all types of epilepsy proved that clobazam was able to control
seizures in 60.2% of epilepsy patients. The research by Scott et al in 30
patients with refractory epilepsy given clobazam for 2-3 years showed a
decrease in seizure frequency in 43% of patients with a few unexpected
effects.
4.3 Diagnoasis Drug-Resistant Epilepsy (DRE)

After the child received three types of AED, the child's seizures were still
felt with a constant frequency. After 4 years of consuming seizure drugs, it
could reach 10-20 times in 24 hours. Based on the stages in determining
drug-resistant epilepsy, the first step was to determine the category of the
patient's treatment outcome. This patient was included in the 2B category
because the child had not been seizure free in the last 12 months with a
failure from several times giving AED therapy to this patient accompanied
by a history of adverse events in the form of alopecia when taking valproic
acid. In the second stage in determining drug-resistant epilepsy, the
42
patient's condition was in accordance with the definition of drug-resistant
epilepsy, namely the failure of two times the administration of adequate
therapy, the right choice and according to the schedule of administering
anti-epileptic drugs (both monotherapy and combination therapy) to
achieve seizure free. The child received five different AED
administrations; the first monotherapy was with valproic acid, the second
was a combination of valproic acid therapy with topiramate, the third was
valproic acid, topiramate and levetiracetam, the fourth was a combination
therapy of valproate acid, topiramate, levetiracetam and lamotrigine, and
the fifth combination therapy was valproate acid, topiramate,
levetiracetam, lamotrigine, and clobazam. The provision of therapy was
adequate, the right choice and always consumed by the child, but the
frequency of seizures increased. Then, based on the definition of ILAE, it
can be said as drug-resistant epilepsy.
There are several risk factors that can cause a child to develop
drug-resistant epilepsy, including the age under 1 year, symptomatic
epilepsy, neuropsychiatric disorders, previous history of febrile seizures,
status epilepticus, abnormal EEG and abnormal imaging results. The
failure to use monotherapy can also put patients at risk of developing drug-
resistant epilepsy. In these patients, the risk of drug-resistant epilepsy
includes an abnormal EEG image and failure to use monotherapy.
4.4 Non-medical management

Before epilepsy surgery, a preoperative evaluation that must be
done is divided into general evaluations and specific evaluations. The
general evaluation includes evaluation of the type of seizure present and
the state of neuropsychological sequelae, psychiatry and quality of life.
Meanwhile, the specific evaluation includes seizure type, seizure location
on EEG, presence or absence of intracranial lesions, clinical and
developmental status, history of epilepsy syndrome. However, the
minimum data that must be available before performing epilepsy surgery
are the data related to semiology, interictal EEG, MRI of at least 1.5 tesla,
43
and psychological data. When there is a discrepancy in the data from the
examination results, the examination is repeated. However, when it is still
not suitable, a Positron Emission Tomography (PET scan) / intracranial
EEG can be performed. The preoperative evaluation data in the patient had
the type of seizure seen from the results when recording the EEG, but
there was no data from the psychologist. The seizure type in this child was
included in the atony seizure type, with the location of the seizure
suspected in the left bifrontal and frontotemporal areas based on the EEG
results, the MRI results showed that the impression was within normal
limits. In the end, the patient was given a longterm EEG. Then, the patient
performed a surgical epileptic craniotomy (corpus callosotomy).
Corpus callosotomy should be considered in patients with
frequent secondary generalized tonic-clonic, tonic, and atonic seizures that
lead to falls and injuries. The goal of the procedure is to disrupt the major
central pathways necessary for the propagation of generalized seizures.
Complete callosal section may result in mutism, apraxia, or frontal lobe
dysfunction. For this reason, the procedure is often performed in two
stages beginning with sectioning of the anterior two thirds followed by
section of the remainder of the corpus callosum, if necessary. Nearly two
thirds of patients experience a significant reduction in seizures, although
few are rendered seizure free.
There is a postoperative assessment according to Engel et al.
divided into 4 classes, consisting of seizure-free criteria, seizures being
infrequent, the presence of improvement and no improvement. In 2001,
ILAE developed a new classification for determining the surgical outcome
of epilepsy based on a modification of Engel et al. Criteria, divided into 6
classifications (Table 6).
44
Table 6. A classification of outcome with respect to epileptic seizures
Klasifikasi Definisi
keluaran
1 Completely seizure free; no auras
2 Only auras; no other seizures
3 One to three seizure days per year; ± auras
4 Four seizure days per year to 50% reduction of baseline
seizure days; ± auras
5 Less than 50% reduction of baseline seizure days to
100% increase of baseline seizure days; ± auras
6 More than 100% increase of baseline seizure days; ±
auras
The patient performed epilepsy surgery in the form of a corpus
callosotomy. Corpus callosotomy achieves seizure freedom from drop attacks in
more than 80% of patients. Total section of the corpus callosum is more effective
than partial section. Corpus callosotomy is employed as a last resort in most
situations because the indications and efficacy are not definitive. The efficacy of
corpus callosotomy has been measured by various criteria, such as more than 50%
seizure reduction, more than 75% reduction, or freedom from drop episodes.
Corpus callosotomy results in complete seizure remission in a small number of
patients. Up to 10% of patients experience Engel Class I outcome after surgery.
However, the clinical factors indicating such curative outcome have never been
investigated. Corpus callosotomy should be considered for the patients with
substantial disability from attacks who are not favorable candidates for focal
resection. (Chan, A. Y., Rolston, J. D., Lee, B., Vadera, S., & Englot, D. J. (2018).
Rates and predictors of seizure outcome after corpus callosotomy for drug-
resistant epilepsy: a meta-analysis. Journal of Neurosurgery, 1–10.
doi:10.3171/2017.12.jns172331)
In the patient, he was included in criteria 5 although there was a rare
postoperative frequency of seizures. The seizures still occurred more than 4 days
in the last year and the seizures had decreased by 50% compared to seizures
before surgery and no aura in the patient. A previous study of callosal electrical
potentials showed that the corpus callosum does not simply transfer the epileptic
activities from one hemisphere to the other, but rather facilitates simultaneous
45
activation, or synchrony, of epileptic neurons in both hemispheres. It is important
to note that epileptiform discharges were present postoperatively in the
electroencephalograms obtained in all of the patients in this study, suggesting that
corpus callosotomy alone does not completely suppress epileptic activities.
Sectioning of the corpus callosum prevents the development of bisynchronous
seizure activities, and in selected cases, a single hemisphere is not sufficient to
generate spontaneous seizures, or the seizures can be well controlled by
antiepileptic medications.( Iwasaki, M., Uematsu, M., Sato, Y., Nakayama, T.,
Haginoya, K., Osawa, S., … Tominaga, T. (2012). Complete remission of seizures after
corpus callosotomy. Journal of Neurosurgery: Pediatrics, 10(1), 7–
13. doi:10.3171/2012.3.peds11544)
Several previous studies had examined various factors thought to
influence seizure-free status in postoperative corpus callosotomy patients. These
factors are: the earlier age at surgery, the absence of abnormalities on the MRI,
unidentified cause, the type of corpus callosotomy (total or partial callosotomy),
and the epilepsy duration of < 15 years. From Shim et al's study, it performed 1
stage total corpus callosotomy in 34 patients with pediatric generalized epilepsy
(the mean age at surgery 8.7 years). A very high total seizure freedom rate (35%)
was reported. Pinard et al. reported that in patients after West syndrome partial
callosotomy for drop attacks was effective in only 3 of 11 (27%) patients, whereas
the total section was effective in 8 of 9 (89%). (De Tisi, J., Bell, G. S., Peacock, J.
L., McEvoy, A. W., Harkness, W. F., Sander, J. W., & Duncan, J. S. 2011. The
long-term outcome of adult epilepsy surgery, patterns of seizure remission, and
relapse: a cohort study. The Lancet, 378(9800), 1388–1395. doi:10.1016/s0140-
6736(11)60890-8 )
The earlier the operation is carried out, it is hoped that it can control the
child's seizures and improve the child's quality of life. A high rate of complete
seizure remission was observed in a study that primarily employed total
callosotomy, and 1-stage total callosotomy is increasingly indicated for poorly
functioning children with intractable epilepsy because of the minimum risks for
disconnection syndrome.
In a previous study conducted by Iwasaki M, et al, the median age at
surgery was 7 years in this study, younger than in previous reports. No permanent
46
neurological deterioration was observed postoperatively. Some studies reported an
association between lower age at surgery and a higher proportion of patients with
sustained seizure freedom. As younger people obviously have had their epilepsy
for a shorter time than older adults, the patient’s age at surgery may confound the
association between epilepsy duration and seizure outcome. However, a positive
association was also found in the studies considering both age and epilepsy
duration at the time of surgery. (Bjellvi J, Ollson I, malmgren K, Ramsay KW.
2019. Epilepsy duration and seizure outcome in epilepsy surgery. Neurology
;93:e159-e166. doi:10.1212/WNL.0000000000007753 )
The age of epilepsy onset determines the prognosis of epilepsy in
children where epilepsy that occurs since the onset of early life will give a worse
prognosis than those aged 4-10 years. High seizure frequency, long seizure time,
and status of epileptic episodes tend to cause congenital impairment. Inadequate
treatment of epilepsy that occurs over a long period of time causes disruption of
psychosocial function which results in decreased quality of life (low academic,
non-independent and limited lifestyle). Based on the research in Semarang, the
length of suffering can also affect the seizure-free ratio after surgery. The duration
of the sufferer was less than 10 years with a value that had a seizure-free ratio.
In the case of age at surgery, in 12 years of age, and the age at the start
of epilepsy, at 8 months of age, the awakening of atonic seizures provided a good
prognosis for post-epilepsy surgical outcome. While the old factor of having
epilepsy was 12 years, the high frequency of seizures reached 10-20 times a day.
A medical history existed before could give a poor prognosis for post-epilepsy
surgical outcome.
4.4.1 Ketogenic diet

The ketogenic diet has been widely used as a treatment for drug-resistant
childhood epilepsy since the first reports of its beneficial action in seizure control.
Although the exact mechanism of action is still unclear, the high fat and restricted
carbohydrate content of the diet is thought to mimic the biochemical response to
starvation. Ketone bodies become the main fuel for the brain’s energy demands. The
diet has been shown to be effective in retrospective and prospective observational
47
studies: more than half of children who were treated showed a greater than 50%
reduction in seizures, and many were seizure free after only 3 months.
This diet is recommended if the use of anti-epileptic drugs has failed or
unwanted side effects of the drugs have occurred. There are 4 types of ketogenic
diets, namely classic ketogenic diet, modified Atkins diet (MAD or modified
Atkins diet), medium chain triglyceride diet (MCT or medium chain triglyceride)
and low glycemic index therapy (LGIT or low glycemic index treatment). The
ketogenic diet is usually applied to children with 2-3 days of fasting first followed
by an increase in calories within 3 days later but with close monitoring of knock
objects because of the risk of ketosis.
The International Ketogenic Diet Study Group, issued a consensus on
the use of the ketogenic diet in children who have failed 2 or 3 anticonvulsant
drugs, especially in children with symptomatic generalized epilepsy. The
ketogenic diet is thought to act as an anticonvulsant, antiepileptogenic and
neuroprotective effect. it is hoped that administration can reduce or eliminate
seizures. In this patient, a ketogenic diet was administered, but because the patient
became weak and the frequency of seizures did not decrease, the parents refused
to continue.
4.4.2 Ketogenic diet mechanism
5. The exact mechanism of

action of ketogenic diet is
still not fully clear.
However, preclinical
studies have
48
6. suggested following
possible mechanism as
alterations in
mitochondrial function,
effect of ketone
7. bodies and fatty acids,
and glucose stabilization.
Ketone bodies easily cross
the blood brain barrier, and
8. are only source of
energy to brain during
starvation. Substitution of
glucose by ketones in
brain results
9. in decreased glycolysis
and increased krebs cycle
49
for energy production.
Ketone bodies causes γ-
10. aminobutyric acid
(GABA) synthesis,
neuronal membrane
hyperpolarization,
decrease release of
11. glutamate, increase
norepinephrine and
adenosine, neuroprotective
and antioxidant activity,
decrease
12. insulin-like growth
factor 1 (IGF-1) and the
mammalian target of
rapamycin (mTOR), and
increase
50
13. sirtuins and adenosine
monophosphate-activated
protein kinase (AMPK) in
brain. The GABA is
14. synthesized during
krebs’s cycle when
glutamate is converted into
GABA by enzyme
glutamate
15. decarboxylase. Studies
have shown that beta-
hydroxybutyrate (ketone
body) inhibits GABA-
16. transaminase expression
thus increases GABA in
brain. Ketone bodies
51
regulates activity of these
enzyme
17. thus increases the level
of GABA in the brain.
Increased level of GABA
results in stimulation of
chloride
18. receptors and ATP
sensitive potassium
channel leading to
hyperpolarisation of
neuronal membrane and
19. inhibition of sodium
and calcium channels
leading to decreased
excitability. Studies have
shown ketone
52
20. bodies (acetoacetate
more than
betahydroxybutyrate)
affect VGLUT channels on
presynaptic glutamate
21. vesicles (via chloride
channels), thus leading
decreased release of
excitatory glutamate
22. neurotransmitters.
Ketone bodies also cause
alteration in level of
biogenic amines such as
increase in
23. nor-epinephrine and
adenosine, and decrease in
dopamine and serotonin.
53
Increased adenosine
causes
24. activation of adenosine
A1 receptors resulting in
decreased brain
excitability. Ketone bodies
have
25. neuroprotective role by
causing mitochondrial
biogenesis. It also have
antioxidant role by
increasing
26. uncoupling protein in
mitochondrial electron
transport chain thus
reducing reactive oxygen
species and
54
studies have
alterations in
effect of ketone
55
brain results
(GABA) synthesis,
neuronal membrane
hyperpolarization,
decrease release of
norepinephrine and
decrease
56
mammalian target of
increase
brain. The GABA is
GABA by enzyme
glutamate
57
enzyme
chloride
sensitive potassium
channel leading to
58
shown ketone
more than
59
increase in
Increased adenosine
causes
decreased brain
have
antioxidant role by
increasing
60
species and
studies have
alterations in
effect of ketone
61
brain results
(GABA) synthesis,
neuronal membrane
hyperpolarization,
decrease release of
62
norepinephrine and
decrease
mammalian target of
increase
brain. The GABA is
63
GABA by enzyme
glutamate
enzyme
chloride
64
sensitive potassium
channel leading to
shown ketone
more than
65
increase in
Increased adenosine
causes
decreased brain
have
66
antioxidant role by
increasing
species and
71. increasing level of
mitochondrial reduced
glutathione level thus
protecting mitochondrial
level from
72. oxidative stress.
Increase in sirtuins causes
67
increase in mitochondrial
number and size, and also
cause
73. decrease in insulin like
growth factor-1. Increase
in AMPK is directly
related to ATP production.
Ketone
74. bodies by inhibiting
mTOR pathway have
anticonvulsant action.
Since ketone bodies
metabolizing
75. enzyme (namely,
monocarboxylic acid
transporter) is found more
68
abundant in infants and
children,
76. which later decreases
with increasing age and
adults. Therefore the KD is
more effective during
infants
77. and childhood than in
adulthood
The exact mechanism of action of ketogenic diet is still not fully clear. However,
preclinical studies have suggested following possible mechanism as alterations in
mitochondrial function, effect of ketone bodies and fatty acids, and glucose
stabilization. Ketone bodies easily cross the blood brain barrier, and are only source
of energy to brain during starvation. Substitution of glucose by ketones in brain
results in decreased glycolysis and increased krebs cycle for energy production.
Ketone bodies causes γaminobutyric acid (GABA) synthesis, neuronal membrane
hyperpolarization, decrease release of glutamate, increase norepinephrine and
adenosine, neuroprotective and antioxidant activity, decrease insulin-like growth
factor 1 (IGF-1) and the mammalian target of rapamycin (mTOR), and increase
sirtuins and adenosine monophosphate-activated protein kinase (AMPK) in brain.
The GABA is synthesized during krebs’s cycle when glutamate is converted into
GABA by enzyme glutamate decarboxylase. Studies have shown that beta-
hydroxybutyrate (ketone body) inhibits GABA transaminase expression thus
increases GABA in brain. Ketone bodies regulates activity of these enzyme thus
increases the level of GABA in the brain. Increased level of GABA results in
69
stimulation of chloride receptors and ATP sensitive potassium channel leading to
hyperpolarisation of neuronal membrane and inhibition of sodium and calcium
channels leading to decreased excitability. Studies have shown ketone bodies
(acetoacetate more than betahydroxybutyrate) affect VGLUT channels on
presynaptic glutamate vesicles (via chloride channels), thus leading decreased
release of excitatory glutamate neurotransmitters. Ketone bodies also cause
alteration in level of biogenic amines such as increase in nor-epinephrine and
adenosine, and decrease in dopamine and serotonin. Increased adenosine causes
activation of adenosine A1 receptors resulting in decreased brain excitability. Ketone
bodies have neuroprotective role by causing mitochondrial biogenesis. It also have
antioxidant role by increasing uncoupling protein in mitochondrial electron transport
chain thus reducing reactive oxygen species and increasing level of mitochondrial
reduced glutathione level thus protecting mitochondrial level from oxidative stress.
Increase in sirtuins causes increase in mitochondrial number and size, and also cause
decrease in insulin like growth factor-1. Increase in AMPK is directly related to ATP
production. Ketone bodies by inhibiting mTOR pathway have anticonvulsant action.
Since ketone bodies metabolizing enzyme (namely, monocarboxylic acid
transporter) is found more abundant in infants and children, which later decreases
with increasing age and adults. Therefore the KD is more effective during infants
and childhood than in adulthood.
(Jain S. Management of Epilepsy with Ketogenic Diet. Indian Journal of Psychosocial Sciences. 2017
Apr; 7(1):15-20)
77.1.1 Ketogenic diet indication and contraindication

There are several specific conditions for which the group considered that KDTs
should be used early in the course of epilepsy management (indications). For 7
specific conditions (glucose transporter 1 deficiency syndrome (Glut1DS), pyruvate
dehydrogenase deficiency (PDHD), epilepsy with myoclonic-atonic seizures,
infantile spasms, tuberous sclerosis complex, children with gastrostomy tubes, and
Dravet syndrome), 88% of the consensus group believed that KDTs should be
strongly considered very early in the course of treatment.
70
Table . Epilepsy syndromes and conditions for which KDT has been consistently
reported as more beneficial (>70%) than the average 50% KDT response (defined as
>50% seizure reduction).
Indications
Angelman syndrome
Complex 1 mitochondrial disorders
Dravet syndrome
Epilepsy with myoclonic–atonic seizures (Doose syndrome)
Glucose transporter protein 1 (Glut-1) deficiency syndrome (Glut1DS)
Febrile infection–related epilepsy syndrome (FIRES)
Formula-fed (solely) children or infants
Infantile spasms
Ohtahara syndrome
Pyruvate dehydrogenase deficiency (PDHD)
Super-refractory status epilepticus
Tuberous sclerosis complex
KDTs are contraindicated in several specific disorders (Table ). The

metabolic adaptation to KDT involves a shift from use of carbohydrates to lipids as
the primary energy source. As such, a patient with a disorder of fat metabolism
might develop a severe deterioration in the setting of fasting or KDTs. Therefore,
before initiating the KDT, a child should be screened for disorders of fatty acid
transport and oxidation if there is clinical concern for one of these conditions,
especially in the setting of epilepsy without a clear etiology.
Table . Contraindications to the use of KDT

Contraindications
Absolute Carnitine deficiency (primary)
Carnitine palmitoyltransferase (CPT) I or II deficiency
Carnitine translocase deficiency
71
β-oxidation defects
Medium-chain acyl dehydrogenase deficiency (MCAD)
Long-chain acyl dehydrogenase deficiency (LCAD)
Short-chain acyl dehydrogenase deficiency (SCAD)
Long-chain 3-hydroxyacyl-CoA deficiency
Medium-chain 3-hydroxyacyl-CoA deficiency
Pyruvate carboxylase deficiency
Porphyria
Relative Inability to maintain adequate nutrition
Surgical focus identified by neuroimaging and video-EEG
monitoring
Parent or caregiver noncompliance
Propofol concurrent use (risk of propofol infusion syndrome may
be higher)
77.1.2 How to provide a ketogenic diet

The ketogenic diet is recommended for a minimum of 2 years, and most
children gradually return to a normal diet after controlled seizures. Anamnesa of
food intake, especially diet recall, food texture, food allergies and intolerances,
supplementation, drugs and bowel habits need to be done before starting the
ketogenic diet.
The ketogenic diet ratio can be increased gradually every 3 days
according to patient response. During treatment, activity levels of blood glucose,
urine ketones, fluid intake and output, serum electrolyte levels, seizure activity,
and bowel pattern. In these patients prior to starting the ketogenic diet, the
evaluation evaluations were carried out which are summarized in the table below.
Table . Recommendations for pre-KDT evaluation
72
The ketogenic diet is also available in the form of a formula, making and
serving it relatively easy. There are currently two commercial products aimed at
the ketogenic diet, namely Ketocal powder® (Nutricia) and Ross carbohydrate −
free® (Abbott). In several studies, formula administration was more beneficial
due to high levels of adherence and efficacy than the ketogenic diet of solid foods.
The following are various types of ketogenic diets that are often used.
Table. Type of Ketogenic Diet
73
(Misiewicz Runyon, A., & So, T.-Y. (2012). The Use of Ketogenic Diet in
Pediatric Patients with Epilepsy. ISRN Pediatrics, 2012, 1–10.
doi:10.5402/2012/263139 )
This patient was given a classic ketogenic diet with a ratio of 4: 1 (4
grams of fat: 1 gram of protein and carbohydrates) obtained from ketogenic milk
(Dancow 4: 1) and ketogenic meals. The calorie needs of these patients are
calculated based on the RDA. The number of calories can be increased according
to the child's tolerance to achieve optimal growth and prevent weight loss.
The ketogenic diet like other therapies is not without side effects and
monitoring is necessary to prevent complications. Short-term side effects include
dehydration, mild metabolic acidosis and hypoglycemia. While long-term side
effects include nephrolithiasis, constipation, vitamin and mineral deficiencies,
increased cholesterol, stunted growth in young children, and decreased bone
mineral density. As an evaluation of the occurrence of side effects, monitoring
and follow-up every 3 months is needed.
The duration of the ketogenic diet was varied in each epilepsy patient.
The estimated duration of this therapy should be discussed with the patient or
patient's family at the time of initiating the ketogenic diet. However, most patients
are advised a minimum of 3 months to initiate the ketogenic diet. During the first
6 weeks it is usually seen whether the ketogenic diet is successful or not. If the
seizure is controlled after several months, it may be considered to reduce or stop
74
anti-epileptic drugs. Patient follow-up was done every 3 months until the diet was
stopped, and monitoring of growth, laboratory parameters (complete blood count,
electrolytes, liver and kidney function, lipid profile and urinalysis), and seizure
activity were carried out.
Patients during previous treatment have been treated with a ketogenic diet after 17
days of children receiving the ketogenic diet, the frequency of seizures is still
around 2 to 15 times a day. This result is different from several studies, such as in
the study of Gall et al, which stated that giving a ketogenic diet can reduce the
frequency of seizures in intractable pediatric epilepsy patients and a 2012
systematic review, Levy et al. Concluded that the ketogenic diet provides short
and medium term benefits in controlling seizures. when compared to modern
antiepileptic drugs.
This patient is post-hospitalized, the patient is discharged for follow-up and
monitoring every 1 month, followed every 3 months to assess the efficacy of the
ketogenic diet and the possibility of side effects. However, parents stop the
ketogenic diet because parents feel their child becomes very weak after running
the ketogenic diet and the frequency of seizures remains the same. So that in
patients, the ketogenic diet only lasted 17 days after hospitalization after which it
was not continued.
77.2 Prognosis
Prognostic factors that are likely to confer a better outcome include:
1. Younger age at surgery and onset of seizures;
2. Seizure type (notably drop attacks) and epilepsy syndrome;
3. Slow spike wave patterns on ictal and interictal electroencephalography
(EEG);
4. Reduced synchronicity of postoperative discharges.
5. normal magnetic resonance imaging (MRI)ntelligence quotient (IQ) > 50.
Moreover drop attacks have a better response than other generalized seizures to
corpus callosotomy. The only outcome classification systems that were referenced
by the studies included were the Engel classification system and Spencer &
75
Spencer’s classification system. The choice of classification of seizure outcomes
may have affected the significance of outcomes. The assessment of outcomes, short
or long term, is further complicated because patients usually have additional often
severe neuropsychological problems. Other outcomes may be more important to
parents than seizure reduction. Early intervention in resective surgery reduces the
progress of epileptic encephalopathy and has been associated with improved
lifespan. One study included in this review found that QOL and behavior outcomes
were correlated with seizure outcome. The primary outcome would be clinically
worthwhile reduction of drop attacks (i.e., Engel class I or II). Secondary outcomes
could include Engel class I or II of other seizures, QOL, disability, and death.
(Graham, D., Tisdall, M. M., & Gill, D. (2016). Corpus callosotomy outcomes in
pediatric patients: A systematic review. Epilepsia, 57(7), 1053–
1068. doi:10.1111/epi.13408 )
After epilepsy surgery, the child experienced clinical improvement in terms
of the frequency of seizures and quality of life. Before surgery, the assessment of the
patient's quality of life was based on the PEDSQL score of the epilepsy module
which was calculated by looking at five aspects consisting of the impact on his life
with a result of 38.8; cognitive function 37.5; sleep quality or feeling tired 66.7;
executive function 37.5; and the quality of feelings or behavior 60. After surgery, it
was found that the quality of life was improved in all functions, namely the impact
on life to 91.67; cognitive function 70.8; sleep quality to 83.3; 58.3 school quality
and the quality of feelings or behavior to 75. There is an increase in the quality of
life in all functions including the quality of children's sleep.
Based on the predictive factors for recurrence in these patients after
epilepsy surgery, these children have more good predictive factors than bad
predictive factors. Death in children with epilepsy can be caused by complications
from seizures such as aspiration or arrhythmia, seizure accidents, comorbid
conditions and suicide or sudden unexpected death in epilepsy (SUDEP). So that
the prognosis in this patient is quo ad vitam: dubia ad bonam, quo ad sanam:
dubia ad night and quo ad functionam: dubia ad bonam.
REFERENCE
76
Kalilani L, Sun X, Pelgrims B, Noack-Rink M, Villanueva V. The epidemiology of drug-
resistant epilepsy: A systematic review and meta-analysis. Epilepsia.
2018;59(12):2179–93.
Jayakar P, Gaillard WD, Tripathi M, Libenson MH, Mathern GW, Cross JH. Diagnostic
test utilization in evaluation for resective epilepsy surgery in children. Epilepsia.
2014;55(4):507–18.
Fisher RS, Acevedo C, Arzimanoglou A, Bogacz A, Cross JH, Elger CE, et al. ILAE
Official Report: A practical clinical definition of epilepsy. Epilepsia.
2014;55(4):475–82.
77

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Diajukan tanggal :

DRUG RESISTANCE EPILEPSY (ATONIC EPILEPSY/DROP ATTACK)

DEPARTMENT OF CHILD HEALTH

Epilepsy is the most prevalent serious neurologic condition, affecting approximately 70

Chief complaint: Seizure

Past medical history

Cranial nerve examinations

Clinical chemistry results

Figure 3. Head MRI with contrast

Communications, information, and educations plan

Head MRI with contrast (16/4/2018) :

Tone : decrease CP Flaccid

Risk factors & findings Diagnosis & complication Management Prognosis

Stimulating – Loving – Caring – Integrated Psychosocial Support

3.1.2 Incidence of Epilepsy

3.2 Drug Resistance Epilepsy (DRE)

Table 2 Scheme for categorizing outcome of an intervention for epilepsy

Administration of adequate therapy is a therapy with the appropriate

Table 3. Minimum dataset required to determine whether the trial of a therapeutic

The term seizure-free is a condition free from all types of seizures

Etiology of Drug Resistant Epilepsy

3.3 Surgical Epilepsy

Epilepsy surgery is considered successful in treating children with drug-

4.2 Epilepsy management

Table 5. Antiepileptic Drugs for the treatment of seizure

If the first OAE fails as monotherapy, there are considerations for

4.3 Diagnoasis Drug-Resistant Epilepsy (DRE)

4.4 Non-medical management

4.4.1 Ketogenic diet

4.4.2 Ketogenic diet mechanism

5. The exact mechanism of

77.1.1 Ketogenic diet indication and contraindication

KDTs are contraindicated in several specific disorders (Table ). The

Table . Contraindications to the use of KDT

77.1.2 How to provide a ketogenic diet

Table . Recommendations for pre-KDT evaluation

Table. Type of Ketogenic Diet

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