Good afternoon doctors. Thank you for the opportunity and the time you given.

I would like
to present my case report about a 2 month old infant with Ohtahara syndrome and
severely malnourished

• The name of the patient was ARA, age 2 months 19 days old, she was hospitalized in
pediatric ward and admitted at 14st July 2020 with the chief complaint of seizure
• Two months prior to admission, when the child was 15-day-old, he started to have seizure
without any preceding fever, diarhea, vomitus, or any history of head trauma. Type of
seizure was head and eye deviation to the right, followed by bended stiffening of the right
hand, and straightening of the left leg and left hand. The duration was 2 minutes and stopped
on its own. (Based on the semiology of seizure this patient seizure is like the form number 4,
leading to lesion in the frontotemporal region) Before the seizure started, the child was
conscious. After the seizure, the child gained consciousness in a few minutes but he seemed
inactive. He was taken to local primary health care and received anticonvulsant drug.
Parents were advised to refer the child to local hospital for further management and
diagnostic procedure, but they refused to bring the child due to the pandemic. He was taken
home and still suffered from seizures up to 8 times a day with the duration of 1-2 minutes.
• Two weeks prior to admission the child still had seizures 5-8 times a day. Parents decided
to bring the child to a pediatrician in a private hospital. The child was diagnosed with
epilepsy and advised to be admitted to the hospital for four days. He received intravenous
anticonvulsants drugs and the seizures frequency was reduced to 3 times a day. He was
discharged and referred to neuropediatric clinic at tertiary hospital for further examination.
• In the pediatric neurology clinic, the child had 5 times seizure around 1 minutes and
stopped by its own. He was alert, inactive, and his vital sign is normal limit. He was
assessed as general epilepsy and severe wasted. The child was admitted for further
management.
• Admission day 2nd -3rd (on 3rd day of admission, the patient was selected as final
evaluation):, the child had seizure 17 times with same type as previous seizures, about 5-10
seconds during 3 hours of EEG procedure, was given bolus phenobarbital 80mg (20mg/kg)
intravenously maintain with phenobarbital 10mg/ 12 hours (5mg/kg/day) He was
programmed for MRI, waiting for results of EEG.
• Past medical history
No history of trauma, no history of seizure with fever
• Family medical history
His uncle was got a seizure when he was child but not clearly epilepsy
• Personal and social history
Patient was born from a G5P3A1, 39-year-old mother, with full-term pregnancy. She had
routine antenatal care. There was no history of high blood pressure, respiratory problems,
diabetes, seizures or trauma during pregnancy. Patient was born via spontaneous vaginal
delivery at primary health care. The baby cried vigorously at birth. The birth weight was
3000 gram, birth length 48 cm and head circumference 33 cm. No history of jaundice,
edema, or bleeding event was reported. Since birth he received breast milk and standard
infant formula. Present BW: 4145 gram, ideal body weight 5150 gram, length 57 cm, head
circumference 40.5 cm. Nutritional history impression is low quality and quantity intake.
Denver II was normal according to age. Basic immunization was not complete, patient
only received one dose of hepatitis B, polio. He is the fourth child in the family, he lives
with his mother, father and his siblings.

• Physical examination revealed

The general state of patient was alert, active, breathing spontaneously, vital signs it’s a
normal limit (HR 128 beats/min, RR 32 breath/min, axillary temperature was 36.5 0C) In
Head, he got normochepal, no facial dysmorphic, there were head lag, no neck stiffness,
heart and lung examination were unremarkable, no organomegaly on abdomen examination.
Neurological examination: found Increased physiological reflexes (That’s indicate upper
motor neuron), in primitive reflexes finding positive palmar and plantar grasp, moro and
rooting reflexes were negative, weak sucking reflexes (signs of central nervous dysfunction).
• Laboratory work ups such us Laboratory findings haematology and electrolyte is normal.
EEG long term (Burst suppression on right hemisphere of brain while wake and sleep state
and Multifocal spike and sharp wave on frontotemporal and intra ictal hypsaritmia), MRI
brain with contrast (Focal atrophy on the left temporal lobe)
• SUMMARY
A 2 month old male patient presented with recurrent seizure without any preceding fever,
diarhea, vomitus, or any history of head trauma. He started to have seizure was 15-day-old.
Type of seizure is head & eye deviation to the right followed by bended stiffening right hand,
straightening of the left leg and left hand, duration about 1-2 minutes and stopped on its own.
Electroencephalography was found burst suppression on right hemisphere of brain during wake
and sleep state and multifocal spike and sharp wave on frontotemporal region. Brain MRI result
focal atrophy on the left temporal lobe.
 (Suppresion burst indicate a major dysfunction of cortical networks, and might evolve into different
patterns. Decreased blood flow velocity in the middle cerebral artery in proportion to the duration of
suppression phase indicates a reduction in brain activity during this phase.) (According to the
International League Against Epilepsy 2017 (ILAE) proposed to include EEG suppression burst in the
list of epileptic encephalopathies, that is, those conditions in which not only epileptic activity, but also
epilepticform EEG abnormalities themselves are believed to contribute to the progressive disturbance
in cerebral function)
• The working diagnosis is Early Infantile Epileptic Encephalopaty (Ohtahara
Syndrome), atrophy cerebri on the left temporal lobe, oromotor delay, severely
malnourished, and incomplete routine immunization.
(Ohtahara syndrome constitute the earliest presenting of the age-dependent epileptic encephalopathy
syndromes. is a rare disease, with characteristics by epileptic tonic spasms and a suppresion-
burst. The suppression burst pattern is present in both wake and sleep states, and appears in the
first 3 months of life. Tonic spasms that can be either generalized or lateralized, This condition
frequently evolved into West syndrome and Lennox Gastaut syndrome)  (The transition is
accompanied by changes in electroencephalography (EEG) pattern, is marked by a transition from
suppression burst to hypsarhythmia), This patient might be in transition phase evolving to West
syndrome, because the EEG also revealed intra ictal hypsarhythmia waves.
(Ohtahara syndrome can result from a variety of etiologies, the majority of cases have been
associated with structural brain abnormalities, genetic mutations and metabolic abnormalities), (The
etiology of Ohtahara syndrome in this patient could be due brain atrophy as found in the results of the
head MRI. Metabolic and genetic disorders that cause seizures in this case cannot be ruled out
because genetic testing and tests for metabolic disorders have not been evaluated to confirm.)
• For management of seizure He was given 1 lpm oksigen via nasal cannula, IVF D51/2NS
20 ml/hour, iv phenobarbital 10mg/12jm, prednisone 7,5 mg/6 hours (8mg/kgbb/hari),
valproic acid 0,6ml/8 hours, pyridoxine 7,5mg/6hours, and sucralfate syr 1ml/8 hours.
(Epileptic encephalopathies with a suppresion burst pattern in neonates might be evident with
deficiency pyridoxine or pyridoxamine phosphate oxidase, therefore, in cases withour a clear etiology,
a treatment with intrvenous pyridoxine or oral pyridoxal-5- phosphate should be tried), (Corticosteroids
can be used as the first line AED and phenobarbitone, vigabatrin, zonisamide, and ketogenic diet for
adjunctive AEDs), (Cochrane Review reported the efficacy of corticosteroids in seizure control,
improvement in cognition and quality of life), (In this case, during hospitalized treatment corticosteroid
and phenobarbitone were effective enough in preventing recurrent seizures.)
• Follow up day-1-2 ( days 4-5 hospitalization) The patient still had seizure, 17 times during
EEG procedure, with the same type as previous seizures, weak of sucking reflex 
programed for oromotor exercise
• Follow up day 3-7 There is no seizure, no fever, therapy phenobarbital switched to oral
administration. The patient prepared to be discharged.
• The problems of this case consist of management prevent of seizure and nutritional status.
• Problem on management: Management of (Seizure, nutritional care, catch-up
immunization & biophsycosocial environment).
(The patient nutrition status was severely malnourished, with underweight, normal stature, and
mesocephaly. Patient received breast milk and standart infant formula 5x 60 ml with appropiate
dilution and breast-fed directly during the night), (The patient experienced an oromotor delay
related to Ohtahara, so even though the patient received nutrition from breast milk and formula
milk because the acceptability was not good, the nutrition provided was not optimal)
• Problems on prevention is to prevent of recurrent seizure and pneumonia complication
• Monitoring progression of ohtahara syndrome, compliance and complications of
therapy, and tolerance of nutritional interventions
• Problem on prognosis: The prognosis of Ohtahara syndrome in this patient based
unilateral suppression burts and hypsarrhythmia findings on EEG which might be poor,
the patient can either dead or handicaps in the future, high probability to become
intractable epilepsy. Is very important providing information to parents about the
disease, prevention seizure and possible longterm outcomes.
(Prognosis of Ohtahara syndrome can be divided into two groups, with considerably different
outcomes. The typical progression of epileptic syndromes starts with an suppression burst
pattern, followed by hypsarrhythmia and subsequently by diffuse slow spike waves. A different
group showed progression to focal spike pattern, becoming free from seizures over time.
Patients with unilateral suppression bursts had better outcomes in walking and
neurodevelopmental status than did patients with bilateral suppression bursts.) (result study
cohort prospektif Taiwan 2018, 22 newborn with EEG suppression bursts with varied etiologies,
In 16 patients with bilateral suppresion bursts, regardless of etiology, all had severe intellectual
disability and could not walk: 10 died before they turned 3 years old. The outcomes were worse
in patients with purely synchronous bilateral supression bursts, 75% (3 out of 4) of whom died
before they turned 3 years old. Mortality rates are higher in those who have hypsarrhythmia or
who change further to diffuse slow spike-waves, probably because of more extensive diffuse
brain damage)
 • Problem on family psychology: The psychological burden of the family is related to the
current disease of the child, fulfillment of caring, loving, and stimulation needs of the
patient form the parents with support and educate parent to accept their child’s
condition.
• Management plans consisted of seizure control, nutrition care, and monitoring progression
of seizure.
• Medical therapies in hospitalization is intravenous fluid dekstrose, and injection
Phenobarbital 10 mg/12 hours dose 5mg/kgbw/day, with oral terapi valproic acid 20
mg/kgbw/day, pyridoxine 7,5 mg/kgbw/day, prednisone 8 mg/kgbw/day and sucralfacte
1ml/8 hours. (There is no seizures during admission day 5 until discharged). Programs for seizure
problem in Outpatient therapy he got valproic acid 20 mg/kgbw/day, pyridoxine 7,5
mg/kgbw/day, prednisone 8 mg/kgbw/day and sucralfacte 1ml/8 hours, and orthomotor
physiotherapy for the problem of sucking in achieving adequate nutrition.
(Prednisone was administered at a fixed dose of 8 mg/kg/day for 14 days and tapered off), evaluate
recurrent seizure, EEG patern after 2 weeks corticosteroid terpeutic)
• Pediatric Nutrition therapy, with the target Calories 120 lcal/kgbw/day, protein 2,2
gram/kgbw/day, with oral nutrition suplemen 6x60ml and breastmilk 6x60ml. (During the
hospitalization, the breastmilk was carried out, the volume of milk obtained was up to 80 to
100 ml every 3 hours.)
(Etiologies of the oromotor disorder in this case, is related to the Ohtahara. Parents should be
educated to stimulate the oromotoric exercise. Oral-motor interventions have been shown to
be effective in improving the oral function of preterm infants and children with neuromotor
disorders) (Stimulations that can be applied when patient was sick are tender care, a cheerful,
stimulating environment, structured therapy 15-30 minutes per day, maternal involvement
(e.g. comforting, feeding, bathing, play)
• The prognosis of this case was Dubia ad malam for Quo ad vitam, Qou ad fungsionam and
Quo ad sanam.
Menurut evidence
(A systematic review of early infantile epileptic encephalopathy found in 17 cases had death as
an outcome. The mean (SD) age at death was 12.9 ± 14.1 months. Most infants (58.8%)
survived less than one year. The cause of death was described only in 8 (47%) patients, the
cause was either pneumonia/respiratory illness or SUDEP(Sudden Unexplained Death in
Epilepsy), other outcomes were excluded from this analysis, namely, progression to other
epileptic syndromes (West Syndrome, Lennox-Gastaut Syndrome, spike foci) or cessation of
seizures. Four cases were described by Ohtahara as deceased after the EEG pattern turned
from suppression burst to hypsarrhythmia. Pneumonia was the cause of death in six cases,
SUDEP in two cases, and an outcome of “respiratory illness” in one case).