Page 349 - Abstract Book KONIKA 18

Neurology

P-NEU-025
Clinical Characteristics of Focal Cortical Dysplasia in Children:
A Retrospective Evaluation in 13 Patients

Nisa Hermina Putri, Nelly Amalia Risan, Mia Milanti Dewi, Riska Munggaran
Department of Child Health, Hasan Sadikin General Hospital/Faculty of Medicine, Universitas Padjadjaran,
Bandung, West Java, Indonesia

Abstract
Background Focal cortical dysplasia (FCD) is a malformation of cortical development caused by
genetic mutation or extrinsic factors leading to abnormal layers of cerebral cortex. It is a common
cause of intractable epilepsy. Objective To know the clinical characteristics of children with FCD.
Methods This is a descriptive study of FCD patient diagnosed in Hasan Sadikin General Hospital since October
2020 to June 2021. Results There were 13 children, age 1 to 17 years old diagnosed as FCD, consisted of 8
boys and 5 girls. Most of them came with intractable epilepsy and others were diagnosed without epilepsy.
From magnetic resonance imaging (MRI), there were same proportion of type I and type II (38,5 %) and type
III was less common (23 %). Five patients had FCD and hippocampal sclerosis (dual pathology) from MRI
result. There was difference seizure onset in each type of FCD, 2 weeks–6 years in type I, 4 months–10 years
in type II and 6 months–10 years in type III. There was 1 patient who had history of febrile seizure. Most
of parents complainted focal seizure type in the onset of seizure. Almost all of the patients had intractable
epilepsy while just 3 patient showed good seizure control using 1 antiepleptic drug (carbamazepin/valproic
acid/topiramate). Conclusion FCD is common in our tertiary hospital with intractable epilepsy as primary
manifestation. FCD result in neuroimaging evaluation can be the reason to perform surgical resection in
order to achieve good seizure control.
Keywords: focal cortical dysplasia; electroencephalogram; neuroimaging; children

P-NEU-026
Acute Disseminated Encephalomyelitis in Child with Dextrocardia Mirror Image,
Double Outlet Right Ventricle, and Severe Infundibular Pulmonary Stenosis:
A Case Report

Irwan Santoso, Kristy Iskandar, Agung triono
Departement of Child Health, Faculty of Medicine, Public Health and Nursing,Universitas Gadjah Mada/
Dr. Sardjito Hospital, Special District of Yogyakarta, Central Java Indonesia
Abstract
Background Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating disorder of the
central nervous system and is characterized by multifocal white matter involvement and diffuse neurological
signs along with multifocal lesions in brain. Diagnostic criteria of ADEM have 75% of sensitivity and
95% of specificity. Most patients with ADEM improve with methyl prednisolone. Objective To report a
case of ADEM in an eleven-year-old male with cyanotic heart disease. Case An eleven-year-old male was
referred from a private hospital with persistent cyanotic spell, altered consciousness and right hemiparesis
due to suspicious of brain abscesses. The patient was diagnosed with double outlet right ventricle (DORV)
since three-year-old but lost to follow up. Three weeks prior, the patient had severe headache and recurrent
cyanotic spell. He was then hospitalized in a private hospital for four days, but no clinical improvement
thus referred to our hospital. His initial oxygen saturation was 66 until 72%. Echocardiography result was
reduced left ventricle function with EF 26,7%, DORV and severe infundibular PS (PG 45 mmHg). Laboratory
studies revealed haemoglobin 21.9 g/dL, leucocyte 7.150/ µL, thrombocyte 139.000/µL, D-Dimer 5634ng/
mL and contrast head MRI revealed multiple pathologic lesions irregular, hypo-iso-hyperintense T1W in
thalamus, corpus callosum, pons and cerebellum. The patient fulfilled ADEM clinical criteria following
encephalopathy, multifocal area of CNS and no other aetiology and ADEM radiological criteria following
multifocal hyperintense and asymmetric lesions. We administered high dose methyl prednisolone for three
days and planned to continuing the protocol for six months. Conclusion ADEM in cyanotic heart disease
child may be a coincidence. Eighty percent patient with ADEM had complete recovery in one until six month
of high dose steroid therapy.

Keywords: acute disseminated encephalomyelitis; double outlet right ventricle; methyl-prednisolone

KONIKA XVIII Abstract Book 301