Page 356 - Abstract Book KONIKA 18

Neurology

P-NEU-039
Corticosteroid and Valproic Acid for Treatment of Chorea
in A Child with Acute Rheumatic Fever: A Case Report

Vetty Kurniawati, Alifiani Hikmah Putranti
Department of Child Health, Faculty of Medicine Universitas Diponegoro/Dr. Kariadi General Hospital,
Semarang, Central Java, Indonesia

Abstract
Background Chorea is one of cardinal sign of acute rheumatic fever (ARF), a movement disorder
characterized by a continuous flow of unpredictable sequences of multiple discrete movements and affect
quality of life. Recent systematic reviews suggested the treatments of chorea in ARF, include prophylactic
penicillin, antipsychotic, anticonvulsant, and immunomodulatory therapy. The rationale of corticosteroid
treatment in chorea is based on the immune mechanism that plays a crucial role in the pathogenesis of ARF
and chorea. Objective To evaluate efficacy of corticosteroid and valproic acid in child with chorea in ARF
Case A 12-year-old girl undergoing treatment of ARF, and Graves' disease presented with inability to move
extremities and choreiform movement. Physical examination showed limb weakness, facial grimacing,
motor aphasia, involuntary movements of extremities, lagophthalmos, and neck struma. The brain MRI
contrast displayed minimally symmetrical lesions on the posterior peritrigonal white matter related to
Sydenham's chorea. The child was hospitalized for 13 days. The initial treatment was haloperidol 1 mg/day for
1 week, but the outcome was not as expected. Then, we stopped haloperidol therapy and started prednisone
th
2 mg/kgBW/day and valproic acid 20 mg/kgBW/day as choice of treatment for chorea in ARF. On the 6
day after prednisone and valproic acid therapy, patient was able to sit alone and spoke clearly. Then, patient
was discharged home. In outpatient care, patient was able to walk after two weeks of prednisone and valproic
acid therapy. Conclusions Prednisone and valproic acid are very effective as the treatment of chorea in ARF.
Keywords: chorea; acute rheumatic fever; treatment; prednisone; valproic acid

P-NEU-040
Evaluation of Corticosteroid Therapy for West Syndrome Patients
in Dr. Kariadi Hospital Semarang

Vetty Kurniawati, Alifiani Hikmah Putranti
Department of Child Health, Faculty of Medicine Universitas Diponegoro/Dr. Kariadi General Hospital,
Semarang, Central Java, Indonesia
Abstract
Background West syndrome (WS) is severe epilepsy syndrome with neurodevelopmental morbidity and
high mortality in children. Adrenocorticotropic hormone (ACTH) dan prednisolone is The United Kingdom
Infantile Spasms Study (UKISS) recommendation therapy for WS, but it is not available in Indonesia. We
attempt prednisone as a substitute therapy for WS in Dr. Kariadi Hospital Semarang. Objective To evaluate
prednisone therapy in West syndrome patients in Dr. Kariadi Hospital Semarang. Methods This descriptive
study used data from medical records of children diagnosed with West Syndrome from January 2019 until
June 2021 in Dr. Kariadi Hospital Semarang. Results Seven patients with WS consist of male (14.3%)
and female (85.7%). Most patients were microcephaly (85.7%) and delayed milestones (100%). The brain
MRI showed cerebral atrophy (71.4%), microcephalic (14.3%), cortical tubers (14.3%). Age of first seizure
≤9 months (100%) and less than 10 seizures a day (85.7%) dominated this research. Six patients (85.7%)
received therapeutic doses of 40-60 mg/day prednisone according to the UKISS Recommendation. During
therapy, four patients showed reduction of seizures frequency, and three patients were seizure-free. After
stopping prednisone, four patients were seizure-free, while others relapse. EEG before treatment showed
hypsarrhythmia (71.4%), burst suppression (14.3%), and normal EEG (14.3%). In the EEG pre-and post-
therapy comparison, we found 5 patients with EEG improvement, 1 patient did not, and 1 patient had not
undergone EEG post-therapy. Conclusion Prednisone improved clinical presentation and EEG outcomes of
patients with WS, but studies with larger samples still need to be done.
Keywords: West syndrome; treatment; prednisone; seizure; EEG

308 KONIKA XVIII Abstract Book