Astri Budikayanti*, Teguh AS Ranakusuma*, Mursyid Bustami*, Joedo Prihartono**
Departemen Neurologi FK Universitas Indonesia / RSUPN Cipto Mangunkusumo, Jakarta

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Journal : Neurona

PROFIL KLINIS BENIGN ROLANDIC EPILEPSY (BRE) DI RSUD PASAR REBO TAHUN 2013-2018 Donny Hamdani Hamid; Syairah Syairah; Syarly Melani; Astri Budikayanti
NEURONA Vol 36 No 4 (2019)
Publisher : PERDOSNI

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.52386/neurona.v36i4.84

Abstract

   CLINICAL PROFILE OF BENIGN ROLANDIC EPILEPSY (BRE) PASAR REBO GENERAL REGIONAL HOSPITAL 2013-2018ABSTRACTIntroduction: Benign rolandic epilepsy (BRE) is the most common focal epileptic syndromes in children, correlated with genetic abnormality, has unique clinical features and specific electroencephalogaphic (EEG) pattern. Most of BRE cases will remit spontaneously even without medication by puberty.Aims: To know the clinical profile of BRE and the association between electroencephalogaphic (EEG) pattern with and seizure types in BRE.Methods: Observational analytic cross-sectional study based on medical record of BRE cases in Pasar Rebo general regional hospital from January 2013–Desember 2018. Subjects were grouped into focal to bilateral tonic clonic seizure (group I) and focal onset seizures with impaired awareness (group II). Relationship between groups and hemispheric lateralization based on interictal epileptiform (unilateral or bilateral) using EEG was analyzed.Results: There were 22 subjects, average age of seizure onset was 7,86+2,3 years old with male-to-female ratio1,75:1. The majority of subjects (40.9%) had only received an EEG after more than 1 year of onset and only 6 (27.3%) subjects had done less than one month.. Amongst all subjects, 15 subjects (68,2%) already consumed anti epileptic drugs (AED), in which 11 subjects (73,3%) consumed valproic acid (VPA). Most common clinical features were hypersalivation and hemifacial sensorimotor symptoms with localization of interictal epileptiform discharges mainly in centro-temporal. There were no significant relationship between hemispheric lateralization of epileptiform discharge and type of seizure.Discussion: Most BRE subjects had onset of 7.86+2.3  years with the main symptoms of hypersalivation  and hemifacial sensorimotor symptoms, as well as localization of interictal epileptiform discharges especially in the centro- temporal.. There were no significant relationship between hemispheric lateralization of interictal epileptiform discharges and seizure type.Keywords: BRE, clinical profiles, epilepsy syndromesABSTRAKPendahuluan: Benign rolandic epilepsy (BRE) merupakan sindrom epilepsi fokal yang paling sering ditemukan pada anak kelompok usia 1-14 tahun, berhubungan dengan kelainan genetik, memiliki gambaran klinis dan EEG khas. Sebagian besar mengalami remisi tanpa obat pada usia pubertas.Tujuan: Mengetahui karakteristik profil klinis dan hubungan antara gambaran elektroensefalografi (EEG) dengan bentuk bangkitan pada BRE.Metode: Studi potong lintang analitik observasional menggunakan data rekam medispasien yang didiagnosis BRE di Klinik Saraf RSUD Pasar Rebo, Jakarta, periode Januari 2013 hingga Desember 2018. Subjek dikelompokkan menjadi tipe bangkitan awitan fokal yang berkembang menjadi tonik klonik bilateral (grup I) dan tipe bangkitan awitan fokal dengan gangguan kesadaran (grup II), yang dihubungkan dengan lateralisasi hemisferik aktivitas epileptiform interiktal (unilateral atau bilateral) pada EEG.Hasil: Dari 22 subjek, rasio perbandingan laki-laki:perempuan sebesar 1,75:1 dan rerata usia awitan bangkitan adalah 7,86+2,3 tahun. Mayoritas subjek (40,9%) baru dilakukan EEG setelah lebih dari 1 tahun dari awitan dan hanya 6 subjek (27,3%) yang dilakukan kurang dari satu bulan. Pada 15 subjek (68,2%) telah mengkonsumsi obat antiepilepsi (OAE) yang sebagian besar berupa asam valproat (73,3%). Gambaran klinis utama adalah hipersalivasi dan gejala sensorimotor hemifasial dengan lokalisasi cetusan epileptiform interiktal terutama di sentro-temporal. Tidak didapatkan hubungan bermakna antara lateralisasi hemisferik aktivitas epileptiform interiktal dengan bentuk bangkitan.Diskusi: Sebagian besar subjek BRE mengalami usia awitan bangkitan 7,86+2,3 tahun dengan gejala utama hipersalivasi dan gejala sensorimotor hemifasial, serta lokalisasi cetusan epileptiform interiktal terutama di sentro-temporal. Tidak didapatkan hubungan bermakna antara lateralisasi aktivitas epileptiform interiktal dengan jenis bangkitan pada BRE.Kata kunci: BRE, profil klinis, sindrom epilepsi  
HUBUNGAN KADAR ANTIBODI RESEPTOR ASETILKOLIN DENGAN DERAJAT KEPARAHAN PENYAKIT MIASTENIA GRAVIS DI RS CIPTO MANGUNKUSUMO Manfaluthy Hakim; Fika Tiara; Ahmad Yanuar; Winnugroho Wiratman; Luh Ari Indriawati; Astri Budikayanti; Fitri Octaviana
NEURONA Vol 37 No 3 (2020)
Publisher : PERDOSNI

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.52386/neurona.v37i3.164

Abstract

Introduction: Acetylcholine receptor (AChR) antibody is main antibody in pathogenesis of myasthenia gravis (MG). AChR antibody is an important diagnostic tests. Myasthenia gravis clinical features can be divided into ocular, generalized and bulbar MG. Severity of MG can be assessed based on MG composite score (MG-cs). Aim: To evaluate association between level of AChR antibody and MG severity based on MG severity in Cipto Mangunkusumo Hospital. Methods: This was a descriptive-analytic cross-sectional study which were done at Cipto Mangunkusumo Hospital during January to November 2017. Inclusion criteria were MG patients aged 18-75 years old. Assessment of MG composite score and blood withdrawal were performed at the same time. The level of AChR antibody was measured by ELISA method. Results: There were 72 subjects met inclusion criteria. Mean age was 43 years old (SD 12.56) with proportion higher in women (2.5:1). Ocular MG was the most initial symptom (79.2%) and early onset was the most common(77.8%). There were 59.7% subject who had seropositive AChR antibody. There was no significant association between seropositive AChR antibody and gender, age of onset, MG type, MGFa classification, immunotherapy. There was no association between level of AChR antibody and MG-cs(p=0.727). Discussion: There wasn’t any association between level of AChR antibody and MG severity based on MG-cs. Keywords: AChR antibody, MG composite score, MG severity
UJI DIAGNOSTIK ICE PACK TEST DAN REPETITIVE NERVE STIMULATION SERTA KOMBINASINYA PADA MIASTENIA GRAVIS Winnugroho Wiratman; Bazzar Ari Mighra; Fitri Octaviana; Astri Budikayanti; Luh Ari Indrawati; Triana Ayuningtyas; Ahmad Yanuar Safri; Manfaluthy Hakim
NEURONA Vol 38 No 3 (2021)
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Abstract

Introduction: Diagnosis of myasthenia gravis (MG) is based on clinical symptoms, physical examination and supporting examination; so far, no examination has become the primary standard in the enforcement of MG. Supporting examinations that are fast, non-invasive and easy to do are ice pack test (IPT) and repetitive nerve stimulation (RNS). Aims: To find the sensitivity and specificity of the IPT, RNS examination, and the combination of both in enforcing the diagnosis of MG. Methods: This study was conducted with a cross-sectional design using primary data and medical records of suspicious MG patients with ptosis in Cipto Mangunkusumo General Hospital between July-November 2019. Results: Of the 38 subjects with ptosis, 35 subjects were confirmed MG with positive SF-EMG/AChR antibodies/response therapy. The ice pack test was positive in 29 subjects (76.3 %). The diagnostic test results of the ice pack test have sensitivity 80%, Specificity 66.67%, area under the curve (AUC) value of 73.3%, the RNS has a sensitivity of 60%, specificity 100%, AUC value 80%, while the combination test has sensitivity 94.28%, specificity 66.67% and AUC value 80.5%. Discussion: The combination of IPT and RNS has good diagnostic value so it can be used as a supporting examination to the diagnosis of MG. Keywords: diagnostic test; ice pack test; myasthenia gravis; ptosis; RNS
FAKTOR-FAKTOR YANG MEMENGARUHI LUARAN THERAPEUTIC PLASMA EXCHANGE PADA KRISIS MIASTENIA DI RSUPN CIPTO MANGUNKUSUMO Manfaluthy Hakim; Triana Ayuningtyas; Ahmad Yanuar; Nadia Ayu Mulansari; Winnugroho Wiratman; Luh Ari Indrawati; Astri Budikayanti; Fitri Octaviana
NEURONA Vol 38 No 4 (2021)
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Introduction: Myasthenic crisis (MC) occurs due to worsening symptoms of myasthenia gravis (MG) characterized by acute respiratory failure, prolonged post-thymectomy intubation or bulbar weakness causing dyspnoea. Therapeutic Plasma Exchange (TPE) has been accepted as first-line therapy for MC. The management of TPE is not quite ideal according to the American Society of Apheresis (ASFA) recommendations. Aim: To determine the factors that in????uenced the outcomes of MC patients who underwent TPE Methods: This study was a cross-sectional design by taking secondary data on MC cases that received TPE at RSUPN Cipto Mangunkusumo from January 2014 to September 2018. The diagnosis of MC is based on clinical examination. Bivariate analysis was carried out on factors that infuenced TPE outcomes in MC. Results: A total of 38 cases met the inclusion criteria. Good outcomes after undergoing TPE are found in 60.5% of cases. The onset of MG (p=0.039) and type of MG (p= 0.001) have a significant relationship with TPE outcome on MC. Early-onset MG and ocular to generalized MG show good outcomes. Antibody specificity variables showed a strong trend in infuencing TPE outcome in MC (p=0.055). Patients with MC who underwent TPE according to ASFA showed faster clinical improvement than those who underwent TPE not according to ASFA. Discussion: Early-onset MG and ocular to generalized MG are factors that can affect the excellent outcome of TPE in MC. Patients who undergo TPE, according to ASFA, show faster clinical improvement. Keywords: ASFA, myasthenic crisis, therapeutic plasma exchange