ABSTRAKLatar belakang: Spasme infantil (SI) sering dikaitkan dengan kejang yang sulit terkontrol dan timbulnya disabilitas intelektual yang berat. Pengobatan SI denganACTH saat ini di Indonesia sangat terbatas. Data mengenai karakteristik, penggunaan terapi, dan luaran jangka pendek berupa bebas spasme, resolusi gambaran EEG, dan angka kekambuhan pasien SI belum terdata dengan baik.Tujuan: (1) Mengetahui insidens pasien SI. (2) Mengetahui karakteristik pasien SI. (3) Mengetahui karakteristik luaran jangka pendek pasien SI berdasarkan jenis terapi yang didapat. (4) Mengetahui faktor risiko luaran SI.(5) Membandingkan angka event free survival pasien SI berdasarkan jenis terapi yang didapat.Metode: Penelitian kohort retrospektif dilakukan pada pasien SI usia 0-2 tahun yang datang ke Poliklinik Neurologi Departemen Ilmu Kesehatan Anak RSCMdari bulan Januari 2010 hingga Desember 2015. Pengambilan data dilakukan dengan melihat data rekam medis dan wawancara kepada orangtua. Faktor- faktor yang dianggap berpengaruh dianalisis secara multivariat.Hasil: Insidens SI selama 2010-2015 86 pasien. Pada analisis bivariat, didapatkan faktor-faktor yang berpengaruh terhadap luaran tidak bebas kejang adalahklasifikasi simptomatik (p=0,039; RR 0,67; IK 95% 0,51-0,88), respon awal yang baik terhadap terapi awal (p<0,001; RR 2,69; IK 95% 1,75-4,14), dan jenis terapisaat bebas kejang (p<0,001). Pada analisis multivariat, tidak didapatkan hubungan antara masing-masing faktor. Median event free survival pada kelompok ACTH dan prednison masing-masing 8 hari dan 14 hari.Simpulan: Status bebas kejang tampaknya lebih banyak didapatkan pada pasien yang mendapat terapi ACTH dengan atau tanpa kombinasi dengna OAE. Pengobatan menggunakan prednison juga menunjukkan luaran bebas kejang yang baik dibandingkan dengan pemberian kombinasi OAE.

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ABSTRACT

Background: Infantile spasm is commonly related with uncontrolled seizure and severe intellectual disabilities. Treatment of infantile spasm with ACTH is

currently limited in Indonesia. Furthermore, some data regarding characteristics, therapies, short-term outcomes - which are free interval of spasm and resolution of electroencephalography, and recurrent rate of infantile spasm, are not well documented.

Objective: (1) To obtain the incidence of infantile spasm. (2) To obtain the characteristic of infantile spasm. (3) To obtain the characteristic of short-term

outcome of infantile spasm based on the therapies given. (4) To obtain the risk factor outcome of infantile spasm. (5) To obtain the event free survival of infantile spasm based on the therapies given.

Method: This was a retrospective cohort study on infantile spasm patients aged 0-2 years old at Neurology Outpatient Clinic of Child Health Department, Cipto

Mangunkusumo General Hospital during a period of January 2010 until December 2015. Data were taken from medical record and direct interview with parents of patients. Factors that had statistically significant were analyzed with multivariate method.

Result: The incidence of infantile spasm in 2010-2015 is 86 patients? On bivariate analysis, it was found that factors which are related with no seizure-free outcome is symptomatic classification (p=0,039; RR 0,67; CI 95% 0,51-0,88), satisfied early responds prior initial treatment (p<0,001; RR 2,69; CI 95% 1,75-4,14), and types of therapies on free interval seizure (p<0,001). On multivariate analysis, it was found that there was no correlation between each factor. The median of event free survival on ACTH and prednisone groups are 8 days and 14

days, respectively.

Conclusion: Free seizure status is generally more common in patients receiving ACTH with or without the combination of anti epileptic drugs. Treatment with

prednisone also shows good seizure-free outcome compared to combination with anti epileptic drugs.