demam rematik akut, generalised epilepsy with febrile seizure dan disabilitas intelektual

MEILY ELVEN NORA, Sasmito Nugroho, Elisabeth Siti Herini

2018 | Tesis-Spesialis | ILMU KESEHATAN ANAK

Intisari Seorang anak laki-laki usia 9,5 tahun yang telah terdiagnosis epilepsi dan disabilitas intelektual (DI) dirujuk ke rumah sakit dengan keluhan nyeri sendi berpindah-pindah, demam, dan terdengar bising jantung dengan riwayat nyeri tenggorokan tanpa batuk beberapa minggu sebelumnya. Ekokardiografi menunjukkan hasil adanya insufisiensi mitral berat. Pemeriksaan fisik dan pemeriksaan penunjang memenuhi kriteria Jones untuk demam rematik akut (DRA). Anak mendapat pengobatan penicillin V (phenoxymethilpenicillin) dan steroid oral untuk karditis berat. Anak telah mendapat obat anti epilepsi dalam 2 tahun terakhir, namun pengobatan tidak teratur. Saat ini juga mengalami kejang yang frekuen dengan dan tanpa demam. Kejang teratasi dengan asam valproat. Setelah kondisi membaik, anak dipulangkan dan direncanakan mendapat phenoxymethilpenicillin sebagai profilaksis sekunder DRA selama 10 tahun. Pada evaluasi bulan ke tiga, bising jantung sudah tidak terdengar dan ekokardiografi menunjukkan sudah tidak ditemukan insufisiensi katup mitral.

We present a 9.5-year-old male diagnosed with epilepsy and intellectual disability since 2012 who is referred to our hospital due to migratory joint pain, fever, fatigue, new murmur following group A Streptococcus (GAS) pharyngitis. The joint pain with swelling causes him to have difficulty to walk. Echocadiography reveals severe mitral insufficiency. The workup for his symptoms are consistent with acute rheumatic fever (ARF) based on Jones criteria. Despite the presentation of ARF, this boy had frequent generalised tonic-clonic seizures (GTCS) which occur with and without fever secondary to poor compliance of anti-epileptic drug. He was managed with penicillin V (phenoxymethilpenicillin) and steroids orally for severe carditis, after which he had dramatic improvement in his symptomatology and was free from seizure. The patient as treated with penicillin V and was started on steroid for severe carditis. The patient was discharged with recommendations to continue secondary prophylaxis with penicillin V everyday for the next 10 years. At the third month evaluation after the first attack, cardiac murmur disappeared and echocardiography revealed normal mitral leaflet.

Kata Kunci : demam rematik akut, epilepsi/acute rheumatic fever, epilepsy