간질로 오인된 QT 간격 연장 증후군 1례

A Case of Idiopathic Kong QT Syndrome Presenting as Epilepsy

저자들은 선천성으로 청각 장애가 있으며 반복되는 간질 발작을 주소로 서울중앙병원에 입원하여 시행한 신경학적 검사와 뇌파 검사, 영상 진단이 모두 정상이었으나 특징적으로 실신이 유발되는 과정과 심전도상 QTc 간격이 연장되어 있어 QT 간격 연장 증후군으로 진단 받고 베타 수용체 차단제로 추적 관찰 중인 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다

"Idiopathic Kong QT Syndrome" is characterized by prolongation of the QT interval due to unusual electrocardiographic repolarization abnormality and associated with variable clinical manifestations from no specific symptoms in lifetime to syncope or even sudden death. The prognosis of this syndrome is very grave and motality is approximately 50% within 10 years among untreated symptomatic patients after the initial syncope. But this sudden onset syncope may be misdiagnosed as epilepsy, being teated with antiepileptic drug for many years. However, this high mortality has been significantly reduced to less than 5% by the effective therapy. Therefore, it is crucial to make an early and accurate dianosis. We experienced a case of 34 months old male who presented with recurrent syncopal attacks. He had no specific neurological abnomal finding except congenital deafness. He had normal EEG and brain MRI findings but ECG showed prolonged QT interval(QTc=0.5), findings of which were compatible with long QT syndrome. He is currently being followed at OPD, but the pateint is still experiencing syncopal attack despite of treatment with β-blocker, atenolol. Therefore, we are considering an insertion of pacemaker of performing thoracic sympathectomy.