Pentobarbital로 치료된 저산소성 뇌손상 후 발생한 난치성 근간대성 경련 간질 중첩증(란스-아담스 증후군) 1례

A Case of The Successful Treatment of Pentobarbital for P osthyp oxic Action Myoclonus(Lance-Adams Syndrome) with Refractory Status Epilepticus

저자들은 란스-아담스 증후군을 보이는 15개월 남아에서 일차 항경련제 투여에도 불구하고 1시간 이상 지속된 난치성 간질 중첩증이 발생하였을 때 pentobarbital 투여로 경련이 조절된 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

Lance-Adams syndrome is a rare complications of cardiorespiratory arrest. We report a 15-month-old child with Lance-Adams syndrome who fell into a refractory status epilepticus after a successful resuscitation after the cardiopulmonary arrest from upper airway obstruction due to the croup. On the day of the admission, he went through a generalized tonic-clonic seizure with poor mental status. His condition became aggravated on 18th day of admission when he developed focal myoclonus in his left arm and leg. The EEG findings before the pentobarbital treatment show partial electrical seizure. The seizures were intractable despite the administration of midazolam, phenobarbital, phenytoin, and valproic acid. Therefore, a pentobarbital(PTB) therapy was required. PTB administered by a continuous infusion pump at a loading dose of 5 mg/kg was sufficient to produce a burst suppression pattern and a seizure control, followed by a maintenance infusion of 0.5-1.5 mg/kg/hour. At that time, the brain MRI showed a diffuse distribution of high signal intensity and swelling in both basal ganglia, thalamus and the temporo-occipital area. The therapy continued for 10 days. Thereafter, PTB was gradually tapered after a minimum 48-hours of a seizure-free status. So we report a case with a brief review of related literature.