급성 전격성 소아 중증 근무력증 1례

A Case Of Acute Fulminating Myasthenia Gravis In Childhood

저자들은 근무력증의 증상이 없었던 환아에서 상기도 감염 후 갑자기 발생한 연하장에 및 사지마비로부터 급격하게 진행된 호홉 마비를 주소로 내원한 3세 남아에서 임상경과와 neostigmine 검사와 반복 신경 자극 근전도 검사에 의해 근무력증 위기로 진행된 급성 전격성 소아 중증 근무력증을 확진하였기에 문헌 고찰과 함께 보고하는 바이다.

Juvenile myasthenia gravis is an acquired autoimmune disorder of childhood, in which circulating antibodies against the acetylcholine receptor(AchR Ab) interfere with normal neuromuscular transmission. Myasthenia gravis in childhood and adolescence is rare, comprising 10% of all cases. The diagnosis is based essentially on the patient's history and clinical examination with a positive anticholinesterase(edrophonium or neostigmine) test. Electromyography (EMG) and serum AchR Ab are also important to confirm the diagnosis. We report a case of acute fulminating myasthenia gravis in a 3 year old male patient who was suspected as Guillain-Barr syndrome or brain-stem encephalitis. Diagnosis was made by neostigmine test and EMG. He recieved high dose intravenous immunoglobulin(IVIG, 400 mg/kg for 5days) and anticholinesterase(pyridostigmine) therapy. He showed gradual improvement in symptoms and signs.