A Case Of Acute Fulminating Myasthenia Gravis In Childhood.

Choi,  Won Seok; Han,  Seung Hun; Kim,  Sun Mi; Lee,  Jung Hyun; Jeong,  Dae Chul; Kim,  Jin Tack; Kang,  Jin Han; Kim,  Min Wook; Chung,  Seung Yun

Case Report

Journal of the Korean Child Neurology Society 2002;10(1):160-165.
Published online May 30, 2002.

A Case Of Acute Fulminating Myasthenia Gravis In Childhood.

Won Seok Choi, Seung Hun Han, Sun Mi Kim, Jung Hyun Lee, Dae Chul Jeong, Jin Tack Kim, Jin Han Kang, Min Wook Kim, Seung Yun Chung

¹Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea. sycped@olmh.cuk.ac.kr
²Department of Rehabilitation Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.

Abstract

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.

Key Words: Acute fulminating myasthenia gravis, Neostigmine test, EMG