J Korean Child Neurol Soc.
2002 May;10(1):160-165.
A Case Of Acute Fulminating Myasthenia Gravis In Childhood
- Affiliations
-
- 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.
