A Case Of Acute Fulminating Myasthenia Gravis In Childhood.
- Author:
Won Seok CHOI
1
;
Seung Hun HAN
;
Sun Mi KIM
;
Jung Hyun LEE
;
Dae Chul JEONG
;
Jin Tack KIM
;
Jin Han KANG
;
Min Wook KIM
;
Seung Yun CHUNG
Author Information
1. Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea. sycped@olmh.cuk.ac.kr
- Publication Type:Case Report
- Keywords:
Acute fulminating myasthenia gravis;
Neostigmine test;
EMG
- MeSH:
Acetylcholine;
Adolescent;
Antibodies;
Child, Preschool;
Diagnosis;
Electromyography;
Encephalitis;
Humans;
Male;
Myasthenia Gravis*;
Neostigmine
- From:
Journal of the Korean Child Neurology Society
2002;10(1):160-165
- CountryRepublic of Korea
- Language:Korean
-
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.
