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Myasthenia gravis seronegative for acetylcholine receptor antibodies in South Korea: Autoantibody profiles and clinical features

Title
Myasthenia gravis seronegative for acetylcholine receptor antibodies in South Korea: Autoantibody profiles and clinical features
Authors
Park, Kee HongWaters, PatrickWoodhall, MarkLang, BethanSmith, ThomasSung, Jung-JoonKim, Kwang-KukLim, Young-MinKim, Jee-EunKim, Byung-JoPark, Jiri-SungLim, Jeong-GeonKim, Dae-SeongKwon, OhyunSohn, Eun HeeBae, Jong SeokYoon, Byung-NamKim, Nam-HeeAhn, Suk-WonOh, JeeyoungPark, Hyung JunShin, Kyong JinHong, Yoon-Ho
Ewha Authors
박형준
SCOPUS Author ID
박형준scopusscopus
Issue Date
2018
Journal Title
PLOS ONE
ISSN
1932-6203JCR Link
Citation
vol. 13, no. 3
Publisher
PUBLIC LIBRARY SCIENCE
Indexed
SCIE; SCOPUS WOS scopus
Abstract
Acquired myasthenia gravis (MG) is a prototype autoimmune disease of the neuromuscular junction, caused in most patients by autoantibodies to the muscle nicotinic acetylcholine receptor (AChR). There seem to be ethnic and regional differences in the frequency and clinical features of MG seronegative for the AChR antibody. This study aimed to describe the autoantibody profiles and clinical features of Korean patients with generalized MG seronegative for the AChR antibody. A total of 62 patients with a high index of clinical suspicion of seronegative generalized MG were identified from 18 centers, and we examined their sera for antibodies to clustered AChR, muscle-specific tyrosine kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (LRP4) by cell-based assays (CBA) and to MuSK by radioimmunoprecipitation assay (RIPA). We also included 8 patients with ocular MG, 3 with Lambert-Eaton myasthenic syndrome, 5 with motor neuron disease, and 9 with other diagnoses as comparators for the serological testing. Antibodies were identified in 25/62 (40.3%) patients: 7 had antibodies to clustered AChR, 17 to MuSK, and 2 to LRP4. Three patients were double seropositive: 1 for MuSK and LRP4, and 2 for MuSK and clustered AChR. The patients with MuSK antibodies were mostly female (88.2%) and characterized by predominantly bulbar involvement (70%) and frequent myasthenic crises (58.3%). The patients with antibodies to clustered AChR, including 2 with ocular MG, tended to have a mild phenotype and good prognosis.
DOI
10.1371/journal.pone.0193723
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의료원 > 의료원 > Journal papers
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