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

Kee Hong Park, Patrick Waters, Mark Woodhall, Bethan Lang, Thomas Smith, Jung Joon Sung, Kwang Kuk Kim, Young Min Lim, Jee Eun Kim, Byung Jo Kim, Jin Sung Park, Jeong Geon Lim, Dae Seong Kim, Ohyun Kwon, Eun Hee Sohn, Jong Seok Bae, Byung Nam Yoon, Nam Hee Kim, Suk Won Ahn, Jeeyoung OhHyung Jun Park, Kyong Jin Shin, Yoon Ho Hong

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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.

Original languageEnglish
Article numbere0193723
JournalPLoS One
Volume13
Issue number3
DOIs
Publication statusPublished - 2018 Mar 1

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ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)
  • Agricultural and Biological Sciences(all)

Cite this

Park, K. H., Waters, P., Woodhall, M., Lang, B., Smith, T., Sung, J. J., Kim, K. K., Lim, Y. M., Kim, J. E., Kim, B. J., Park, J. S., Lim, J. G., Kim, D. S., Kwon, O., Sohn, E. H., Bae, J. S., Yoon, B. N., Kim, N. H., Ahn, S. W., ... Hong, Y. H. (2018). Myasthenia gravis seronegative for acetylcholine receptor antibodies in South Korea: Autoantibody profiles and clinical features. PLoS One, 13(3), [e0193723]. https://doi.org/10.1371/journal.pone.0193723