Lu sur :https://www.ncbi.nlm.nih.gov/pubmed/29518096
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Myasthenia gravis seronegative for acetylcholine receptor antibodies in South Korea: Autoantibody profiles and clinical features.
Park KH1, Waters P2, Woodhall M2, Lang B2, Smith T2, Sung JJ3, Kim KK4, Lim YM4, Kim JE5, Kim BJ6, Park JS7, Lim JG8, Kim DS9, Kwon O10, Sohn EH11, Bae JS12, Yoon BN13, Kim NH14, Ahn SW15, Oh J16, Park HJ17, Shin KJ18, Hong YH19.
PLoS One. 2018 Mar 8;13(3):e0193723. doi: 10.1371/journal.pone.0193723. eCollection 2018.
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