Motor end-plate analysis to diagnose immune-mediated myasthenia gravis in seronegative patients

This study aimed to evaluate the diagnostic usefulness of motor end-plate (MEP) analysis along with clustered acetylcholine receptor (AChR) antibody (Ab) assays in patients with myasthenia-like symptoms but negative routine AChR and muscle-specific kinase (MuSK) Ab tests. MEP analysis of muscle biop...

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Veröffentlicht in:Journal of the neurological sciences 2022-12, Vol.443, p.120494-120494, Article 120494
Hauptverfasser: Nagaoka, Atsushi, Tsujino, Akira, Shiraishi, Hirokazu, Kanamoto, Tadashi, Shima, Tomoaki, Yoshimura, Shunsuke, Miyazaki, Teiichiro, Tateishi, Yohei, Tsujihata, Mitsuhiro, Motomura, Masakatsu, Maxwell, Susan, Higuchi, Osamu, Beeson, David, Vincent, Angela
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container_title Journal of the neurological sciences
container_volume 443
creator Nagaoka, Atsushi
Tsujino, Akira
Shiraishi, Hirokazu
Kanamoto, Tadashi
Shima, Tomoaki
Yoshimura, Shunsuke
Miyazaki, Teiichiro
Tateishi, Yohei
Tsujihata, Mitsuhiro
Motomura, Masakatsu
Maxwell, Susan
Higuchi, Osamu
Beeson, David
Vincent, Angela
description This study aimed to evaluate the diagnostic usefulness of motor end-plate (MEP) analysis along with clustered acetylcholine receptor (AChR) antibody (Ab) assays in patients with myasthenia-like symptoms but negative routine AChR and muscle-specific kinase (MuSK) Ab tests. MEP analysis of muscle biopsies of the biceps brachii was performed in 20 patients to try to differentiate between those with or without immune-mediated myasthenia gravis (MG). Using a quantitative method, complement C3 deposition and AChR densities in MEPs were examined. Independently, cell-based assays were used to detect serum clustered-AChR Abs. Only five of 20 patients had complement deposition at MEPs; four of these patients had reduced AChR densities similar to those in patients with typical AChR Ab positive MG, and distinct from those in the remaining 15 patients. Two of the four serum samples from these patients had clustered-AChR Abs. All complement-positive patients were considered as having immune-mediated MG and improved with appropriate treatments; although one patient presented with MG 3 years later, the remaining patients had other diagnoses during over 10 years of follow-up. These results suggest the usefulness of MEP analysis of muscle biopsies in diagnosing immune-mediated MG in seronegative patients with myasthenia-like symptoms but, due to the invasiveness of the muscle biopsy procedure, clustered AChR Abs should, if possible, be tested first. •A proportion of MG patients remain seronegative, providing a challenge for diagnosis.•We performed MEP analysis for seronegative patients with myasthenia-like symptoms, and observed the clinical course.•Reduced AChR densities and complement C3 deposition are sensitive and specific for diagnosing for those patients.
doi_str_mv 10.1016/j.jns.2022.120494
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MEP analysis of muscle biopsies of the biceps brachii was performed in 20 patients to try to differentiate between those with or without immune-mediated myasthenia gravis (MG). Using a quantitative method, complement C3 deposition and AChR densities in MEPs were examined. Independently, cell-based assays were used to detect serum clustered-AChR Abs. Only five of 20 patients had complement deposition at MEPs; four of these patients had reduced AChR densities similar to those in patients with typical AChR Ab positive MG, and distinct from those in the remaining 15 patients. Two of the four serum samples from these patients had clustered-AChR Abs. All complement-positive patients were considered as having immune-mediated MG and improved with appropriate treatments; although one patient presented with MG 3 years later, the remaining patients had other diagnoses during over 10 years of follow-up. 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These results suggest the usefulness of MEP analysis of muscle biopsies in diagnosing immune-mediated MG in seronegative patients with myasthenia-like symptoms but, due to the invasiveness of the muscle biopsy procedure, clustered AChR Abs should, if possible, be tested first. •A proportion of MG patients remain seronegative, providing a challenge for diagnosis.•We performed MEP analysis for seronegative patients with myasthenia-like symptoms, and observed the clinical course.•Reduced AChR densities and complement C3 deposition are sensitive and specific for diagnosing for those patients.</description><subject>Autoantibodies</subject><subject>Biopsy</subject><subject>Clustered AChR antibody</subject><subject>Complement</subject><subject>Humans</subject><subject>Motor end-plate</subject><subject>Motor Endplate</subject><subject>Myasthenia gravis</subject><subject>Myasthenia Gravis - diagnosis</subject><subject>Research Design</subject><subject>Seronegative</subject><issn>0022-510X</issn><issn>1878-5883</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2022</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kE1LAzEQhoMotlZ_gBfJ0cvWJPuRLJ5E_IKKFwVvIZudrVl2k5qkhf57U1o9ehqYed4X5kHokpI5JbS66ee9DXNGGJtTRoq6OEJTKrjISiHyYzQl6ZKVlHxO0FkIPSGkEqI-RZO8KkjOaj5F8tVF5zHYNlsNKgJWVg3bYAKODrdGLa0LgM04ri1kI6RNhBaPWxXiF1ij8NKrTaKNxQG8s7BU0WwAr9IAG8M5OunUEODiMGfo4_Hh_f45W7w9vdzfLTJdsCpmTdkpyommXcUoKwreVlqphhNoWwDNKDR5nYu8TiAVnWaVaLhoFAWeAqTNZ-h637vy7nsNIcrRBA3DoCy4dZCM56IQNS9JQuke1d6F4KGTK29G5beSErnzKnuZvMqdV7n3mjJXh_p1kyz8JX5FJuB2D0B6cmPAy6CTAJ2MedBRts78U_8D1MqKcA</recordid><startdate>20221215</startdate><enddate>20221215</enddate><creator>Nagaoka, Atsushi</creator><creator>Tsujino, Akira</creator><creator>Shiraishi, Hirokazu</creator><creator>Kanamoto, Tadashi</creator><creator>Shima, Tomoaki</creator><creator>Yoshimura, Shunsuke</creator><creator>Miyazaki, Teiichiro</creator><creator>Tateishi, Yohei</creator><creator>Tsujihata, Mitsuhiro</creator><creator>Motomura, Masakatsu</creator><creator>Maxwell, Susan</creator><creator>Higuchi, Osamu</creator><creator>Beeson, David</creator><creator>Vincent, Angela</creator><general>Elsevier B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20221215</creationdate><title>Motor end-plate analysis to diagnose immune-mediated myasthenia gravis in seronegative patients</title><author>Nagaoka, Atsushi ; 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subjects Autoantibodies
Biopsy
Clustered AChR antibody
Complement
Humans
Motor end-plate
Motor Endplate
Myasthenia gravis
Myasthenia Gravis - diagnosis
Research Design
Seronegative
title Motor end-plate analysis to diagnose immune-mediated myasthenia gravis in seronegative patients
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