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Antibodies to clustered acetylcholine receptor: expanding the phenotype

Authors

  • P. Devic,

    1. Ecole Normale Supérieure de Lyon, Laboratoire de Biologie Moléculaire de la Cellule, CNRS UMR 5239, Université Lyon 1, Lyon
    2. Hospices Civils de Lyon, Service d'explorations neurologiques, Centre de Référence des Maladies Neuromusculaires, Lyon
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    • These authors have contributed equally to this work.
  • P. Petiot,

    1. Hôpital de la croix rousse, Hospices Civils de Lyon, Service d'explorations neurologiques, Centre de Référence des Maladies Neuromusculaires, Lyon
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    • These authors have contributed equally to this work.
  • T. Simonet,

    1. Ecole Normale Supérieure de Lyon, Laboratoire de Biologie Moléculaire de la Cellule, CNRS UMR 5239, Université Lyon 1, Lyon
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  • T. Stojkovic,

    1. Institut de Myologie, Hôpital de la Pitié Salpétrière, Paris
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  • E. Delmont,

    1. CHU de Nice, Centre de Référence des Maladies Neuromusculaires et SLA, Nice
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  • J. Franques,

    1. Département de Neurologie et des Maladies Neuromusculaires, CHU la Timone, Marseille
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  • A. Magot,

    1. Département de Neurologie, CHU de Nantes, Centre de Référence des Maladies Neuromusculaires, Nantes
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  • C. Vial,

    1. Hôpital neurologique Pierre Wertheimer, Hospices Civils de Lyon, Service d'électroneuromyographie, Centre de Référence des Maladies Neuromusculaires, Bron
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  • E. Lagrange,

    1. Département de Neurologie, CHU de Grenoble, Centre de Référence des Maladies Neuromusculaires, Grenoble
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  • A. S. Nicot,

    1. Ecole Normale Supérieure de Lyon, Laboratoire de Biologie Moléculaire de la Cellule, CNRS UMR 5239, Université Lyon 1, Lyon
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  • V. Risson,

    1. Ecole Normale Supérieure de Lyon, Laboratoire de Biologie Moléculaire de la Cellule, CNRS UMR 5239, Université Lyon 1, Lyon
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  • B. Eymard,

    1. Institut de Myologie, Hôpital de la Pitié Salpétrière, Paris
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  • L. Schaeffer

    Corresponding author
    1. Ecole Normale Supérieure de Lyon, Laboratoire de Biologie Moléculaire de la Cellule, CNRS UMR 5239, Université Lyon 1, Lyon
    • Correspondence: L. Schaeffer, Laboratoire de Biologie Moléculaire de la Cellule, Ecole Normale Supérieure de Lyon, 46 allée d'Italie, 69 364 Lyon Cedex 07, France (tel.: 0033 472 728171; fax: 0033 472 728600; e-mail: laurent.schaeffer@ens-lyon.fr).

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Abstract

Background and purpose

To provide a detailed phenotypical description of seronegative patients with generalized myasthenia gravis and antibodies to clustered acetylcholine receptors (AChRs) and to assess their frequency amongst a French seronegative generalized myasthenia gravis (SNMG) population.

Methods

A French SNMG database was created and the sera from the 37 patients included in it were analysed by immunofluorescence of cell-based assays using cotransfection of AChR subunit genes together with rapsyn to densely cluster the AChRs.

Results

Sixteen per cent (n = 6) of the SNMG patients were found to have antibodies to clustered AChR. They presented either with early onset MG and thymic hyperplasia, late onset MG and thymic involution, or thymoma associated MG. They responded well to cholinesterase inhibitors and immunosuppressants.

Conclusions

Patients with antibodies to clustered AChR account for a significant proportion of SNMG patients and resemble patients with AChR antibodies detected by standard radio-immunoprecipitation.

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