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Autoimmune autonomic failure in a patient with myeloma-associated Shy-Drager syndrome

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Abstract

We report here the case of a patient with the Shy-Drager syndrome and multiple myeloma who had evidence consistent with a central neural autoimmune basis for sympathetic autonomic failure. Autonomic function testing showed no recordable peroneal skeletal muscle sympathoneural traffic, normal arterial norepinephrine (NE) spillover during supine rest and no increment in NE spillover during exposure to lower body negative pressure. The patient's cerebrospinal fluid and serum contained an immunoglobulin G that bound to rat locus ceruleus (LC) in an in vitro test system. The myeloma protein was of the λ subtype and bound in the rat LC, without binding in the substantia nigra, as demonstrated with anti-λ antiserum. Since in this case the monoclonal antibody produced by the myeloma bound specifically to LC cells, the results are consistent with the hypothesis that in this patient the Shy-Drager syndrome may have had an immune-mediated basis.

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Authors and Affiliations

  1. Clinical Neuroscience Branch, National Institute of Neurological Disorders and Stroke, Building 10, Room 6N252, Bethesda, MD, USA

    D. S. Goldstein MD PhD & C. Holmes CMT

  2. Department of Anatomy, University of Goteborg, Goteborg, Sweden

    A. McRae PhD

  3. Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, 20892, Bethesda, MD, USA

    M. C. Dalakas MD

Authors
  1. D. S. Goldstein MD PhD
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  2. A. McRae PhD
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  3. C. Holmes CMT
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  4. M. C. Dalakas MD
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Corresponding author

Correspondence to D. S. Goldstein MD PhD.

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Goldstein, D.S., McRae, A., Holmes, C. et al. Autoimmune autonomic failure in a patient with myeloma-associated Shy-Drager syndrome. Clinical Autonomic Research 6, 17–21 (1996). https://doi.org/10.1007/BF02291401

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  • DOI: https://doi.org/10.1007/BF02291401

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